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FHB

(Radiology golden books).


Revision Notes
for the
Final FRCR Part A

Kshitij Mankad MRCP FRCR


Neuroradiology Fellow
National Hospital for Neurology and Neurosurgery
London, UK

Edward TD Hoey MRCP FRCR


Consultant Cardiothoracic Radiologist
Heartlands Hospital
Birmingham, UK

London • St Louis • Panama City • New Delhi

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© 2010 JP Medical Ltd.
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Preface
The Final FRCR Part A examination is daunting to prepare for as it requires an in-depth knowledge
of anatomy, disease, differential diagnoses and applied radiology practice.
This book has been compiled to ease the revision process by presenting the requisite core
knowledge for each of the six system-based modules, namely cardiothoracic and vascular,
musculoskeletal and trauma, gastrointestinal and hepatobiliary, genitourinary, gynaecology
and breast, paediatrics, and central nervous system and head and neck. Each chapter has been
authored by recent exam candidates and in turn edited by radiology consultants with subspecialty
expertise in that field, to ensure focused and succinct coverage of high-yield exam topics.
Only the essential facts are presented, selected entirely on the basis of the scope of remembered
past questions. This should speed up the revision process by reducing time spent searching
through large textbooks and journals. Being revision notes, the content is designed to make the
knowledge-finding process easy for the stressed candidate. Each topic is presented in a concise
manner and exhaustive lists of differential diagnoses have been restricted to those that most
often get tested. For ease of reading, the main reference sources have been listed in a general
bibliography at the very end of the book rather than cited in the text.
Although primarily aimed at radiology trainees, this book will also be useful to physicians,
surgeons, medical students and anyone with an interest in radiology.

Kshitij Mankad
Edward Hoey

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Contents

Preface v
Chapter 1 Cardiothoracic and vascular systems 1
Edward Hoey, Sapna Puppala
Chapter 2 Musculoskeletal system and trauma 89
Kshitij Mankad
Chapter 3 Gastrointestinal system 133
Amit Lakkaraju, Nasim Tahir
Chapter 4 Genitourinary system, adrenal gland,
obstetrics and gynaecology, and breast 187
Prasanna Tirukonda, Jooly Joseph
Chapter 5 Paediatrics 233
Kshitij Mankad
Chapter 6 Central nervous system, and head and neck 273
Kshitij Mankad, Edward Hoey
Bibiography 365
Index 367

vii

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Contributing Authors
Jooly Joseph Nasim Tahir
Specialty Registrar in Radiology Specialty Registrar in Radiology
Leeds Teaching Hospitals NHS Trust Leeds Teaching Hospitals NHS Trust
Leeds, UK Leeds, UK

Amit Lakkaraju Prasanna Tirukonda


Specialty Registrar in Radiology Specialty Registrar in Radiology
Leeds Teaching Hospitals NHS Trust Leeds Teaching Hospitals NHS Trust
Leeds, UK Leeds, UK

Sapna Puppala
Consultant Cardiovascular and
Interventional Radiologist
Leeds Teaching Hospitals NHS Trust
Leeds, UK

Reviewers
David Gilmour Sapna Puppala
Specialty Registrar in Ophthalmology Consultant Cardiovascular and
Leeds Teaching Hospitals NHS Trust Interventional Radiologist
Leeds, UK Leeds Teaching Hospitals NHS Trust
Leeds, UK
Sanjoy Nagaraja
Consultant Interventional Neuroradiologist Nabil el Saiety
University Hospitals Coventry and Consultant Gastrointestinal Radiologist
Warwickshire NHS Trust University Hospital of North Staffordshire
Coventry, UK Stoke on Trent, UK

Naveen Parasu Hemant Sonwalkar


Assistant Professor, McMaster University Consultant Interventional Radiologist
Staff Musculoskeletal Radiologist Calderdale and Huddersfield Foundation
Henderson General Hospital, Hamilton NHS Trust
Health Sciences Halifax, UK
Hamilton, Ontario
Canada Ashok Raghavan
Consultant Paediatric Radiologist
Sheffield Children’s Hospital
Sheffield, UK

ix

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Chapter 1
Cardiothoracic and
vascular system

1.1 Lung cancer


1.2 Solitary pulmonary nodule and
congenital lung disease
1.3 Airways disease
1.4 Air space diseases
1.5 Diffuse lung disease
1.6 Occupational lung disease
1.7 Pulmonary infections
1.8 Pulmonary thromboembolism
1.9 Postoperative chest radiology
1.10 Chest trauma
1.11 Mediastinum
1.12 Aorta
1.13 Vascular disease
1.14 Cardiac imaging

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1.1 Lung cancer

• Leading cause of cancer deaths in the • Small cell cancer occurs almost exclusively
western world in smokers. It is associated with rapid
• 60% occur in men, 40% in women doubling time, early development of
(incidence increasing) metastatic disease and initial sensitivity to
• Risk factors are smoking and exposure to chemotherapy and radiation
asbestos, radon and nickel • Despite initial response to treatment, long-
term survival is much worse than that of
WHO classification patients with non-small cell carcinoma

Small cell (20% of cases) Non-small cell carcinoma


Non-small cell:
• Squamous cell (35% of cases) Squamous cell carcinoma
• Adenocarcinoma (30% of cases) • Commonest histological subtype in the UK
• Large cell (10% of cases) • Slow growing tumour with a 90-day
• Others, e.g. carcinoid (5% of cases) doubling time
• Strongest link with smoking
• Two thirds arise centrally, one third
peripherally
Small cell lung cancer • Commonest cause of a Pancoast’s tumour
• May secrete parathyroid hormone-related
• Arises from Kulchitsky’s cells of the amine peptide (PTHrP), causing hypercalcaemia
precursor uptake decarboxylase (APUD) • Strong association with hypertrophic
line pulmonary osteoarthropathy (HPOA)
• The majority arise proximally in the • Central necrosis is common, and 30%
bronchial submucosa cavitate
• Highly malignant tumour with 30-day
doubling time HPOA
• 5–10% associated with paraneoplastic
syndromes
• Symmetrical laminated periostitis of
–– Syndrome of inappropriate antidiuretic
extremities
hormone secretion (low sodium)
• Finger clubbing and painful
–– Cushing’s syndrome (increased
oedematous skin
adrenocorticotropic hormone (ACTH)
• Other thoracic causes include
secretion)
bronchiectasis, mesothelioma, pleural
–– Eaton–Lambert syndrome (myasthenia-
fibroma, cardiac myxoma and bacterial
like syndrome)
endocarditis
–– Subacute cerebellar degeneration
• Squamous cell carcinoma is
–– Limbic encephalopathy
commonest cause
Staging of small cell lung • Regresses following tumour resection
cancer
• 2009 TNM staging system stages small
cell lung cancer as part of the same Adenocarcinoma
classification system as non-small • Arises from bronchiolar epithelial glands
cell carcinoma and replaces the older • Typically a peripheral nodule with
description system of limited and spiculated margins
extensive stage disease • More common in females

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Lung cancer 3

• Predominant lung cancer type in surgically excised tissue and is the


non-smokers reference standard technique.
• Occasionally arises in scarred or fibrotic
lung T staging
• T1: tumour diameter ≤ 2 cm (T1a) or
Bronchoalveolar cell 2–3  cm (T1b) and completely surrounded
carcinoma by lung
• 2–5% of all lung cancers • T2: tumour 3–5 cm (T2a) or 5–7 cm
• Subtype of adenocarcinoma derived from (T2b) or invading either left or right
type 2 pneumocytes and bronchiolar main bronchus > 2 cm distal to carina or
epithelium invading the visceral pleura
• High prevalence within scarred or fibrotic • T3: tumour > 7 cm or tumour invading
lung either the main bronchus within 2 cm of
• Mucin-secreting tumour, which may the carina or invading non-vital structures
present with bronchorrhoea such as chest wall, mediastinal pleura,
• Malignant cells may be carried to the pericardium or diaphragm
contralateral lung –– CT is unreliable for subtle chest wall
• Extrathoracic spread is unusual invasion (65% accurate)
• F-18-DG uptake is often low grade (i.e. –– MRI and ultrasound are useful in
false negative) equivocal cases
–– Localised chest wall pain is a good
Imaging features clinical indicator of invasion
• Four recognised patterns of disease –– Chest wall invasion can be treated with
–– Solitary pulmonary nodule en-bloc resection
(commonest pattern) • T4: tumour invading a vertebral body or
–– Unifocal area of consolidation vital mediastinal structures such as the
mimicking pneumonia heart, trachea, great vessels or oesophagus
–– Multifocal areas of consolidation with a or a malignant pleural effusion or satellite
ground-glass appearance tumour nodules in the same lung as the
–– Multiple nodules, which may cavitate primary tumour
(‘cheerio’ sign) –– CT is unreliable for subtle mediastinal
invasion (65% accuracy)
Large cell carcinoma –– Suggestive CT findings include tumour
• Large (majority > 7 cm), highly malignant, or mediastinal contact > 3 cm and
bulky necrotic tumour tumour or vessel contact > 90% of
• Locally invasive with early hilar and circumference
mediastinal adenopathy –– Cytology analysis of aspirate from a
• Often widely disseminated with malignant pleural effusion gives false-
metastases at presentation negative results in around one third of
cases
Staging of non-small cell N staging
lung cancer • N1: ipsilateral positive hilar nodes
• Revised TNM staging system published in • N2: ipsilateral positive mediastinal nodes
2009 or subcarinal nodes
• Clinical staging uses information from –– Boundary between N1 and N2 nodes is
non-invasive techniques such as CT and the superior pulmonary vein
minimally invasive procedures such as –– Equivocal N2 nodes require PET
endoscopic ultrasound, bronchoscopy and scanning, endobronchial ultrasound
mediastinoscopy sampling or mediastinoscopy sampling
• Pathological staging uses findings from –– Bulky N2 nodes may be treated with
neoadjuvant therapy prior to surgery

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4 Cardiothoracic and vascular system

• N3: contralateral positive mediastinal or lesions and lesions contiguous with the
hilar nodes or supraclavicular or neck pleural surface
nodes • Reported diagnostic accuracy in the region
of 80–90% with an experienced operator
M staging • Core biopsy is more accurate than fine-
• M0: no distant metastases needle aspiration
• M1a: contralateral lung metastases,
pleural or pericardial dissemination PET–CT in non-small cell lung
• M1b: distant metastases cancer
• PET–CT using F-18-DG is routinely
Pancoast’s tumour performed in all patients considered
• Tumour of the superior pulmonary sulcus operable candidates on CT criteria alone
• Most commonly a squamous cell • Limited role in T staging (though it can
carcinoma help to delineate tumour from collapse)
• Tumour invades lower brachial plexus and • More accurate than CT for N staging
sympathetic chain because size criteria (short axis node
• Treatment is surgical resection if possible diameter > 1 cm) is unreliable
and radiotherapy • CT has a sensitivity of approximately 70%
for enlarged mediastinal nodes
Clinical triad • PET–CT has a sensitivity of approximately
90% for enlarged mediastinal nodes
• Ipsilateral Horner’s syndrome • PET–CT shows metastases in
• Ipsilateral medial arm pain approximately 20% of cases with a negative
• Wasting of small muscles of the hand CT
• PET–CT is more accurate than CT and
bone scintigraphy for extrathoracic disease
• MRI is superior to CT for staging including bone, adrenals, nodes and liver
Pancoast’s tumours
• Sagittal post-contrast T1-weighted Structures with normal PET uptake
imaging (T1WI) is sequence of choice for
assessing invasion into brachial plexus and • Myocardium
subclavian vessels • Brown fat
• Pancoast’s tumours are almost always • Thymus
associated with chest wall invasion • Strap muscles
• Ipsilateral supraclavicular nodes, nerve • Extra-ocular muscles
root invasion and rib destruction are all • Vocal cords (if talking during the
potentially resectable acquisition)
• Mediastinal nodes (N2) are considered
non-resectable

CT-guided lung biopsy


Lung metastases
• Ideally forced expiratory volume in
1 second (FEV1) should be > 1.5 L • Most metastases occur via haematogenous
• Performed using a co-axial technique spread, with tumour lodging peripherally
which minimizes the number of passes in the pulmonary capillaries
through the pleural • Majority are multiple
• Pneumothorax occurs in up to 40% of • Commonest primary sites are the breast,
cases. Risk is highest with small central colon, kidneys and head and neck

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Lung cancer 5

Cavitating metastases Lymphangitis carcinomatosis


• Permeation of pulmonary lymphatics by
Causes of cavitating metastases tumour cells
• Commonest primary tumours are
• Squamous cell carcinoma (commonest) bronchial, breast and stomach cancers
• Cervical carcinoma • Typically unilateral if from the bronchus
• Colorectal carcinoma and bilateral if from other sites
• Melanoma Imaging features
• Transitional cell carcinoma
• Sarcomas CXR
• Any tumour after chemotherapy • Often normal
• May see reticulonodular shadowing and
septal (Kerley B) lines
• Main differential diagnoses are pulmonary
Miliary metastases oedema and sarcoidosis
• Rare High-resolution CT (HRCT)
• Innumerable pulmonary nodules < 5 mm • Most sensitive imaging test
in diameter • Nodular interlobular septal thickening
• Indistinguishable from miliary (‘beaded septum’ sign)
tuberculosis • Nodular bronchovascular bundle
• Commonest primary tumours are the renal thickening
tumours, thyroid carcinoma, melanoma,
choriocarcinoma and bone sarcoma
Lymphoma
Calcified metastases
Hodgkin’s lymphoma
• Very rare except in osteosarcoma and
chondrosarcoma • Bimodal age peaks: 20–30 years and 70–80
• Osteosarcoma metastases are associated years
with pneumothorax • Presence of Reed–Sternberg cells (a type of
T cell) is the diagnostic hallmark
Other recognised primary sites • Nodular sclerosing subtype is the
commonest histology
• Spreads contiguously along the lymphatic
• Breast chain
• Thyroid • Commonly presents as non-tender
• Colon and rectum cervical lymphadenopathy
• Ovary • One third of patients present with fever
• Any tumour following chemotherapy (prolonged), weight loss and night sweats
• Alcohol-induced nodal pain is recognised
• > 80% have mediastinal adenopathy at
Endobronchial metastases presentation
• Lung parenchymal involvement is seen in
• Very rare
15% of cases; it is rare without adenopathy
• Seeding of tumour cells in the bronchial
• Thymic infiltration is seen in 30–50% of
submucosa
newly diagnosed Hodgkin’s lymphoma
• Cause airways obstruction and distal
collapse Non-Hodgkin’s lymphoma
• Commonest primary tumours are
• Four times commoner than Hodgkin’s
bronchial carcinoma, carcinoma of
lymphoma
the breast, lymphoma and colorectal
• 90% are of B cell origin
carcinoma
• Diverse group subdivided into low,
intermediate and high grade

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6 Cardiothoracic and vascular system

• Some types are associated with infectious • False positives from brown fat and from
agents, e.g. Helicobacter pylori and gastric inflammatory and infective conditions
mucosa-associated lymphoid tissue
(MALT)
Assessing initial response to therapy
• Increased risk in immunocompromised • Usually carried out after two or three
patients, e.g. post-transplant in a cycles of chemotherapy
lymphoproliferative disorder • A negative scan is a reliable indicator of
• Most present with lymphadenopathy and treatment response
systemic symptoms Post-therapy response
• One third present with extranodal • Performed after completion of
involvement, e.g. in the gastrointestinal chemotherapy course
tract • Unlike CT, PET–CT can distinguish a
• Potential for cure varies with histological fibrotic nodal mass from residual active
subtype and stage disease
• 40% have intrathoracic disease • A negative scan is associated with a low
• Lung parenchymal involvement is seen in risk of recurrence
5%, often without adenopathy • A positive scan carries a high risk of
recurrence
Parenchymal disease in Hodgkin’s • False-positive scans are seen with thymic
and non-Hodgkin’s lymphoma ‘rebound’ hyperplasia
• False-negatives scans are seen with
Wide range of imaging appearances granulocyte stimulating factor, which can
• Multifocal non-segmental cause intense marrow uptake, masking any
consolidation residual disease
• Reticulonodular infiltrates
• Multiple discrete pulmonary masses Long-term follow-up
• Pleural effusions • Higher sensitivity than CT and MRI in
assessing recurrent disease

Leukaemia
Lymphoma staging: Ann Arbor • Infiltration is common but is rarely
• Stage I: single lymph node region detected radiologically
involvement only • Leukostasis can obstruct the pulmonary
• Stage II: two or more lymph node regions vasculature in patients with high
on same side of diaphragm peripheral blast counts; this is seen as
• Stage III: lymph node regions (including pulmonary oedema
spleen) on both sides of diaphragm • More typically, lung pathology is related to
• Stage IV: extranodal involvement, e.g. neutropenic infection
bone marrow, liver, lung
• E: extranodal disease Castleman’s disease
• A: absence of constitutional symptoms • Rare group of post-viral
• B: presence of constitutional symptoms lymphoproliferative disorders
• X: bulky mass > 10 cm or mediastinal • Massive lymph node hyperplasia
widening more than one third of CXR • Large soft tissue mass, most often in the
mediastinum
Role of PET–CT in lymphoma • Highly vascular, with intense contrast
Diagnosis and staging enhancement
• > 90% sensitivity for detecting most types • Rarely contains areas of calcification
of lymphoma • 10% have disseminated disease with skin
• Less reliable with MALT and lymphocytic lesions, splenomegaly and lymphocytic
non-Hodgkin’s lymphoma interstitial pneumonitis

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Solitary pulmonary
1.2 nodule and congenital
lung disease

Solitary pulmonary nodule Clinical assessment


• A discrete pulmonary mass < 3 cm in • Only 1% of solitary pulmonary nodules in
diameter patients aged < 35 years are malignant
• Common incidental CXR finding • Smoking history greatly increases risk of
• Approximately 30% are malignant malignancy
• Common causes • If there is a history of smoking-related
–– Granuloma, e.g. tuberculosis, cancer, then a solitary pulmonary nodule
sarcoidosis (50%) is more likely a lung primary
–– Bronchogenic carcinoma (30%) • If there is a history of melanoma or
–– Hamartoma (10%) sarcoma, then a solitary pulmonary
• Other causes (10%) nodule is more likely a metastasis
–– Metastases Growth rate assessment
–– Bronchocele
–– Round pneumonia • Malignant lesions double in size between
–– Round atelectasis 30 days and 18 months
–– Fluid-filled abscess • Absence of growth over 2 years is reliable
–– Carcinoid tumour sign of a benign lesion
–– Arteriovenous malformation • Serial volume measurements are more
–– Progressive massive fibrosis accurate than diameter measurements
–– Developmental, e.g. sequestration,
bronchogenic cyst Three reliable signs of a benign
lesion
Morphological assessment
• 80% of nodules < 2 cm in diameter are • Fat density
benign • Benign calcification pattern
• Irregular, ‘spiculated’ margin suggests • No interval growth over 2 years
malignancy
• 80% of malignant nodules have well-
defined margins Imaging features
• Four recognised patterns of benign
PET
calcification: central nidus, diffuse solid,
• 85% of metabolically active solitary
laminated and ‘popcorn’
pulmonary nodules are malignant
• Amorphous calcifications seen in 6% of
• Standardised uptake value (SUV) > 3 is
lung cancers
used as a reference
• Intranodular fat (–40 to –120 HU) suggests
• High negative predictive value for lesions
a hamartoma
> 1 cm in diameter
• Benign cavitation typically has a smooth,
• False positives
thin wall
–– Tuberculosis
• Malignant cavitation typically has a thick,
–– Histoplasmosis
irregular wall
–– Sarcoidosis

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8 Cardiothoracic and vascular system

–– Progressive massive fibrosis Imaging features


–– Rheumatoid nodules
CXR
–– Intercurrent infection
• Well-circumscribed, lobulated pulmonary
• False negatives
nodule
–– Lesions < 1 cm in diameter
• 80% are located peripherally
–– Carcinoid tumour
–– Bronchoalveolar cell carcinoma CT
• 50% contain fat (–40 to –120 HU), which is
diagnostic
Carcinoid tumour • 30% contain ‘popcorn’ calcification, which
• Neuroendocrine neoplasms of the lung is diagnostic
(accounting for 1–2% of all lung cancers)
• Arise from Kulchitsky cells of the bronchial
mucosa
Arteriovenous
• Highly vascular tumours supplied by malformation
bronchial arteries • Capillary-free connection between
• 90% are ‘typical’ and of low-grade pulmonary arterial and pulmonary venous
malignancy systems
• 10% are ‘atypical’, with nodal and sclerotic • 70% are associated with Osler–Weber–
bone metastases Rendu syndrome
• 80% arise centrally, 20% are seen as a • 30% of patients with Osler–Weber–Rendu
peripheral solitary nodule syndrome have a pulmonary arteriovenous
• Present clinically with haemoptysis (in malformation
50% of cases) or recurrent pneumonia • Typically presents in middle age with
• Can cause carcinoid syndrome (in 2–5% of exertional dyspnoea and haemoptysis
cases) or Cushing’s syndrome (in 2%) • A recognised cause of finger clubbing
• Can cause carcinoid syndrome in the • Extracardiac right-to-left shunt with risk of
absence of liver metastases paradoxical embolism
Imaging features • Risk of transient ischaemic attack is 40%,
stroke 20% and brain abscess 10%
CXR or CT • Transarterial coil embolisation is
• Well-defined perihilar mass with treatment of choice if feeding artery
endobronchial component or well- measures > 3 mm in diameter
circumscribed parenchymal nodule • Post-embolisation syndrome can occur
• Atypical tumours may show mediastinal (pleuritic pain, atelectasis and fever)
invasion
• 30% contain calcifications Imaging features
F-18-DG PET CXR
• Absent or low-grade uptake • Small lesions, often not visible
• Hypervascularity may be the only sign
MIBG or indium-111 octreotide scan
• Well-defined lobulated oval mass
• More sensitive (60–90% sensitivity)
(multiple lesions in one third of cases)
• Band shadow connecting mass to hilum
Hamartoma • Medial third of lower lobe is commonest
location
• Commonest benign pulmonary neoplasm
• Decrease in size with Valsalva manoeuvre
• Composed of cartilage, epithelium and fat
and erect posture
• Peak incidence in fifth and sixth decades
• Associated with Carney’s triad: CT
gastrointestinal stroma tumour (GIST), • Enhancement of feeding artery and
paraganglioma, hamartoma draining vein
• Most exhibit slight growth if followed up • Rarely contains calcifications (phleboliths)
over many years

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Solitary pulmonary nodule and congenital lung disease 9

Congenital lung disease • Almost exclusively involves the right lung


• Adult form is most often an incidental CXR
Pulmonary agenesis, aplasia finding
and hypoplasia • Re-implantation of anomalous vein into
the left atrium is only considered if left-to–
Pulmonary agenesis right shunt fraction > 50%
• Complete absence of one or both lungs • Horseshoe lung is a rare variant in which
• No trace of bronchial or vascular supply or an isthmus of lung parenchyma extends
parenchymal tissue from the right lung base across the midline
• Simulates a pneumonectomy on CXR, with behind the pericardium and fuses with the
a small ipsilateral hemithorax, an elevated left lung base
hemidiaphragm and displacement of the
mediastinum towards affected side Imaging features
• CT confirms absence of ipsilateral CXR
pulmonary artery and bronchus • Small right hemithorax
Pulmonary aplasia • Rightward shift of heart and mediastinum
• Curvilinear tubular opacity coursing
• Absence of pulmonary vasculature and
inferiorly from the right hilum towards the
parenchyma
diaphragm (looks like a Turkish sword or
• There remains a rudimentary bronchus
scimitar)
• Similar CXR appearance to agenesis
• CT distinguishes from agenesis by showing
rudimentary bronchus Cystic adenomatoid
Pulmonary hypoplasia malformation
• An underdeveloped lung or lobe • Malformation of terminal respiratory
• Most commonly secondary to a space- structures affecting part or whole of one
occupying lesion (e.g. diaphragmatic lobe, sometimes two lobes, or even an
hernia) that stunts the growth of the lung entire lung
in utero • Communicates with the tracheobronchial
• Affected lung is small with ipsilateral tree
displacement of mediastinum • Supplied by a direct branch of the
pulmonary artery and drains into
Scimitar syndrome pulmonary veins
• Usually diagnosed in neonates and infants
• Encompasses a variety of congenital • Can rarely present in adulthood with
abnormalities of the thorax which occur in recurrent chest infections or haemoptysis
combination or (very rarely) malignant transformation
• Major components of scimitar syndrome (sarcomas and bronchoalveolar cell
–– Hypoplastic lung and pulmonary artery carcinoma have been reported)
–– Partial systemic arterial supply (aorta or
a branch) Imaging features
–– Partial anomalous pulmonary venous CXR
drainage • Multicystic parenchymal mass with air–
• Draining vein most often empties into fluid levels
infra-diaphragmatic inferior vena cava
(IVC)
• Hepatic veins, portal vein and right atrial
Pulmonary sequestration
drainage are described • Mass of non-functioning lung tissue
• Associated with a spectrum of pulmonary not in normal continuity with the
anomalies, including tracheal stenosis, tracheobronchial tree and receiving a
bilobed right lung and diaphragmatic systemic arterial supply
hernia

Ch-01.indd 9 8/7/2010 3:33:55 PM


10 Cardiothoracic and vascular system

• Almost always in lower lobes and more Imaging features


common in the left lung
CXR
• Divided into extralobular and intralobar
• Lower lobe soft tissue mass
types, depending on the pleural
• Air–fluid levels (especially if infected)
investment and pattern of venous
drainage: CT angiogram
• Enhancement of mass
Extralobar sequestrations • Demonstration of anomalous feeding
• Discrete accessory lobe of non-aerated artery
lung tissue invested in its own pleural
envelope Cystic fibrosis
• Majority are located immediately above
or below left hemidiaphragm and have • Autosomal-recessive pattern of
systemic venous drainage via the IVC or inheritance
the azygous venous system • Abnormally viscous secretions (elevated
• Commonly associated with other sweat test chloride level)
congenital anomalies • Recurrent respiratory tract infections and
• Presents in early childhood with shunt- bronchiectasis
induced respiratory distress • Bronchial wall thickening and mucus
plugging
Intralobar sequestrations • Bullous lung disease (in 20–30% of cases);
• Commoner than the extralobar type upper zone is predominantly affected
• Segment of non-functioning lung tissue, • Hyperinflation (in 80–90% of cases); lower
which is enclosed by the visceral pleura of zone is predominantly affected
an otherwise normal lung • Liver disease is the second leading cause
• Systemic arterial supply is usually via a of death in CF
single large artery arising from the lower • Viscous secretions cause biliary stasis and
thoracic or upper abdominal aorta, periductal fibrosis
though arterial supply from other arteries, • A small percentage progress to
including the coronary arteries, has been multinodular stage cirrhosis
described
Imaging features
• Venous drainage is via the pulmonary
veins into the left atrium HRCT
• Rarely associated with other congenital • Air trapping (‘mosaic attenuation’)
anomalies • Tree-in-bud appearance (mucoid
• Usually presents in adulthood with secretions or atypical mycobacterial
recurrent pneumonias in a persistent infection)
location, caused by of insufficient drainage • Bronchiectasis
• Haemoptysis is common as a consequence
of high pressure within the feeding artery

Ch-01.indd 10 8/7/2010 3:33:55 PM


1.3 Airways disease

Anatomy • Azygous vein arches over it from behind to


reach the superior vena cava (SVC)
Trachea • The right pulmonary artery lies anteriorly
• Cartilaginous tube lined by ciliated • The first branch of the right main bronchus
columnar epithelium is to the upper lobe: the eparterial
• Extends from lower border of the cricoid bronchus
cartilage (C6 level) to the carina (T5 level) • The right main bronchus continues as
• Passes downwards and slightly posteriorly, the bronchus intermedius (the interlobar
often deviating to right artery is lateral)
• Approximately 11 cm long and supported • The bronchus intermedius divides into
by around 20 incomplete cartilaginous middle and lower lobe bronchi
rings • The middle lobe bronchus arises opposite
• Trachealis muscle bridges the gap between the lower lobe apical segmental bronchus
these rings • Usually terminates as four basal segmental
• Oval in cross-section with a flattened bronchi
posterior margin
• Normal diameter < 23 mm in males and Left main bronchus
< 20 mm in females, measured at the level • Passes beneath the aortic arch
of aortic arch; diameter decreases with • The left pulmonary artery arches over it to
expiration lie posteriorly
• Diameter normally increases with age • The first branch of the left main bronchus
• Crossed by the left brachiocephalic vein, supplies both the upper lobe and the
aortic arch, left common carotid artery and lingula
innominate artery • The left main bronchus continues as the
• The right vagus nerve descends on its right lower lobe bronchus, from which arises
lateral aspect the lower lobe apical segmental bronchus
• The left recurrent laryngeal nerve ascends • Usually terminates as three basal
on its posterolateral aspect segmental bronchi
• Right-sided deviation on expiratory films is
normal in children
• Deviation to the left may be seen with a
Non-neoplastic tracheal
right-sided aortic arch disease
• Normal carinal angle is approximately 65°
(20° to the right and 45° to the left of the Tracheal bronchus
midline) • Anatomical variant whereby an anomalous
airway arises from the lateral wall of the
Right paratracheal stripe trachea
• Majority are right-sided and arise within
• Separates the trachea from the right 2 cm of the carina
lung • Prevalence of 0.1–2% (more common in
• Composed of paratracheal fat, lymph Down’s syndrome)
nodes, and visceral and parietal pleura • Displaced bronchus most often supplies
• Normally < 3 mm thick (thicker in the right apical segment; it less commonly
obese people) supplies all three upper lobe segments
• Most often an incidental finding; may
cause persisting lobar atelectasis in an
intubated patient despite an adequately
Right main bronchus positioned endotracheal tube on CXR
• Shorter (around 2 cm long), wider and
more vertical than the left main bronchus

Ch-01.indd 11 8/7/2010 3:33:55 PM


12 Cardiothoracic and vascular system

Tracheobronchomegaly • Pulmonary spread is even more unusual


(< 2%)
• Atrophy of elastic and smooth muscle
• Presents with hoarseness, inspiratory
fibres of the trachea and main bronchi,
stridor, wheezing, recurrent pneumonia
which become markedly dilated
• High-resolution CT shows diffuse airways
• Abrupt change to normal calibre at fourth
nodularity
or fifth bronchial divisions
• Poor prognosis secondary to airways
• Associated with Ehlers–Danlos syndrome,
compromise
Marfan’s syndrome, cutis laxa and
• Small risk of transformation into
ankylosing spondylitis
squamous cell carcinoma
• Can be acquired secondary to prolonged
• Pulmonary lesions are seen as multiple
mechanical ventilation and inhalation of
cavitating nodules
chemical irritants
• Most cases present in adult life with Relapsing polychondritis
chronic cough, excessive sputum
• Idiopathic episodic inflammation of
production, recurrent infections and
cartilaginous structures
expiratory stridor
• Predominantly affects the pinna, nose and
• Y-shaped tracheal stents are used to
upper airways
maintain airway patency
• Probably an autoimmune reaction against
Imaging features type III collagen
• Respiratory involvement occurs in 50% of
CT
cases
• Trachea and mainstem bronchial
–– Associated with a poor prognosis
diameters > 3 standard deviations from
–– Can be life-threatening requiring
mean
tracheostomy
• Tracheal diameter > 3 cm, right main
–– Causes tracheal thickening that spares
bronchus > 24 mm diameter, left main
the posterior wall and focal or diffuse
bronchus > 2 mm diameter
stenosis of the trachea or bronchi
• Tracheobronchial diverticulosis and cystic
• Non-erosive polyarthropathy is common
bronchiectasis
• Associations include aortic aneurysm,
• Collapse of central airways on expiration
aortic regurgitation and aortic dissection
Tracheobronchial stenosis • Nasal chondritis with saddle nose
deformity if long-standing
Causes
• Post-intubation or post-tracheostomy
(commonest cause)
Neoplastic tracheal disease
• Post-lung transplantation • Tracheal tumours account for 1% of all
• Tracheobronchial papillomatosis thoracic malignancies
• Tracheopathia osteoplastica • 90% of adult tracheal tumours are
• Tuberculosis, sarcoidosis or amyloidosis malignant
• Relapsing polychondritis or Wegener’s • Squamous carcinoma is the commonest
granulomatosis histological type
• Inflammatory bowel disease
• Post-traumatic, malignancy or idiopathic Squamous cell carcinoma
• Majority located in distal trachea within
Tracheobronchial 3 cm of the carina
papillomatosis • Presents with dyspnoea, cough and
• Infection of tracheobronchial tree by hoarseness
human papilloma virus • Poor prognosis: 50% have mediastinal
• Occasionally caused by aerial invasion at time of diagnosis
dissemination of laryngeal disease (5–10%) • Strong association with smoking

Ch-01.indd 12 8/7/2010 3:33:55 PM


Airways disease 13

Adenoid cystic carcinoma Churg–Strauss syndrome


(cylindroma)
• Slow growing malignant tumour, • Adult-onset asthma
commonest in proximal trachea • Peripheral blood eosinophilia
• Tendency for submucosal extension and • Systemic vasculitis
late recurrence
• Peak age is the fifth decade
• Equally common in males and females
• Better prognosis than squamous cell Chronic bronchitis
carcinoma • Chronic airways irritation from smoking
• Smoking is not a risk factor causes mucous gland hypertrophy and
hyper-secretion, and secondary infections
Imaging features maintain and promote the airways injury
CT • A clinical diagnosis: persistent cough with
• Four recognised patterns: sputum production for at least 3 months in
–– Intraluminal soft tissue mass with at least 2 consecutive years
extension through the tracheal wall
–– Diffuse or circumferential thickening of
Imaging features
the tracheal wall CXR
–– Soft tissue mass filling the trachea • Thick-walled, mildly dilated bronchi
–– Homogeneous mass encircling the • Accentuation of peripheral vascular
trachea markings (‘dirty chest’)
• Areas of hyperinflation from small airways
obstruction
Chronic obstructive
pulmonary disease Emphysema
• Includes asthma, chronic bronchitis and • Permanent enlargement of air spaces
emphysema distal to the terminal bronchioles, with
destruction of alveolar walls and the elastic
Asthma fibre network
• Chronic inflammatory disorder of the • Imbalance of elastase–anti-elastase
airways caused by hyper-reactivity to a activity within the lung
variety of stimuli • Tobacco smoke causes a neutrophil
• Smooth muscle and mucus gland influx, with release of elastase and
hypertrophy consequent proteolytic destruction of lung
• Airways obstruction, which reverses with parenchyma. Anti-elastase enzymes act to
bronchodilator agents limit the degree of destruction
• Complications include pneumothorax, Imaging features
pneumomediastinum and allergic
bronchopulmonary aspergillosis CXR
• Hyperinflation: flat diaphragms,
Imaging features retrosternal air space > 2.5 cm
CXR • Bullae: avascular radiolucent areas with
• Bronchial wall thickening in up to 50% of thin curvilinear walls
patients • Pulmonary hypertension: enlarged central
arteries and pruning
CT
• Bronchial wall thickening Scintigram
• Air trapping (more pronounced on • Delayed wash-in and delayed wash-out of
expiratory phase images) ventilation component

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14 Cardiothoracic and vascular system

HRCT Bronchiectasis
Three main patterns: centrilobular (the
• Irreversible dilatation of one or more
commonest), panlobular and paraseptal
bronchi
• Centrilobular pattern (commonest) • Presents with purulent sputum
–– Low-attenuation areas located production, recurrent chest infections and
centrally, within the second pulmonary haemoptysis
lobule
–– Spares the distal alveoli until late-stage Causes
disease • Acute or chronic necrotizing infection
–– Upper lobe predominance (commonest cause), e.g. tuberculosis,
• Panlobular pattern Mycobacterium avium-intracellulare
–– Low-attenuation areas throughout the complex
entire second pulmonary lobule • Congenital: cystic fibrosis, dyskinetic cilia
–– Strong association with alpha syndrome, Mounier–Kuhn syndrome,
1-antitrypsin deficiency hypogammaglobulinaemia
–– Lower lobe predominance • Allergic bronchopulmonary aspergillosis
• Paraseptal pattern • Pulmonary fibrosis ‘traction
–– Low-attenuation areas in the subpleural bronchiectasis’
regions • Bronchiolitis obliterans
–– Strong association with spontaneous • Yellow nail syndrome
pneumothorax • Foreign body
• Idiopathic (in 40% of cases)
Alpha-1 anti-trypsin deficiency
Dyskinetic cilia syndrome
• Autosomal-recessive inheritance (Kartagener’s syndrome)
• Most severe form is with the
homozygous PiZZ genotype • Structural abnormality of cilia and
• Deficiency of elastase inhibitor with spermatozoa
progressive panlobular emphysema • Recurrent sinus, ear and chest
• Disease process is accelerated by infections
smoking • Male infertility
• Patients typically present in their 40s • Triad: dextrocardia or situs inversus,
• Can also cause childhood hepatitis and sinusitis and bronchiectasis
cirrhosis

Imaging features
Operative management of CXR
emphysema • Dilated, thick-walled bronchi as seen end-
on (‘ring shadows’)
• Bullectomy • Dilated thick walled bronchi as seen in
• Excision of a large bulla or bullae to profile (‘tram lines’)
enable re-expansion of compressed
HRCT
adjacent lung
• Bronchial dilatation, with the internal
• Lung volume reduction surgery
diameter greater than the diameter of the
• Removal of severely emphysematous
adjacent artery
portions of lung
• Lack of peripheral bronchial tapering
• Outcome better with heterogeneous
(seen within 1 cm of the pleura)
disease and upper lobe predominance
• Lung transplantation is reserved for
end-stage disease

Ch-01.indd 14 8/7/2010 3:33:55 PM


Airways disease 15

Morphological patterns of Swyer–James syndrome


bronchiectasis
• Severe respiratory infection in
• Cylindrical: uniform bronchial dilatation childhood, in which damage of
• Varicose: beaded appearance (‘string of respiratory bronchioles causes
pearls’) incomplete development of the
• Cystic: central, clustered saccular alveolar buds
dilatations • Unilateral hyperlucent lung with
reduced volume on inspiration and air
trapping on expiration
• Matched perfusion and ventilation
Bronchiolitis obliterans defect on ventilation–perfusion (V/Q)
scan
• Airflow limitation caused by submucosal
inflammation or fibrosis of respiratory
bronchioles
• Affected areas are under-ventilated,
resulting in reflex vasoconstriction
Lobar anatomy
• Wide variation in normal appearance
Causes
• Post-infectious: respiratory syncytial Right lung
virus, influenza, Pneumocystis carinii
• Oblique fissure separates the upper and
pneumonia
middle lobes from the lower lobe
• Inhalation of toxic fumes, e.g. sulphur
• Oblique fissure runs at 50° to the
dioxide
horizontal from the T4 level posteriorly to
• Connective tissue disease, e.g. rheumatoid
diaphragm anteriorly
arthritis
• Only visceral pleura extends into oblique
• Lung or bone marrow transplantation
and horizontal fissures
Imaging features
Upper lobe
CXR • Divided into anterior, posterior and apical
• Usually normal, though may show mild segments
hyperinflation • Anterior segment lies adjacent to the
HRCT horizontal fissure
• Well-defined areas of reduced lung opacity
Middle lobe
(‘mosaic attenuation’)
• Divided into medial and lateral segments
• Reduced vessel calibre and number in
• Medial segment lies adjacent to right heart
low-attenuation areas
border
• Findings more prominent on expiratory
phase (because of air trapping) Lower lobe
• Bronchiectasis is commonly present as • Apical segment can extend as high as T3
well • Typically divided into four basal segments:
anterior, lateral, posterior and medial

Left lung
• Left oblique fissure runs more vertically
than the right, at 60° to the horizontal

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16 Cardiothoracic and vascular system

Upper lobe Posteroanterior


• Divided into apicoposterior and anterior
segments
radiographic appearances
of lobar collapse
Lingula
• Divided into superior and inferior Right upper lobe
segments • Elevation of the right hilum and horizontal
• Lies adjacent to the left heart border fissure
• Tracheal deviation to right
Lower lobe • Localized convexity at the right hilum
• Typically apical segment and three basal indicates an underlying mass lesion
segments: anterior, posterior and lateral (Golden’s ‘S’ sign)
Fissures and junctional lines Left upper lobe
• Horizontal fissure is seen in 50% of CXRs • Veil-like opacity in the left upper zone
• Azygous fissure is caused by failure of • Elevation of the left hilum
normal migration of the azygous vein, seen • Sharply demarcated aortic arch
as a ‘teardrop’ in 0.5% of CXRs (Luftsichel’s sign)
• Superior accessory fissure separates apical • Splaying of vessels from compensatory
segment of the right lower lobe from basal lower lobe expansion
segments, seen parallel and inferior to the
horizontal fissure in 5% of CXRs Middle lobe
• Inferior accessory fissure separates the • Depression of the horizontal fissure
medial basal segment of the right lower • Hazy opacity in right mid-zone
lobe from the basal segments, seen in 8% • Loss of clarity of the right heart border
of CXRs
Posterior junctional line
Lingula
• Loss of clarity of left heart border
• Normal landmark on CXR
• Posterior apposition of parietal and Left lower lobe
visceral pleura of both lungs
• Triangular opacity behind cardiac shadow
• Descends vertically from approximately T1
(sail sign)
to the level of the arch of the aorta
• Loss of clarity of the medial
• May occasionally form again below the
hemidiaphragm
arch
Anterior junctional line CXR
• Normal landmark on CXR
• Anterior apposition of parietal and visceral Conventional technique
pleura of both lungs as they come into • Focus–film distance of 180 cm
contact anterior to the arch of aorta • Centred at T5 level
Azygo-oesophageal line High-voltage technique
• Normal landmark on CXR
• Obscures fine rib detail
• Invagination of the right lung into the
• Improves mediastinal penetration
recess between the azygous vein and the
• Shorter exposure time
oesophagus
• Similar dose to conventional technique
• Begins below the clavicles and descends
(0.02 mSv)
inferiorly and to the left, ending at level of
• Air gap of 15 cm is used instead of grid to
right ventricle
reduce scatter

Ch-01.indd 16 8/7/2010 3:33:55 PM


Airways disease 17

• Better visualisation of some structures Pleural effusions


–– Lung vasculature
• Visible on a lateral decubitus X-ray when
–– Arch of the aorta
> 25 mL
–– Trachea and main bronchi
• Visible on a PA CXR when > 300 mL
–– Junctional lines
• More commonly left-sided in acute
• Worse visualisation of some structures
pancreatitis
–– Small pulmonary nodules
• More commonly right-sided in Meigs’
–– Pleural plaques
syndrome

Ch-01.indd 17 8/7/2010 3:33:56 PM


1.4 Air space diseases

Cardiogenic pulmonary • PCWP > 20 mmHg


–– Leakage of fluid into the interstitial
oedema spaces
• Impairment of blood flow through the –– Kerley B lines: perpendicular
left heart chambers causing elevated left subpleural linear opacities
atrial pressure and pulmonary venous –– Kerley A lines: longer opacities
hypertension radiating out from the hilum
• Causes include –– Peribronchial cuffing: thickened
–– Ischaemic heart disease oedematous bronchial walls
–– Arrhythmias, e.g. atrial fibrillation • PCWP > 25 mmHg
–– Mitral stenosis or mitral regurgitation –– Fluid spills over into the pleural spaces,
–– Aortic stenosis or aortic regurgitation fissures and alveoli
–– Systemic hypertension –– Pleural effusions (usually bilateral and
–– Constrictive pericarditis large)
–– Fluid in the horizontal and oblique
Non-cardiogenic pulmonary fissures
oedema –– Bilateral perihilar air space
consolidation (‘bats wings’)
• Defined as increased permeability
of the pulmonary capillaries in the Atypical patterns of pulmonary
presence of a normal left atrial pressure oedema
• Causes include adult respiratory
distress syndrome (ARDS), neurogenic • Unilateral: patient on side, re-expansion
oedema and drug reactions oedema
• Patchy: seen with emphysematous
bullae
• Right upper lobe: mitral regurgitation
Pathophysiology jet
• Raised pulmonary venous pressure
causes an imbalance between capillary
hydrostatic and plasma oncotic pressures
• Radiological changes correlate with left ARDS
atrial pressure
• This is measured indirectly via a Swan– • Life-threatening respiratory illness with
Ganz catheter as the pulmonary capillary 50% mortality
wedge pressure (PCWP) • Diffuse alveolar damage causes increased
• Normal PCWP is 8–10 mmHg capillary permeability
• Later, type 2 pneumocyte proliferation and
Imaging features interstitial fibrosis develops
Evolution of CXR changes • Severe respiratory failure develops 12–24
• PCWP > 15 mmHg hours after insult
–– Redistribution of blood flow to the • Patients require positive-pressure
upper zones ventilatory support
–– Seen on erect film as the diameter of • Mechanism of injury:
vessels in the upper zone being the –– Direct, from inhaled toxic fumes or
same as or greater than in the lower aspirated gastric contents
zone –– Indirect, e.g. sepsis, pancreatitis, burns,
hypovolaemia

Ch-01.indd 18 8/7/2010 3:33:56 PM


Air space diseases 19

Imaging features Goodpasture’s syndrome


Evolution of CXR changes • Autoimmune disease caused by antibodies
• 0–24 hours: normal against glomerular and alveolar basement
• 24–48 hours: widespread patchy air space membranes
consolidation; massive consolidation with • Pulmonary haemorrhage and
air bronchograms glomerulonephritis
• 3–14 days: slow resolution of air space
changes; fine reticular pattern (‘bubbly
Imaging features
lung’) CXR
• Multifocal consolidation with air
Features distinguishing ARDS from bronchograms
cardiogenic pulmonary oedema
Other causes of pulmonary–renal
• No pleural effusions syndrome
• No cardiomegaly
• Wegener’s granulomatosis
• IgA nephropathy
CT • Henoch–Schönlein purpura
• Early • Microscopic polyangiitis
–– Bilateral consolidation and ground- • Systemic lupus erythematosus
glass appearance
–– Predilection for gravity-dependent lung
• Late Mitral stenosis
–– Fibrosis (reticulation) and bronchial
dilatation • Chronic disease causing pulmonary
–– Predilection for non-gravity-dependent venous hypertension
lung • Frequently associated with atrial
–– Extent of fibrosis correlates with fibrillation due to left atrial enlargement
duration of ventilation • Intra-alveolar haemorrhage is well
recognised

Pulmonary haemorrhage Other lung manifestations of mitral


• Associated with a diverse range of stenosis
conditions
• Severity ranges from subclinical to massive • Pulmonary oedema
life-threatening haemoptysis • Pulmonary ossifications
• Imaging features are those of patchy air • Pulmonary haemosiderosis
space opacification
• Alveolar macrophages clear blood
products quickly, and resolution of
changes may be seen within a few days
Idiopathic pulmonary
(unlike infection) haemosiderosis
• Repeated episodes can induce a fibrotic • Rare cause of recurrent diffuse alveolar
response haemorrhage
• Causes include • Most frequent cause of diffuse alveolar
–– Goodpasture’s syndrome haemorrhage in children
–– Vasculitides, e.g. Wegener’s • Presents with recurrent episodes of
granulomatosis, systemic lupus haemoptysis
erythematosus • Associated with iron-deficiency anaemia
–– Drug, e.g. anticoagulants • Bronchoalveolar lavage shows
–– Mitral stenosis haemosiderin laden macrophages
–– Idiopathic pulmonary haemosiderosis • Fibrotic response may be seen with
recurrent episodes

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20 Cardiothoracic and vascular system

Imaging features Alveolar proteinosis


CXR • Idiopathic condition characterised by
• Patchy air-space opacities accumulation of periodic acid–Schiff
Cryptogenic organising (PAS)-positive phospholipid within the
alveoli and interstitial spaces
pneumonia • Caused by excess surfactant production
• Non-infectious illness with a subacute from type 2 pneumocytes
onset over several months • Antibodies against granulocyte
• Pathology shows small airways macrophage colony-stimulating factor
inflammation with buds of granulation (GM-CSF)
tissue filling the bronchioles, alveolar • Associated with lymphoma, leukaemia
ducts and alveoli and immunodeficiency
• Presents with dry cough, fever and • Typically presents in middle age with
dyspnoea progressive dyspnoea
• 50% of cases are idiopathic • Secondary infections are common,
• 50% have an identifiable cause especially with Nocardia
–– Drug reaction, e.g. amiodarone, • Runs a variable clinical course: one third
bleomycin, methotrexate recover, one third remain stable, and in
–– Connective tissue disease, e.g. one third it is fatal
rheumatoid arthritis • Treat options include regular whole-lung
–– Post-lung transplantation lavage
–– Radiotherapy injury
• Diagnosis is established via lung biopsy Imaging features
• Characteristically responds rapidly to CXR
corticosteroids • Bilateral symmetrical air space
opacification
Imaging features
• Mimics cardiogenic oedema but no
CXR cardiomegaly and no effusions
• Non-specific
• Multifocal patchy air space consolidation
HRCT
• ‘Crazy-paving’ pattern
HRCT –– Bilateral, patchy ground-glass opacities
• Non-specific –– Superimposed smooth, interlobular
• Patchy ground-glass opacities septal thickening
• Patchy bilateral air space consolidation

Causes of multifocal air space


consolidation

• Infection, e.g. aspergillosis


• Pulmonary oedema
• Pulmonary haemorrhage, e.g. from a
drug reaction
• Neoplasia, e.g. bronchoalveolar cell
carcinoma, lymphoma
• Eosinophilic pneumonia
• Crytogenic organising pneumonia
• ARDS
• Alveolar proteinosis
• Amyloidosis

Ch-01.indd 20 8/7/2010 3:33:56 PM


1.5 Diffuse lung disease

HRCT HRCT signs


Technical aspects Halo sign
• Modality of choice for assessing • Ground-glass attenuation surrounding a
parenchymal lung disease pulmonary nodule
• Narrow beam collimation (0.5–2 mm slice • Usually caused by haemorrhage into
thickness) surrounding lung
• Edge-enhancing high-spatial resolution
reconstruction algorithm
Causes
• Increased noise: poor for assessing • Angioinvasive aspergillosis
mediastinal structures • After biopsy or radiofrequency ablation
• Performed in suspended full inspiration • Haemorrhagic lung metastases (from
• Expiratory imaging is used for evaluation angiosarcoma or choriocarcinoma)
of air trapping • Kaposi’s sarcoma
• Prone imaging is used to distinguish • Wegener’s granulomatosis
dependent change from early fibrosis • Bronchoalveolar cell carcinoma
• Incremental HRCT • Eosinophilic pneumonia
–– Single slices at spaced intervals • Cryptogenic organising pneumonia
–– 10–15% of radiation dose of • Infection, e.g. tuberculosis, Mycobacterium
conventional chest CT avium-intracellulare complex, herpes
• Volumetric HRCT simplex virus, cytomegalovirus,
–– Contiguous spiral acquisition of entire coccidioidomycosis
thorax Mosaic attenuation
Secondary pulmonary lobule • Well-defined high- and low-attenuation
• Basic unit of lung structure marginated by areas in a lobular distribution
connective tissue septa • Three main causes: small airways disease,
• Polyhedral in shape and 1–2 cm in size vascular disease and parenchymal disease
(smaller centrally) Small airways disease
• Contains between 10 and 20 pulmonary • Causes include asthma, bronchiolitis
acini obliterans, cystic fibrosis and allergic
• Supplied centrally by a small bronchiole bronchopulmonary aspergillosis
and pulmonary artery, which are • Abnormal lung shows as lower-attenuation
surrounded by interstitial fibres and area
lymphatics • More pronounced on expiratory phase
• A peripheral connective tissue septa imaging
extends between the lobules and contains • Reduced vessel calibre and number
both veins and lymphatics
Vascular disease
Structures normally visible on • Chronic thromboembolic pulmonary
HRCT hypertension
• Centrilobular artery • Abnormal lung shows as lower-attenuation
• Bronchi (not normally seen within 2 cm of area (owing to the relative hypoperfusion
pleura) in these areas)
• Interlobular septa (only occasionally • Does not become more pronounced on
visible) expiratory phase imaging
• Not centrilobular bronchiole and not • Reduced vessel calibre and number in low
pulmonary acini attenuation regions

Ch-01.indd 21 8/7/2010 3:33:56 PM


22 Cardiothoracic and vascular system

Parenchymal disease ‘Crazy-paving’ pattern


• Caused by any condition that produces • Ground-glass opacity with superimposed
ground-glass attenuation septal thickening
• Abnormal lung shows as higher- • Causes
attenuation area –– Alveolar proteinosis
• Vessel calibre and number is normal –– Infections, e.g. Pneumocystis
carinii pneumonia, viral infection,
Ground-glass opacification mycoplasma infection, bacterial
• Hazy increase in lung opacity not infection
obscuring the vessels –– Pulmonary oedema, e.g. from heart
• Caused by alveolitis, partial air space failure or ARDS)
filling or interstitial disease below the –– Pulmonary haemorrhage, e.g.
spatial resolution of HRCT (partial volume Wegener’s granulomatosis,
effect) Goodpasture’s syndrome, systemic
Causes lupus erythematosus
• Pulmonary oedema (heart failure, ARDS) –– Bronchoalveolar cell carcinoma
• Pulmonary haemorrhage –– Non-specific interstitial pneumonia
• Usual interstitial pneumonia (UIP), –– Cryptogenic organising pneumonia
desquamative interstitial pneumonia –– Severe acute respiratory illness (SARS)
(DIP), non-specific interstitial pneumonia –– Lipoid pneumonia
(NSIP) or lymphocytic interstitial –– Sarcoidosis
pneumonitis (LIP) Centrilobular nodules
• Respiratory bronchiolitis associated with
interstitial lung disease (RB-ILD) Causes
• Infections, e.g. Pneumocystis carinii • Diseases involving the centrilobular
pneumonia, viral infection, mycoplasma bronchioles
infection –– Bronchiectasis
• Cryptogenic organising pneumonia –– Bronchiolitis obliterans
• Extrinsic allergic alveolitis –– Hypersensitivity pneumonitis
• Bronchoalveolar cell carcinoma –– Endobronchial spread of tuberculosis
• Sarcoidosis –– Bronchopneumonia
• Eosinophilic pneumonia –– Cryptogenic organising pneumonia
• Alveolar proteinosis –– Respiratory bronchiolitis associated
• Sickle cell disease with interstitial lung disease (RB-ILD)
–– Tracheobronchial papillomatosis
Interlobular septal thickening –– Diffuse panbronchiolitis
• Represents interstitial fluid, fibrosis or –– Bronchioalveolar cell carcinoma
cellular infiltration –– Histiocytosis X
• Causes of smooth septal thickening • Diseases involving the central perivascular
–– Interstitial pulmonary oedema lymphatics
–– Lymphangitis carcinomatosis (typically –– Sarcoidosis
causes unilateral interlobular septal –– Lymphangitis carcinomatosis
thickening) –– Lymphocytic interstitial pneumonia
–– Alveolar proteinosis • Diseases involving the centrilobular
–– Lymphoma arteries
• Causes of nodular septal thickening –– Pulmonary oedema
–– Lymphangitis carcinomatosis –– Vasculitis
–– Sarcoidosis
–– Pneumoconiosis Tree-in-bud pattern
–– Silicosis • Small, peripheral centrilobular nodules
–– Berylliosis of soft-tissue attenuation connected to

Ch-01.indd 22 8/7/2010 3:33:56 PM


Diffuse lung disease 23

multiple linear branching opacities • These changes are reversible with


• Represents plugging of small airways with cessation of exposure
mucus, pus or fluid
• Can also occur with intravascular
Imaging features
pulmonary tumour emboli CXR
• Not visible on CXR • Normal in 50% of cases
• Generalised haziness (ground-glass
Causes appearance) is common
• Infections, e.g. tuberculosis, • Multifocal consolidation is less common
Mycobacterium avium-intracellulare
complex, pyogenic infections, fungal HRCT
infections, cytomegalovirus, other • Multiple, small, ill-defined nodules
viral infections, Pneumocystis carinii (< 5 mm in diameter)
pneumonia • Centrilobular nodules and ground-glass
• Bronchiectasis (of any cause) appearance
• Bronchiolitis obliterans (of any cause) • Air trapping on expiratory phase imaging
• Diffuse panbronchiolitis • Mid-zone predominance
• Aspiration Chronic extrinsic allergic
• Allergic bronchopulmonary aspergillosis
• Connective tissue diseases, e.g.
alveolitis
rheumatoid arthritis, Sjögren’s syndrome • Prolonged (usually low-grade) exposure
• Intravascular tumour emboli, e.g. from causes pulmonary fibrosis, with insidious
breast or stomach onset of dyspnoea, type I respiratory
failure and cor pulmonale
Extrinsic allergic alveolitis Imaging features
• Immunologically mediated disorder HRCT
whereby inhalation of dust or other • Pulmonary fibrosis with mid-zone-
particulate antigens provokes a predominance
hypersensitivity reaction
• Includes pigeon fancier’s lung (caused by Sarcoidosis
avian proteins), farmer’s lung (caused by
spores of thermophilic actinomycetes) and • Idiopathic multisystem disease
malt worker’s lung (caused by Aspergillus characterised by widespread development
clavatus) of non-caseating epithelioid granulomas
• < 50% of patients have serum IgG • 90% of patients have pulmonary
antibodies against a provoking antigen involvement
• The presence of serum antibodies • Granulomas are distributed along the
indicates exposure and not necessarily lymphatics
disease • Most commonly presents in those aged
20–40 years
Acute and subacute extrinsic • More common in Afro-Caribbeans
allergic alveolitis Eponymous sarcoidosis
• Inhaled antigens reach the alveoli and
provoke an inflammatory cell infiltrate via
syndromes
a type III hypersensitivity reaction • Löfgren’s syndrome (20% of cases of
• Fever, rigors, cough and dyspnoea develop sarcoidosis)
6 hours after exposure and settle within –– Bilateral hilar lymphadenopathy
12–24 hours –– Erythema nodosum
• Repeated exposures cause a cell-mediated –– Arthralgia
(type IV) reaction, with granuloma • Heerfordt’s syndrome (5% of cases of
formation and obliterative bronchiolitis sarcoidosis)

Ch-01.indd 23 8/7/2010 3:33:56 PM


24 Cardiothoracic and vascular system

–– Anterior uveitis
–– Parotitis Causes of bilateral hilar
–– Eighth cranial nerve palsy lymphadenopathy

Investigations • Sarcoidosis
• Serum angiotensin-converting enzyme: • Lymphoma
elevated in 60%, and level correlates with • Metastases
granuloma burden • Tuberculosis
• Serum calcium levels: elevated in 10% • Silicosis
• Bronchoalveolar lavage: increased • Berylliosis
CD4:CD8 ratio
• Gallium-67 scintigraphy: uptake in nodes
and lung parenchyma
• Transbronchial lung biopsy: accuracy of
Parenchymal disease
90% • 40% have parenchymal disease at the time
of diagnosis
Staging at time of presentation • A further 40% develop parenchymal
• Stage 0: clear CXR (10% of cases) changes; this usually occurs within the
• Stage 1: hilar nodal enlargement (50% of first year and is classically accompanied by
cases) nodal regression
• Stage 2: hilar nodal enlargement and Micronodular parenchymal disease
pulmonary infiltrates (30% of cases)
• Accounts for 75% of cases
• Stage 3: pulmonary infiltrates alone (10%
• Mid-zone and upper zone predominance
of cases)
• Multiple small (< 5 mm), well-defined
• Stage 4: late-stage fibrosis
nodules
• The lower the stage at presentation the
• Nodules are located along
better the prognosis
–– Bronchovascular bundles
• 60% of patients with stage 1 disease
–– Fissures
undergo spontaneous remission
–– Subpleural regions
• Poor prognostic factors include male sex,
–– Interlobular septa (‘beaded septum
insidious-onset disease, lupus pernio,
sign’)
tracheal involvement and extrapulmonary
–– Centrilobular regions
manifestations
• Overall mortality 5–10% Air space parenchymal disease
• Accounts for 20% of cases
Lymphadenopathy • Multifocal bilateral, ill-defined air space
• Hilar, paratracheal and bronchopulmonary disease
nodes are most commonly involved • Air bronchograms
• Symmetrical, bilateral hilar adenopathy • Mid-zone and upper zone predominance
with a lobulated outer margin and well
demarcated inner margin is classical CXR Macronodular parenchymal disease
picture • Accounts for 5% of cases
• Degree of hilar adenopathy ranges from • Bilateral multiple, ill-defined opacities of
minimal to massive > 1 cm diameter
• Asymmetric and unilateral hilar • Mid-zone predominance
adenopathy are rare (< 5% of cases) • Rarely cavitated
• Adenopathy usually resolves within the
first year after diagnosis and almost never
Fibrotic stage (stage IV)
returns • Two thirds of parenchymal infiltrates
• Classically nodes develop light ‘eggshell’ resolve completely
calcifications: seen in 3% of cases after 5 • One third progress to pulmonary fibrosis
years and 30% after 10 years over several years

Ch-01.indd 24 8/7/2010 3:33:56 PM


Diffuse lung disease 25

• Fibrosis is usually predominant in the mid- • Enlarged ‘reactive’ mediastinal


zone and upper zone lymphadenopathy is common
• Bullae, cysts and traction bronchiectasis • Pleural effusion is recognised but rare
are common • Ground-glass appearance is thought to
• Mycetoma formation in upper lobe bullae represent active alveolitis, which may be
is well recognised corticosteroid-responsive in some cases
• Honeycombing represents irreversible
Other thoracic manifestations established fibrosis
• Pleural effusion (in 2% of cases)
• Bronchial stenosis (in 2% of cases)
Connective tissue diseases
Cryptogenic fibrosing Rheumatoid arthritis
alveolitis • Symmetrical deforming polyarthropathy of
peripheral joints
• Idiopathic pulmonary fibrosis with • IgM rheumatoid factor-positive
progressive dyspnoea, pulmonary • Extra-articular manifestations include a
hypertension and cor pulmonale range of chest conditions
• Typical onset is in late middle age with
mean survival of 3 years Pleural effusion
• Finger clubbing and bilateral basal end- • Males nine times more commonly affected
inspiratory crackles than females
• Commonest histological appearance is • Associated with the presence of
usual interstitial pneumonia (UIP) subcutaneous rheumatoid nodules
–– Parenchymal inflammatory cell • Usually develops years after onset of the
infiltrates, fibroblastic foci and areas rheumatoid arthritis
of established fibrosis (temporal • Typically small, unilateral and painless
heterogeneity) • Exudate
• Increased risk of bronchogenic carcinoma –– Low glucose
–– Low pH
Hamman–Rich syndrome –– Lymphocyte-predominant
–– May contain rheumatoid factor
• Most resolve spontaneously
• Acute form of cryptogenic fibrosing
alveolitis seen in 10% of cases Interstitial fibrosis
• Rapidly fatal, within a few months • Males twice as commonly affected as
• Histologically there is diffuse alveolar females
damage • 20% of patients with rheumatoid arthritis
• Multifocal air space consolidation develop it
• Background fibrotic and ground-glass • Lower-zone predominant
change • Commonest histological pattern is usual
interstitial pneumonia (UIP); it can mimic
cryptogenic fibrosing alveolitis
Imaging features
Pulmonary nodules
CXR • Pathologically the same as subcutaneous
• Reticulonodular shadowing in the lower nodules
zone • Males twice as commonly affected as
HRCT females
• Ground-glass opacity in a peripheral basal • Typically lobulated soft-tissue masses of
subpleural distribution 1–5 cm diameter
• Reticulation with honeycombing and • Peripheral zone and mid-zone
traction bronchiectasis predominance

Ch-01.indd 25 8/7/2010 3:33:56 PM


26 Cardiothoracic and vascular system

• 50% cavitate Polymyositis


• Rarely calcify
• Inflammatory proximal myopathy
Caplan’s syndrome • Raised creatine kinase
• Development of pulmonary nodules • Electromyograph abnormalities
indistinguishable radiologically from • 5% of patients have anti-Jo-1 antibodies,
rheumatoid nodules in patients with coal which are associated with lower zone
worker’s pneumoconiosis and rheumatoid pulmonary fibrosis
arthritis • Dermatomyositis has the features of
• Background micronodular change polymyositis plus skin involvement
suggests the diagnosis • Dermatomyositis is associated with an
underlying malignancy
Other chest manifestations of
rheumatoid disease Scleroderma
Limited form of scleroderma
• Bronchiectasis • Formerly called the CREST syndrome:
• Bronchiolitis obliterans calcinosis, Raynaud’s phenomenon,
• Drug toxicity, e.g. methotrexate oesophageal dysmotility, sclerodactyly,
pneumonitis telangiectasias
• Anti-centromere antibodies are present
Diffuse form of scleroderma
Systemic lupus erythematosus • 90% develop lung fibrosis; non-specific
• Multisystem autoimmune disease interstitial pneumonia (NSIP) is the
• Anti-double-stranded DNA antibody- commonest pattern
positive • Increased incidence of bronchoalveolar
• Females affected ten times more cell carcinoma and adenocarcinoma
commonly than males • Pulmonary arterial hypertension
• Can be drug-induced by hydralazine, • Gastrointestinal tract involvement (dilated
phenytoin or procainamide small bowel ‘hidebound’)
• Antiphospholipid antibody and • Acute renal failure and malignant
prothrombotic tendency occur in 30% of hypertension secondary to an endarteritis
patients obliterans
• Butterfly facial rash, photosensitivity, • Anti-Scl-70 antibodies are present
Raynaud’s phenomenon, non-erosive
arthropathy, renal impairment, Liebmann–
Imaging features
Sachs endocarditis CXR
• Pulmonary disease is seen in 60% of • Bilateral lower zone pulmonary fibrosis
patients • Dilated oesophagus with air–fluid level
• Apical surgical clips from sympathectomy
Pulmonary manifestations of • Dilated sub-diaphragmatic small bowel
systemic lupus erythematosus loops
• Spontaneous pneumoperitoneum
• Pleural effusions (common)
• Multifocal consolidation: vasculitic Pulmonary vasculitides
haemorrhage, atypical infection or
lupus pneumonitis Wegener’s granulomatosis
• Lower zone pulmonary fibrosis in < 5% • Necrotizing vasculitis of small and
of patients medium-sized arteries
• Diaphragmatic elevation from • Positive for antineutrophil cytoplasmic
myopathy antibodies (cANCA), specifically
proteinase-3 (PR3)

Ch-01.indd 26 8/7/2010 3:33:56 PM


Diffuse lung disease 27

• Multisystem disorder • Other features


• Males twice as commonly affects as –– Associated with HLA-B51
females –– Most prevalent in Turkish men
–– Positive pathergy reaction (pustule at
Pulmonary disease site of a needle puncture)
• Occurs in 95% of cases –– Arterial and venous thromboses
• Multiple large (> 2 cm), peripheral –– Lung involvement in 5% of cases:
nodules, which often cavitate pulmonary artery aneurysms,
• Pulmonary haemorrhage, producing pulmonary infarcts and pulmonary
multifocal air space consolidation haemorrhage
• Tracheobronchial inflammation, causing
subglottic tracheal stenosis
• Pleural effusion occurs in 25% cases, Drug-induced lung disease
lymphadenopathy occurs but is rare • Numerous agents have the potential to
Renal disease cause pulmonary toxicity
• Wide range of histopathological processes
• Occurs in 90% of cases
including diffuse alveolar damage (DAD),
• Rapidly progressive glomerulonephritis
non-specific interstitial pneumonia
• Commonest cause of death
(NSIP), cryptogenic organizing pneumonia
Nasal and paranasal sinus disease (COP), eosinophilic pneumonia and
• Occurs in 80% of cases pulmonary haemorrhage
• Bloody sinus discharge, epistaxis and • Fibrosis in a basal distribution is a typical
collapse of nasal septum late-stage manifestation
• Clinically, cytotoxic drugs are the largest
Microscopic polyangiitis and most important group of causative
• Small vessel vasculitis agents
• Positive for perinuclear antineutrophil
cytoplasmic antibody (p-ANCA), Agents commonly causing
specifically myeloperoxidase (MPO) pulmonary drug toxicity
• Pulmonary haemorrhage, which produces
multifocal consolidation Cytotoxic agents
• Methotrexate
Churg–Strauss syndrome • Bleomycin
• Triad of clinical features • Cyclophosphamide
–– Adult onset asthma • Doxorubicin
–– Peripheral blood eosinophilia Non-cytotoxic agents
–– Systemic vasculitis • Amiodarone
• Nitrofurantoin
Imaging features
• Sulfasalazine
CXR
• Transient pulmonary infiltrates
HRCT Amiodarone-induced lung
• Patchy, multifocal ground-glass opacities disease
• Peripheral ill-defined areas of
• Tri-iodinated benzofuran with a long half-
consolidation
life (60 days)
Behçet’s disease • 5–15% of patients taking amiodarone
develop pulmonary toxicity
• Triad of clinical features
• If detected early, the prognosis is good
–– Oral ulceration
following discontinuation
–– Genital ulceration
• Drug metabolites are trapped by foamy
–– Iritis
macrophages, inducing a fibrotic reaction

Ch-01.indd 27 8/7/2010 3:33:57 PM


28 Cardiothoracic and vascular system

Imaging features bundles, which occlude the lymphatics,


blood vessels and bronchioles
CXR and HRCT
• Distal air trapping causes cyst formation
• Areas of peripheral high-attenuation
and progressive destruction of lung tissue
consolidation
• Subpleural cysts may rupture, resulting in
• Pleural inflammation and effusion are
pneumothorax
common
• Exclusively affects women, usually during
• Ground-glass opacities with septal
the reproductive years
thickening progressing to established
• A small percentage of cases are associated
fibrosis in chronic stage (basal
with tuberous sclerosis
predominance)
• High attenuation may also be seen within Imaging features
the liver and thyroid
CXR
• Normal or increased lung volumes
Patterns of • Diffuse reticulonodular shadowing
pulmonary fibrosis (caused by superimposition of cyst walls)
• Pneumothorax develops in approximately
• Predominantly upper zone 70% of cases
–– Sarcoidosis • Pleural effusion (chylous, unilateral)
–– Silicosis develops in approximately 20% of cases
–– Coal worker’s pneumoconiosis
–– Histiocytosis X
CT
• Multiple thin-walled cysts (most 2–5 mm
–– Allergic bronchopulmonary
in diameter)
aspergillosis
• Typically cysts are round or polygonal in
–– Radiation fibrosis
shape
–– Tuberculosis
• Symmetrical, uniform distribution
• Predominantly lower zone
• Lung parenchyma between the cysts is
–– Rheumatoid lung disease
normal
–– Asbestosis
–– Scleroderma Pulmonary Langerhans’ cell
–– Cryptogenic fibrosing alveolitis
–– Neurofibromatosis type 1
histiocytosis
–– Drug-induced lung disease • Isolated form of histiocytosis X
–– Extrinsic allergic alveolitis (in the mid- • Proliferation of atypical Langerhans’ cells
zone and lower zone) within lung parenchyma
• Presents in young adults; 90% are smokers
• Imaging features evolve and have a
Interstitial lung diseases temporal heterogeneity
with preserved lung • Shows a mid-zone and upper zone
predominance
volumes
• Virtually all interstitial lung diseases cause Imaging features
a progressive reduction in lung volume, CXR
with the exception of four conditions, in • Often indistinguishable from
which volumes are preserved or increased lymphangioleiomyomatosis
–– Lymphangioleiomyomatosis • Normal or increased lung volumes
–– Pulmonary Langerhans’ cell • Diffuse reticulonodular shadowing
histiocytosis • Pneumothorax develops in approximately
–– Tuberous sclerosis 25% of cases
–– Neurofibromatosis type 1 HRCT
Lymphangioleiomyomatosis • Bilateral small centrilobular nodules
(1–10 mm in diameter)
• Proliferation of interstitial smooth muscle

Ch-01.indd 28 8/7/2010 3:33:57 PM


Diffuse lung disease 29

• Irregularly shaped, thick- and thin-walled • Two main patterns of pulmonary disease,
cysts tracheobronchial and parenchymal,
• Pneumothorax develops in 25% of cases as well as a third form, reticulonodular
• Fibrosis seen with late-stage disease infiltration
• Tracheobronchial form
Features suggesting Histiocytosis X –– Submucosal deposits are commonest
over lymphangioleiomyomatosis pulmonary form
–– Circumferential nodular luminal
• Spares lung bases narrowing with distal collapse
• Cysts have irregular shapes • Parenychymal form
• Nodules seen in parenchyma between –– Single or multiple pulmonary nodules
cysts mimicking malignancy
–– Larger lesions may cavitate or calcify
• Reticulonodular infiltration
–– Hilar and mediastinal adenopathy,
Tuberous sclerosis which often calcifies
• Lung involvement in 1–2% of cases
• Radiologically indistinguishable from
lymphangioleiomyomatosis
Alveolar microlithiasis
• Progressive, widespread calcium
Neurofibromatosis type 1 deposition within the alveoli
• Interstitial lung changes seen in 20% of • Serum calcium levels are normal
cases • Often minimal early symptoms; an
incidental imaging finding
Imaging features
• Many cases present in middle age with
CXR slowly progressive dyspnoea and eventual
• Pulmonary fibrosis with a lower zone cor pulmonale from secondary fibrosis
predominance
• Asymmetric bullae with an upper zone Imaging features
predominance CXR
• ‘Twisted ribbon’ ribs • Scattered, dense micronodules
• High thoracic-curve scoliosis (‘sandstorm’ appearance)
• Paraspinal neurofibromas and lateral • Mid-zone and lower zone predominance
meningocele
Scintigram
• Subcutaneous neurofibromas
• Nodules show intense uptake on bone
scan
Amyloidosis
• Extracellular deposition of an insoluble Radiation-induced lung
fibrillar protein disease
• Takes up Congo red stain
• Amyloid light chain (AL) form • Common: occurs in up to 10% of patients
–– Derived from plasma cells and elevated receiving radiotherapy
in multiple myeloma • Radiographic changes are usually confined
• AA – Amyloid-associated protein (AA) to the radiation portal
form • Rarely produces more diffuse changes
–– Synthesised in the liver and elevated in secondary to organising pneumonia
chronic inflammatory states • Tangential beam commonly used for
• AL form is predominant type to affect the breast cancer typically causes a 2–3 cm
lung strip of anterolateral fibrosis

Ch-01.indd 29 8/7/2010 3:33:57 PM


30 Cardiothoracic and vascular system

• Sharply defined linear interface with


Risk factors adjacent normal lung tissue
• Pleural effusions are uncommon
• Rarely occurs with doses < 20 Gy • Changes may resolve completely but most
• Almost always occurs with doses progress to fibrosis
> 40 Gy
• Concomitant chemotherapy increases Chronic radiation-induced
risk lung disease
• Corticosteroid cover reduces risk • Fibrosis develops 6–12 months after
treatment
Imaging features
Acute radiation-induced lung
CXR
disease • Volume loss
• Transient pneumonitis develops 1–3 • Linear scarring
months after treatment • Traction bronchiectasis
• Most patients are asymptomatic at this • Sharply defined linear interface with
stage adjacent normal lung tissue
Imaging features PET
CXR • Accurately detects residual or recurrent
• Non-segmental patchy consolidation and tumour within fibrosis
ground-glass changes

Ch-01.indd 30 8/7/2010 3:33:57 PM


1.6 Occupational lung disease

Silicosis and coal worker’s • Commences in lung periphery and


migrates towards hila
pneumoconiosis • Mid-zone and upper zone predominance
• Caused by inhalation of various inorganic • Typically bilateral and cavitating
dusts
• Particles lodge in respiratory bronchioles
Berylliosis
• Silicosis caused inhaled dust containing • Chronic exposure incites a granulomatous
silicon dioxide parenchymal reaction, which is
• Coal worker’s pneumoconiosis caused by indistinguishable from sarcoidosis
inhaled coal dust particles • Widespread micronodular shadowing,
• Usually asymptomatic unless complicated progressing to fibrosis
by massive fibrosis • Hilar and mediastinal lymphadenopathy
• Significant overlap in radiological
appearances Asbestos-related lung
Imaging features disease
CXR • Inhaled fibres are trapped in the lung,
• Micronodular opacities in the mid-zone inciting a fibrogenic reaction
and upper zone • Amphibolic fibres (crocidolite and
• Pulmonary fibrosis in the upper zone amosite) are the main cause of clinically
• Hilar adenopathy with ‘eggshell’ significant asbestos-related disease
calcifications (in 5% of cases of silicosis;
seen in coal worker’s pneumoconiosis only Pleural plaques
if the coal dust contains silica particles) • Commonest radiological manifestation of
HRCT asbestos exposure
• Well-defined centrilobular micronodules • Irritant effect from transpleural migration
of asbestos fibres
Causes of hilar ‘eggshell’ • Usually occurs 20–30 years after exposure
calcifications • Clinically asymptomatic
• Discrete areas of fibrosis that usually arise
from parietal pleura
• Silicosis
• Visceral pleural plaques are less common
• Sarcoidosis
• Most often located along posterolateral
• Scleroderma
chest wall and diaphragm
• Histoplasmosis
• Typically spare the apices and
• Amyloidosis
costophrenic angles
• Tuberculosis
• Calcification occurs in 10–15% of cases
• Berylliosis
• CT is more sensitive than CXR for plaque
• Lymphoma (post-treatment)
detection
• Plaques have no relationship with
mesothelioma
Progressive massive fibrosis
• Late complication of coal worker’s Causes of pleural calcification
pneumoconiosis or silicosis
• Can cause severe dyspnoea and • Asbestos-related pleural plaques
respiratory failure • Talc pleurodesis
• Large conglomerate fibrotic masses (no air • Haemothorax
bronchograms) • Tuberculous empyema

Ch-01.indd 31 8/7/2010 3:33:57 PM


32 Cardiothoracic and vascular system

Pleural effusion • Round or oval subpleural opacity forming


acute angle with pleura
• Earliest manifestation of asbestos exposure
• A history of asbestos exposure is common
(within 10 years)
(in 70% of cases)
• Benign haemorrhagic exudate of mixed
• Other causes include parapneumonic
cellularity
effusions, pneumothorax, uraemia,
• Usually small (< 500 mL) and
Dressler’s syndrome, mesothelioma and
asymptomatic
cardiac failure
• May be unilateral or bilateral
• Resolves over several months to leave Imaging features
residual pleural thickening • Most commonly located posteriorly within
the lower lobes
Diffuse pleural thickening • Comet tail sign: crowding of bronchi and
• Definition: smooth, uninterrupted pleural vessels entering lesion
thickening (> 5 mm) that extends over • Shows enhancement post-contrast
at least 25% of the chest wall (50% if • Air bronchograms seen within it in 60% of
unilateral), or continuous sheet of pleural cases
thickening (> 3 mm) that extends more
than 8 cm in craniocaudal extent and more Asbestosis
than 5 cm laterally • Caused by prolonged heavy exposure with
• Fibrosis of visceral pleura with fusion of a lag of > 20 years
the pleural layers • Progressive dyspnoea and a restrictive
• Usually preceded by a benign pleural ventilatory defect
effusion • Finger clubbing and basal end inspiratory
• Progressive restrictive ventilatory defect crepitations are typical
• Features helping to distinguish it from • Increased risk of developing bronchogenic
pleural plaques carcinoma
–– Ill-defined irregular margins • Basal subpleural curvilinear opacities are
–– Involvement of interlobar fissures the earliest sign
–– Lack of sparing of the costophrenic • Established disease appears similar to
angles or apices fibrosing alveolitis with predominantly
–– Calcification rare lower zone and mid-zone pulmonary
fibrosis
Causes of diffuse pleural thickening
Bronchogenic carcinoma
Benign • Exposure to asbestos increases the risk of
• Asbestos-related developing bronchogenic carcinoma 20-
• Tuberculous empyema fold
• Haemothorax • Synergistic effect with smoking: risk is
• Talcosis increased 100-fold with both risk factors
• Connective tissue diseases • Amphibole fibres are more potent
Malignant inducers than chrysotile
• Mesothelioma • Adenocarcinoma and squamous cell
• Metastatic adenocarcinoma carcinoma are commonest tumour types
• Lymphoma • Tumours are usually located peripherally
and in the lower lobes

Malignant mesothelioma
Round atelectasis • Malignant pleural tumour, which slowly
• Peripheral lobar collapse adjacent to an encases the lung
area of pleural thickening

Ch-01.indd 32 8/7/2010 3:33:57 PM


Occupational lung disease 33

• Commonest primary neoplasm of the Imaging features


pleura
CXR
• Accounts for < 1% of all thoracic
• Unilateral pleural effusion is commonest
neoplasms
finding
• Latent period between exposure and
• Often there is an absence of mediastinal
disease is 30–40 years
shift (‘frozen hemithorax’)
• Most patients die within 1 year of diagnosis
• Tumour can arise from either pleural layer CT
• Frequently extends into the interlobar • Markers of prior asbestos exposure are
fissures, chest wall, mediastinum, often absent
diaphragm and underlying lung • Lobulated pleural based soft tissue mass
• Haematogenous spread to involve liver encasing the lung
and lung is recognised • Mediastinal lymphadenopathy is common
• Can spread along the needle track of a PET/CT
percutaneous biopsy • Circumferential pleural uptake is
• Mesothelioma can also arise from the characteristic
peritoneum, pericardium and tunica • Most accurate modality for detecting
vaginalis of the testis mediastinal nodal disease

Ch-01.indd 33 8/7/2010 3:33:57 PM


1.7 Pulmonary infections

Community-acquired Klebsiella pneumonia


pneumonias • Rare cause of community-acquired
pneumonia
Streptococcal pneumonia • Typically affects elderly patients and
• Commonest community-acquired alcoholics
pneumonia • Classical CXR appearance is of a cavitating
• Risk factors include post-splenectomy, upper lobe consolidation with bulging of
alcoholism, immunocompromise the adjacent fissure
• Infection begins in distal air spaces (lobar • Commonly progresses to empyema
pneumonia) • Mortality is 30%
• Typically unifocal peripheral consolidation
with air bronchograms
Severe acute respiratory
• Resolves promptly with antibiotic syndrome (SARS)
treatment • Transmissible respiratory illness caused by
• Only 1–2% of patients develop an a coronavirus
empyema • Incubation period is 2–10 days
• Presents with high fever, dry cough,
Viral pneumonia dyspnoea and arthralgia
• Account for 10–15% of community- • 10–20% of patients require mechanical
acquired pneumonia ventilation
• Influenza is the commonest virus affecting
adults Imaging features
• CXR may show a fine reticulonodular • Extent of disease is underestimated with
infiltrate CXR
• Secondary bacterial infection is common • HRCT shows a mixture of ground glass and
(especially with Staphylococcus aureus) consolidation
• 80% have air space opacification of varying
Varicella-zoster virus extent at presentation
• Complicates chickenpox in 5% of adults • A peripheral lower zone focal opacity is
(rare in children) commonest pattern (40%)
• Widespread nodular opacities appear 1–5 • Extent of consolidation peaks 6 days after
days after the skin rash presentation and in those who recover has
• These nodules occasionally persist and resolved by day 16
some eventually calcify • Diffuse consolidation (14%) is associated
with a high mortality
Causes of bilateral calcified lung • Cavitation, lymphadenopathy, pleural
nodules effusions are not a feature

• Varicella pneumonia Atypical pneumonias


• Histoplasmosis
• Tuberculosis Mycoplasma pneumonia
• Mitral stenosis • Common cause of non-bacterial
• Alveolar microlithiasis pneumonia in young adults
• Metastases (from osteosarcoma, • Presents initially with flu-like symptoms,
mucinous adenocarcinoma, papillary which are followed by fever and dry cough
thyroid tumours, testicular tumours,
ovarian tumours or angiosarcoma)

Ch-01.indd 34 8/7/2010 3:33:57 PM


Pulmonary infections 35

Imaging features Pseudomonas pneumonia


CXR • Infection develops in patients with pre-
• Severity of findings are discrepant to existing lung disease
clinical picture • Delayed clearance of secretions
• Reticulonodular opacities radiate out from predisposes to colonisation
hilum into lower lobe • A common cause of recurrent pneumonia
• Progresses to confluent consolidation, in cystic fibrosis
which is bilateral in 40% of cases • Difficult to eradicate because of
• Pulmonary fibrosis and bronchiolitis widespread drug resistance
obliterans are recognised • Multifocal consolidation on a background
of chronic lung disease
Extrapulmonary complications
• IgM cold agglutinins, causing haemolytic
anaemia Aspergillosis
• Erythema multiforme and Stevens– • Aspergillus fungus causes a broad
Johnson syndrome spectrum of disease in humans, ranging
• Meningoencephalitis from hypersensitivity reaction to
• Myocarditis aggressive angioinvasion
Legionella pneumophila Aspergilloma
• Organism colonises air-conditioning • Colonisation of a chronic lung cavity, e.g.
systems and humidifiers in tuberculosis or sarcoidosis
• Acquired by inhalation of the aerosol mist • Non-invasive process
• Mainly affects older adults, with a 20% • Most often occurs in lung apex
mortality • Can cause severe haemoptysis requiring
• Patients can be critically ill with renal and bronchial embolisation
hepatic failure
• CXR is non-specific with areas of Imaging features
consolidation CXR
• Thin-walled cavity containing a rounded
Other pneumonias gravity-dependent opacity
• Air crescent sign between the opacity and
Aspiration pneumonia the cavity wall
• Caused by irritant effect of gastric contents • Fungal ball may contain calcifications
combined with anaerobic infection
• Risk factors include reduced Glasgow
Invasive aspergillosis
coma scale, stroke and gastroesophageal • An often fatal infection in the severely
reflux disease immunocompromised
• Affects the superior segment of the lower • Angioinvasion occurs, with haemorrhage
lobes and the posterior segment of the and lung infarction
upper lobes • Mortality is 60%
• Often progresses to abscess formation and Imaging features
empyema
CXR
Staphylococcal pneumonia • Multiple, ill-defined patches of
• Occurs as a superinfection following consolidation
influenza virus or from septic emboli from • Lesions may cavitate in later stages of
tricuspid valve endocarditis related to disease
intravenous drug use HRCT
• Consolidated areas have a propensity to • Multiple nodules with surrounding
cavitate ground-glass halos

Ch-01.indd 35 8/7/2010 3:33:57 PM


36 Cardiothoracic and vascular system

• Lesions progress into more diffuse Hydatid disease


consolidation
• Principally caused by ingestion of
Allergic bronchopulmonary Echinococcus tapeworm eggs excreted in
aspergillosis the faeces of infected dogs
• Commonest cause of pulmonary • The eggs hatch in the small bowel and
eosinophilia in the UK penetrate the intestinal wall to reach the
• Airway colonisation in patients with portal venous system, spreading primarily
asthma or cystic fibrosis to liver
• Recurrent hypersensitivity reactions with • In the liver they form cystic lesions, which
eosinophil influx grow slowly over many years
• Positive Aspergillus serum precipitins and • Lung is involved in 5–15% of cases (with
elevated IgE levels multiple lesions in 30% of these cases)
• Causes progression of asthma symptoms • Most patients are asymptomatic
• Expectoration of thick mucus plugs Imaging features
• Complications of recurrent attacks • Seen as a well-defined, round or oval mass
–– Central mucus plugging with distal of uniform density
collapse/consolidation/abscess • Thin-walled water-attenuation lesion on
–– Central cystic bronchiectasis CT
–– Upper zone pulmonary fibrosis • Daughter cysts seen as curved internal
Imaging features septations
• Cyst calcification in the lung is rare
CXR • Rupture into the pleural or pericardial
• Recurrent transient upper zone pulmonary
cavity is recognised: a ruptured cyst may
infiltrates
have a crumpled wall or air–fluid levels
Pulmonary eosinophilia
Tuberculosis
• Blood eosinophilia (> 0.4 × 109/L) and • 97% of cases caused by Mycobacterium
pulmonary infiltration tuberculosis
• 3% caused by Mycobacterium avium-
Causes: intracellulare complex or Mycobacterium
• Asthma kansasii
• Allergic bronchopulmonary • Acquired via droplet inhalation
aspergillosis • At-risk groups are the elderly, alcoholics
• Vasculitides, e.g. Churg–Strauss and immunocompromised patients
syndrome, Wegener’s granulomatosis
• Drug-induced disease, e.g. from non- Primary tuberculosis
steroidal anti-inflammatory drugs, • Majority of cases are subclinical
sulphonamides • Commonest in infants and young children
• Parasitic infestation, e.g. • Inhaled organisms settle in alveoli
schistosomiasis, ascariasis
• Simple eosinophilic pneumonia Imaging features
(Löffler’s syndrome) • Lobar or segmental consolidation (with a
• Acute eosinophilic pneumonia predilection for middle and lower lobes)
• Chronic eosinophilic pneumonia with ipsilateral hilar lymphadenopathy
• Tropical pulmonary eosinophilia (filarial • Bilateral hilar adenopathy is an unusual
sensitivity) feature

Ch-01.indd 36 8/7/2010 3:33:57 PM


Pulmonary infections 37

• Lymph nodes have low-attenuation Other pulmonary


centres and rim enhancement
• Painless unilateral pleural effusion in 25%
manifestations
and is often the only feature Miliary tuberculosis
• Infection is self-limiting in • Occurs in 1–7% of all patients with
immunocompetent patients tuberculosis (more commonly in
• Consolidation resolves completely in 70% secondary than primary disease)
of patients and with a scar in 30% • Represents widespread haematogenous
• Ranke’s complex may be seen: a calcified dissemination of disease
scar and calcified hilar nodes • Typically seen in infants, the elderly and
• Failure to contain primary infection causes immunocompromised patients
dissemination of disease (progressive • Multiple opacities of < 5 mm seen
primary) with miliary and systemic spread throughout both lungs (in 50% of cases)
• Very rarely associated with pleural effusion
Post-primary tuberculosis • Also causes meningitis,
• Re-infection or reactivation of primary hepatosplenomegaly and
tuberculosis lymphadenopathy
• Parenchymal consolidation (usually • Delay in diagnosis is common because it
in apical segment of the upper lobe) can mimic a wide range of pathology
progressing to cavitation (in 50% of • Nodules resolve after treatment without
patients) is the hallmark of reactivation TB scarring or calcification
• Cavitation leads to haematogenous and
endobronchial dissemination (tree-in-bud Causes of miliary shadowing
pattern)
• Tracheobronchial stenosis and
• Tuberculosis
bronchiectasis occurs in 10–40% of cases
• Non-tuberculous infections:
• Pleural effusion is less common than in
histoplasmosis, varicella
primary disease, occurring in 18% of cases
• Pneumoconiosis: silicosis, coalworker’s
• Lymphadenopathy is unusual, occurring
pneumoconiosis
in 5% of cases
• Metastases, e.g. from melanoma,
• Miliary disease more common than in
thyroid tumour, renal tumour,
primary tuberculosis
choriocarcinoma
• Sarcoidosis
Causes of lung cavities • Ossifications, e.g. in microlithiasis,
mitral stenosis
• Abscess: staphylocci, Klebsiella, septic • Histiocytosis X
emboli • Amyloidosis
• Granulomas: tuberculosis,
histoplasmosis, sarcoidosis
• Bronchogenic carcinoma: squamous
cell carcinoma, adenocarcinomoa
Tuberculous empyema
• Metastases, e.g. from squamous cell • Associated with residual pleural
carcinoma in the head and neck thickening and calcification
• Vasculitis: Wegener’s granulomatosis, Mycetoma formation
rheumatoid arthritis, systemic lupus • Colonisation of an apical scar cavity with
erythermatosus Aspergillus
• Cystic bronchiectasis • Haemoptysis is very common
• Pulmonary infarction • Air crescent sign on CXR

Ch-01.indd 37 8/7/2010 3:33:58 PM


38 Cardiothoracic and vascular system

Tuberculoma Opportunistic infections


• Seen in both primary and post-primary
tuberculosis Pneumocystis carinii
• Localised disease, which alternately pneumonia (now renamed
activates and heals Pneumocystis jiroveci)
• Well-defined nodule of 1–4 cm diameter • AIDS-defining illness occurring with CD4+
with satellite lesions T-cell counts < 200 cells/mm3
• Commonest in right upper lobe • Reactivation of infection acquired in early
• Calcifications may be seen but cavitation is life
rare • Pneumocystis carinii is a unicellular
fungus that adheres to type 1 pneumocytes
HIV- and AIDS-related lung • Proliferation causes a foamy exudate and
disease interstitial thickening
• Presents with insidious onset of dry cough,
• The HIV retrovirus impairs cellular fever and exertional dyspnoea
immunity by targeting CD4+ T-helper cells • Raised serum lactate dehydrogenase
• It also impairs humoral immunity, • Gallium-67 uptake occurs before CXR
and patients develop a range of chest changes
complications, including opportunistic • Mortality of 10% from respiratory failure
infections and rare malignancies • Radiology can suggest the diagnosis but
confirmation is by silver stain of induced
CD4+ T-cell count and pulmonary sputum
disease
Imaging features
< 400 cells/mm3: pyogenic infection, CXR
tuberculosis • Normal CXR at presentation in 20% of
< 200 cells/mm3: pneumocystis cases
< 100 cells/mm3: Kaposi’s sarcoma, • Classically CXR shows bilateral
cytomegalovirus symmetrical perihilar reticular opacities
< 50 cells/mm3: lymphoma, • 10% develop thin-walled cysts
Mycobacterium avium-intracellulare (pneumatoceles)
complex • One third of pneumatoceles rupture,
causing pneumothorax
HRCT
Bacterial pneumonia • Diffuse ground-glass change with
• Often the cause of a first AIDS-related peripheral sparing
chest infection
• Occurs at relatively high CD4+ T-cell Atypical patterns of Pneumocystis
counts carinii pneumonia
• Streptococcus pneumonia and
Haemophilus influenza are the commonest • Miliary nodules
pathogens • Larger nodules, which cavitate
• Same radiological pattern as seen in • Focal air space consolidations
immunocompetent patients • Pleural effusion
• Lymphadenopathy
• Upper zone distribution (seen with
patients on pentamidine aerosol
prophylaxis)

Ch-01.indd 38 8/7/2010 3:33:58 PM


Pulmonary infections 39

Invasive aspergillosis Non-infectious pulmonary


• An often fatal infection in the severely Disease
immunocompromised
• Angioinvasion occurs, with haemorrhage Kaposi’s sarcoma
and lung infarction • Commonest AIDS-associated malignancy
• Mortality is 60% • Causal agent is human herpes virus-8
• Almost all cases occur in homosexual or
Imaging features
bisexual men
CXR • Occurs in late-stage disease; pulmonary
• Multiple, ill-defined patches of disease is rare in the absence of
consolidation mucocutaneous lesions
• Lesions may cavitate in later stages of • Bronchoscopy shows raised erythematous
disease lesions along the tracheobronchial tree
HRCT • Occasionally lesions are large and cause
• Multiple nodules with surrounding collapse of distal airways
ground-glass halos Imaging features
• Lesions progress into more diffuse
consolidation CXR
• Coarse linear opacities radiating from the
Tuberculosis hilum
• Commonest worldwide manifestation of • Multiple, ill-defined pulmonary nodules
AIDS • Pleural effusions (serosanguinous) in the
• Radiological features depend on severity of majority of cases
immunosuppression HRCT
Early-stage disease • Nodules with surrounding ground-glass
halos
• Manifestations are similar to those of non-
• Distribution along the bronchovascular
AIDS-related tuberculosis
bundles
• Reactivation can occur with CD4+ T-cell
• Lymphadenopathy in 30% of cases, which
counts < 400 cells/mm3
shows uniform enhancement
Late-stage disease
• Inability to mount a cell-mediated
Lymphocytic interstitial
response (negative tuberculin test) pneumonia
• Rapid progression of newly acquired • Diffuse interstitial infiltration by
infection lymphocytes and plasma cells
• Diffuse bilateral reticulonodular infiltrates • Rare in adults but commonest pulmonary
complication in children
Mycobacterium avium- • Other associations include Sjögren’s
intracellulare complex syndrome and primary biliary cirrhosis
• Commonly isolated but rarely clinically Imaging features
significant
• Complication of end-stage disease (when CXR
CD4+ T-cell counts < 50 cells/mm3) • Reticulonodular pattern in the mid-zone
• Usually causes extrathoracic disease, e.g. and lower zone
small bowel infiltration HRCT
• Pulmonary appearances are non-specific • Diffuse bilateral ground-glass appearance
and similar to those of tuberculosis and centrilobular nodules
• Slowly progressive over several years

Ch-01.indd 39 8/7/2010 3:33:58 PM


40 Cardiothoracic and vascular system

AIDS-related lymphoma • Rapidly enlarging parenchymal nodules,


which may cavitate
• High-grade non-Hodgkin’s lymphoma of
B-cell type
Hilar and mediastinal adenopathy
• Usually involves extranodal sites, e.g. the
in AIDS
CNS
• Intrathoracic involvement is uncommon
• Most cases are due to reactive nodal
Imaging features hyperplasia
CXR • Other causes include tuberculosis,
• Hilar and mediastinal lymphadenopathy Kaposi’s sarcoma and lymphoma
• Interstitial infiltrates

Ch-01.indd 40 8/7/2010 3:33:58 PM


Pulmonary
1.8 thromboembolism

Anatomy • Most lodge peripherally, but large thrombi


can lodge within the right heart or straddle
• The pulmonary trunk is approximately the main pulmonary artery bifurcation
5 cm long and begins at the pulmonary (saddle embolus)
valve (anterior to the aorta) • Entrapment within a pulmonary artery
• It passes posteriorly and to the left to lie causes cessation of distal blood flow
in the concavity of the aortic arch where and an increase in pulmonary vascular
it bifurcates into right and left pulmonary resistance
arteries • There is continuing ventilation beyond the
• The pulmonary trunk is covered by embolus as the airways remain patent and
pericardium to its bifurcation ventilated
• The right pulmonary artery runs • Large or multiple emboli can cause
horizontally to the right, posterior to the sudden death secondary to severe right
ascending aorta and anterior to the right ventricular strain and circulatory collapse
main bronchus; it is crossed anteriorly by • Only around 10% of pulmonary emboli
the right superior pulmonary vein cause lung infarction because there is
• The left pulmonary artery arches over the generally good collateral supply via the
left main bronchus to lie posterior to it and bronchial arteries
is crossed anteriorly by the left superior
pulmonary vein; it is attached to the aortic Clinical assessment
arch by the ligamentum arteriosum • Pulmonary embolism has a wide spectrum
• Each main pulmonary artery subdivides of clinical manifestations ranging from
into lobar arteries and then segmental absence of symptoms to sudden death
arteries, which accompany the bronchi • The classic triad of dyspnoea, haemoptysis
and pleuritic pain is rare
Thromboembolic disease • In the majority of patients a combination
of clinical and laboratory findings is
• Majority of pulmonary emboli are caused insufficient for reliable diagnosis of acute
by venous thromboembolism pulmonary embolism
• Non-thrombotic causes include fat emboli, • Scoring criteria that are based on clinical
amniotic emboli and septic emboli features, D-dimer and ECG findings can
• Pulmonary emboli account for 5–10% of help in the decision about which patients
deaths in hospital patients should proceed to imaging investigations
• Mortality is 30% mortality if untreated
(from recurrent pulmonary embolism) D-dimer blood test
• Mortality is 3% with anticoagulation and/
or IVC filter placement
• By-product of clot fibrinolysis
Pathophysiology • Considered raised if > 0.5 mg/L
• Negative result virtually excludes a
• More than 90% of pulmonary emboli arise
pulmonary embolism
from venous thrombosis within the deep
• Low specificity, because it is raised in
leg or pelvic veins
many other conditions
• Emboli reach the right atrium, where they
fragment and shower into the pulmonary
arterial tree

Ch-01.indd 41 8/7/2010 3:33:58 PM


42 Cardiothoracic and vascular system

Imaging features • Perfusion component


–– Albumin macroaggregates labelled with
CXR
technetium-99m
• Main role is to exclude other diagnoses,
–– 300,000 particles in a standard dose,
e.g. pneumothorax
each 10–30 micrometres in size
• Normal in around 15% of cases
–– Intravenous injection given with
• Non-specific signs (common)
the patient in the supine position to
–– Linear atelectasis (in 70% of cases)
minimise gravitational gradient
–– Parenchymal opacity (in 60% of cases)
–– The particles are microembolised in the
–– Pleural effusion (in 50% of cases);
pulmonary arterial capillary bed during
typically small and unilateral
first pass through the lungs
–– Elevated hemidiaphragm (in 30% of
–– The particles obstruct about 0.2% of the
cases)
pulmonary capillary bed
• Specific signs (in < 10% of cases)
–– A half dose perfusion study is
–– Oligaemia distal to embolus
performed in pregnancy
(Westermark sign)
–– Normal study shows homogeneous
–– Enlarged artery proximal to embolus
tracer distribution
(Fleischner sign)
–– Areas of absent perfusion are seen as
–– Pleural-based wedge-shaped opacity
photopenic defects
(Hampton hump)—an infarction
–– A normal perfusion scan excludes 95%
causing haemorrhagic congestion that
of pulmonary emboli
is visible 12–24 hours after an embolic
episode; it rarely undergoes cavitation,
Conditions in which a reduced
and it resolves over months from the
number of particles should be used
periphery inwards (‘melts away’) to
leave a residual linear scar or pleural
thickening • Right-to-left shunt
• Pregnancy
Causes of an oligaemic lung • Pulmonary hypertension

• Pulmonary embolism Interpretation


• Pneumothorax • Pulmonary embolus indicated by absent
• Mastectomy perfusion and normal ventilation (a V/Q
• Emphysema mismatch)
• Foreign body • Reporting is based on the modified
• Poland’s syndrome (absent pectoralis) PIOPED criteria, derived from the
• Macleod’s syndrome Prospective Investigation of Pulmonary
Embolism Diagnosis (PIOPED) study
–– A high-probability scan suggests > 80%
V/Q scan likelihood of a pulmonary embolus
• Assessment of pulmonary blood flow and –– An intermediate scan suggests 20–80%
alveolar ventilation likelihood of a pulmonary embolus
• Effective dose is approximately 2 mSv –– A low-probability scan suggests < 20%
• Ventilation component likelihood of a pulmonary embolus
–– Xenon-133 or krypton-81 are the most • A high-probability scan in combination
commonly used agents with high clinical pretest probability is
–– Particle size 0.1–0.5 micrometres > 90% accurate
–– Inhaled via aerosol in upright position • A low-probability scan in combination
–– Performed before perfusion scan with low clinical pretest probability is
–– Normal study shows homogeneous > 90% accurate
tracer accumulation

Ch-01.indd 42 8/7/2010 3:33:58 PM


Pulmonary thromboembolism 43

• However 50–70% of V/Q scans are • Main use is prior to in situ thrombolysis
intermediate (non-diagnostic) • Arrhythmias are most common non-fatal
• Around 30% of patients with intermediate complication (occurring in 5% of cases)
scans will have a PE • Quoted mortality is approximately 0.5%
• Brain or kidney uptake is diagnostic of a • Emboli seen as intraluminal filling defects
right-to-left shunt
• The stripe sign is a perfusion defect with
a peripheral zone of preserved perfusion
CT pulmonary angiography
and suggests a diagnosis other than (CTPA)
pulmonary embolism • Multi-detector CT has replaced pulmonary
• Patients with persisting perfusion defects angiography as the reference standard for
are at risk of developing pulmonary diagnosing acute pulmonary embolism
hypertension • Quoted sensitivity and specificity > 95%
down to segmental level
Causes of V/Q mismatch • Test bolus or bolus tracking technique
to ensure optimal opacification of the
• Pulmonary embolism pulmonary arterial tree
• Emphysema • Effective dose 3–6 mSv (equivalent to
• Extrinsic arterial compression, e.g. 1–2 years’ background radiation)
tumour • Cannot reliably exclude subsegmental
• Primary pulmonary hypertension emboli
• Pulmonary vasculitis • The presence of right ventricular
• Intralobar sequestration dysfunction carries a worse prognosis and
• Pulmonary artery hypoplasia is an indication for thrombolysis or surgery
Imaging features
• Intraluminal filling defects surrounded by
Causes of a matched V/Q defect contrast
–– ‘Polo mint’ sign on axial section
• Pulmonary infarction –– ‘Railway track’ sign on coronal section
• Emphysema • Pulmonary infarct shows as a subpleural
• Pulmonary fibrosis opacity and pleural effusion in most cases
• Right ventricular strain is seen with
massive pulmonary embolism
–– Right ventricular dilatation (right
Causes of a reverse V/Q mismatch, ventricular cavity wider than left
in which perfusion exceeds ventricular cavity)
ventilation –– Reverse bowing of the interventricular
septum
• Chronic obstructive pulmonary disease –– Contrast reflux into the hepatic veins
• Bronchiectasis
• Consolidation or collapse Pitfalls in CTPA
• Pleural effusion • Causes of false-negative results
–– Inadequate contrast opacification
–– Subsegmental emboli
–– Partial volume and respiratory motion
Pulmonary angiography artefacts
• Causes of false-positive results
• An invasive test, formerly the gold- –– Inadequate contrast opacification
standard imaging test for pulmonary –– Lymph nodes adjacent to central
embolism pulmonary arteries
• Quoted sensitivity and specificity of > 95%

Ch-01.indd 43 8/7/2010 3:33:58 PM


44 Cardiothoracic and vascular system

–– Bronchial mucus plugging adjacent to Deep vein thrombosis


peripheral branches
–– Contrast mixing with unenhanced (DVT)
blood from the IVC (caused by patient • Risk factors include immobility,
inspiration) pregnancy, malignancy, intravenous drug
use and hypercoagulable states
CT venography • The left leg is more commonly affected
• Performed 3–4 minutes after than the right leg, owing to the
administration of intravenous contrast compressive effect on the left iliac vein of
• 95% concordance with ultrasound for the right iliac artery crossing it
femoral vein thrombus • Below-knee DVT has a 20% risk of
• Superior to ultrasound for iliac vein and proximal extension if untreated
IVC thrombi • Saphenous vein thrombosis has a risk of
• Improves sensitivity of venous propagation into the deep venous system
thromboembolism diagnosis
• Increases radiation exposure by 2–4 mSv Imaging features
Ultrasound
Pulmonary embolism in • > 90% sensitive for above-knee DVT
• Acute DVT
pregnancy –– Dilated vein containing echo-poor
• Pulmonary embolism is twice to four times thrombus
as common in pregnancy –– Non-compressible and absent colour
• Increased frequency caused by pressure flow
effects on pelvic veins by the gravid uterus –– Non-occlusive thrombus may allow
in combination with increased blood some compressibility
viscosity –– Adductor canal segment may normally
• Second commonest cause of maternal be non-compressible
death during pregnancy • Chronic DVT
• D-dimer levels are normally raised during –– Echo-bright thrombus in a contracted
pregnancy vein (although echogenicity is an
unreliable sign in that both fresh and
Imaging features old thrombus can appear hypoechoic or
V/Q scan hyperechoic relative to muscle)
• Absorbed fetal dose is 0.4–0.6 mSv –– Colour flow may be seen within the
• Approximately 70% of V/Q scans in thrombus as a result of partial re-
pregnancy are normal canalisation
• Approximately 25% of V/Q scans in
pregnancy are non-diagnostic Indirect signs of DVT
• Half-dose perfusion-only scintigraphy is
commonly used • Loss of phasicity (normal respiratory
CTPA variation) when scanning distal to clot
• Absorbed fetal dose is 0.03–0.06 mSv • Loss of normal velocity increase with
• Around 5–10% of CTPA examinations in distal compression when scanning
pregnancy are non-diagnostic proximal to clot
• Lactating breasts are very radiosensitive,
and CTPA carries a high effective dose
(7.4 mSv per breast)

Ch-01.indd 44 8/7/2010 3:33:58 PM


Pulmonary thromboembolism 45

Non-thrombotic pulmonary • This triggers a massive anaphylactic


reaction with disseminated intravascular
embolism coagulation
Septic emboli • Abrupt onset of dyspnoea, cyanosis and
hypotension
• Most commonly arise from tricuspid valve • Mortality is 80%, secondary to
endocarditis cardiopulmonary collapse
• Typically patients are intravenous drug
users Imaging features
• Staphylococcus aureus is the commonest CXR
pathogen • Widespread bilateral air space
Imaging features consolidation

CXR
• Multiple peripheral nodular opacities IVC filters
• Mid- and lower zone predominance • Prevent pulmonary embolism by trapping
• Frequently undergo cavitation thrombus from the pelvic and leg veins
• Empyema is a well-recognised • Endovascular placement under
complication fluoroscopic guidance via a femoral or
right internal jugular vein approach
Fat embolism • Most filters are permanent and placed in
• Mechanical obstruction of the pulmonary the IVC
capillary bed by fat globules, which incite a • Indications for filter placement
toxic endothelial reaction –– DVT or pulmonary embolism with a
• Closed fracture of a lower limb long bone contraindication to anticoagulation
is the commonest cause –– DVT or pulmonary embolism with a
• Occurs in 1–3% of patients with femoral complication from anticoagulation
neck fractures –– Pulmonary embolism while on
• Occurs in up to 20% of patients following anticoagulation
major pelvic trauma –– Prophylaxis in high-risk patients,
• Other causes include liposuction and e.g. following pulmonary
pancreatitis thromboendarterectomy
• Symptom lag > 24 hours
• Symptoms include dyspnoea, agitation Indications for supra-renal filter
and petechial rash placement
Imaging features
• Renal vein thrombosis
CXR
• IVC thrombus extending above the
• Lag > 24 hours
renal veins
• Bilateral patchy infiltrates
• Clears in 7–14 days, or may progress to
ARDS
• Not associated with pleural effusion or Complications
pneumothorax • Failure: ongoing pulmonary embolism in
3% of patients
Amniotic fluid embolism • IVC thrombosis in 10%
• Rare obstetrical emergency • Embolisation of the filter
• Amniotic fluid enters the maternal
circulation through small tears in the
uterine veins during labour

Ch-01.indd 45 8/7/2010 3:33:58 PM


46 Cardiothoracic and vascular system

Pulmonary arterial CT
• General features of pulmonary
hypertension hypertension
• Mean pulmonary artery pressure • Lungs may be clear or have multiple
> 25 mmHg at rest and > 30 mmHg with centrilobular ground glass opacities
exercise
• Causes include idiopathic pulmonary
Intra- and extracardiac shunts
hypertension, chronic thromboembolic • Chronically raised blood flow induces
pulmonary hypertension, longstanding intimal hyperplasia and a plexiform
left-to-right shunts and pulmonary veno- arteriopathy
occlusive disease • Pulmonary pressure (if untreated)
may exceed systemic blood pressure,
Imaging features with partial or complete shunt reversal
CXR (Eisenmenger’s syndrome). This is
• Enlarged proximal pulmonary arteries heralded by cyanosis and is considered
• Peripheral pruning of vessels inoperable
• Right ventricular enlargement Causes
–– Atrial septal defects: ostium secundum,
CT
ostium primum, sinus venosus
• Main pulmonary artery diameter > aortic
–– Ventricular septal defects:
diameter at corresponding level
perimembranous, muscular
• Right-sided cardiac chamber enlargement
–– Partial anomalous pulmonary venous
• Right ventricular hypertrophy (free wall
drainage (often associated with sinus
> 3 mm)
venous type atrial septal defect)
• Tricuspid regurgitation (shows as reflux of
–– Patent ductus arteriosus
contrast into hepatic veins)
–– Truncus arteriosus
• Laminated (often partially calcified)
thrombus lining the central pulmonary Chronic thromboembolic
arteries pulmonary hypertension
Idiopathic pulmonary arterial (CTEPH)
hypertension • 3–4% of patients with acute PE are thought
• Smooth muscle hypertrophy of small pre- to develop CTEPH
capillary pulmonary arteries (plexiform • Those with proximal disease affecting 5 or
lesion) more segmental vessels may be candidates
• Mean age of presentation is 36 years, with for thromboendarterectomy surgery
a slight female preponderance Imaging features
• Presents with progressive dyspnoea and
signs of right heart strain V/Q scan
• 5-year survival rates around 55% with • Multiple segmental and subsegmental
vasodilator infusions perfusion defects
CT
Imaging features • Multiple stenoses, webs and occlusions
CXR • Laminated central thrombi are also
• Findings suggestive of pulmonary common; approximately 10% are calcified
hypertension at time of presentation in • Mosaic attenuation pattern on lung
90% of cases windows in 80–100% patients
V/Q scan • Bronchial artery hyperplasia, pericardial
• Mottled appearance or low probability for effusion and peripheral lung infarcts
pulmonary embolism

Ch-01.indd 46 8/7/2010 3:33:58 PM


Pulmonary thromboembolism 47

Left heart disease Imaging features


• Any longstanding condition that impedes CT
pulmonary venous return can lead to • Interstitial oedema with septal thickening
pulmonary arterial hypertension predominates
• Imaging findings are those of pulmonary • Normal left atrial and pulmonary vein
venous hypertension with upper zone dimensions
venous diversion and interstitial oedema • May be associated ground-glass opacities
• Left atrial enlargement may be evident
with mitral stenosis Pulmonary capillary
• Causes haemangiomatosis
–– Mitral stenosis • Uncontrolled capillary proliferation
–– Left ventricular failure • Treatment with vasodilator therapy can be
–– Left atrial myxoma fatal
–– Fibrosing mediastinitis
Imaging features
Pulmonary veno-occlusive CT
disease • Centrilobular ‘fluffy’, ground–glass nodules
• Intimal fibrosis of the pulmonary venules predominate
• Associated with cytotoxic drugs and the • Interlobular septal thickening may also be
ingestion of herbal teas seen
• Focal areas of oedema and haemorrhage

Ch-01.indd 47 8/7/2010 3:33:59 PM


Postoperative chest
1.9 radiography

Post-thoracotomy • A lower positioned tube may descend into


the right main bronchus with neck flexion
• Lung resections are performed via a (causing right lung hyperinflation and
posterolateral incision through the fifth or carrying the risk of pneumothorax)
fifth intercostal space
Complications of positive pressure
Pneumonectomy ventilation
• Fluid slowly fills the vacant hemithorax
Imaging features • Pneumothorax
• Rising air–fluid level over weeks to months • Pneumomediastinum
• Rate of fluid accumulation is very variable • Subcutaneous emphysema
• Tracheal and mediastinal shift towards the • Pneumoperitoneum
surgical side
• Bronchopleural fistula
–– Caused by dehiscence of the bronchial Tracheostomy
stump, infection or recurrent tumour • Should lie centrally in the trachea with the
–– Drop in an existing air–fluid level tip a few centimetres above the carina
–– Air in a previously obliterated space • A small amount of surgical emphysema is
–– Mediastinal shift away from the surgical expected
side • Large-volume emphysema suggests tube
–– Aspiration of pneumonectomy space leak or tracheal injury
fluid into the contralateral lung • Long-term complication is tracheal
• Empyema stenosis
–– Reported incidence of 1–5%
–– CT is modality of choice for assessment Central venous pressure line
–– Expansion of post-pneumonectomy • Used to monitor right atrial pressure
space, mediastinal shift towards the • Tip should be around the junction of the
opposite side, straightening of the SVC and the right atrium
normally concave mediastinal border, • Rarely, the tip may fracture, with
and irregular, thick, enhancing residual embolisation
pleura
Swan–Ganz catheter
Lobectomy • Monitors pulmonary artery pressure
• The remaining lobes hyperinflate, the • Tip should lie 5 cm beyond pulmonary
diaphragm elevates and the mediastinum trunk in left or right pulmonary artery
shifts towards the surgical side
• Fluid slowly fills any remaining space with
a rising air–fluid level over several weeks Lung transplantation
Indications
Lines and tubes • Accepted therapy for many forms of end-
stage lung disease
Endotracheal tube • Single lung transplantation is the more
• Correct position is in mid-trachea, 5 cm commonly performed
above the carina

Ch-01.indd 48 8/7/2010 3:33:59 PM


Postoperative chest radiography 49

• Indications include chronic obstructive • Airway anastomotic dehiscence


pulmonary disease and interstitial lung –– Potentially fatal complication affecting
disease e.g. lymphangioleiomyomatosis < 2% of patients
• Double lung transplantation is performed –– Blood supply to the bronchi is via the
in pulmonary hypertension and cystic bronchial artery and is impaired by
fibrosis low cardiac output states, leading to
• Contraindicated in malignancy or with ischaemia
active extrathoracic infection –– Bronchial stenosis is a recognised late
complication
Early complications
• Reperfusion oedema Late complications
–– A re-implantation response • Chronic rejection
–– Non-cardiogenic pulmonary oedema –– Pathologically the features are those
–– Begins in the first 48 hours of bronchiolitis obliterans, with a
after transplant and resolves by submucosal inflammatory process of
day 10 the terminal bronchioles
–– Caused by a combination of surgical –– Eventually leads to fibrosis and
trauma, ischaemia and denervation of permanent obliteration
transplanted lung –– HRCT shows mosaic attenuation (air
–– CXR shows perihilar haziness of the trapping)
transplanted lung • Post-transplant lymphoproliferative
–– Diagnosis of exclusion (not due to disorder
infection, oedema or rejection) –– Occurs in approximately 5% of patients
• Acute rejection after a lung transplant (higher than for
–– Very common: 80% have an episode in any other organ)
the first 3 months after transplant –– Associated with Epstein–Barr virus
–– Non-specific symptoms (fever, –– Occurs at a mean of 4 months’
tachypnoea and dyspnoea) postoperatively
–– CXR shows non-specific features: septal –– Ranges in severity from low to
lines and pleural effusions aggressive high-grade non-Hodgkin’s
–– Transbronchial biopsy shows lymphoma
perivascular lymphocytic infiltrate –– CT shows multiple pulmonary nodules
–– HRCT shows multifocal patchy areas of and mediastinal adenopathy
ground-glass attenuation
–– Treated by increasing the degree of
immunosuppression
Post-cardiac surgery
• Infection • Most operations performed via a midline
–– Commonest cause of perioperative sternotomy
mortality • Left lateral thoracotomy used for mitral
–– Occurs in up to 50% of lung transplant valvotomy or coarctation of the aorta
recipients • Majority have a small left pleural effusion
–– Denervated lung has impaired and basal atelectasis
mucociliary drainage • Phrenic nerve damage can cause
–– Commonest organisms are hemidiaphragm paralysis
Pseudomonas, Aspergillus and • Thoracic duct damage causes a chylous
cytomegalovirus pleural effusion
–– Patients are given prophylactic • Mediastinitis is uncommon, occurring in
antibacterial, antifungal and antiviral < 1% of patients
drugs • Late autoimmune pericarditis is
recognised (Dressler’s syndrome)

Ch-01.indd 49 8/7/2010 3:33:59 PM


1.10 Chest trauma

• Leading cause of death in children and Imaging features


young adults in the UK
CXR
• Majority of cases caused by road traffic
• Haematoma seen as a bulge in the left
accidents
paraspinal line

Fractures Pleural space


Rib fractures Haemothorax
• Commonest skeletal injury resulting from
• Blood in the pleural space
blunt chest trauma
• Usually from intercostal venous or arterial
• Complications include pneumothorax and
bleeding
pulmonary contusions
• First to third rib fractures Imaging features
–– Markers of high-velocity injury
CXR
–– Associated with brachial plexus and
• Detected on frontal CXR when > 250 mL
subclavian vessel injury
• Concave upward sloping of costophrenic
• Eighth to 11th rib fractures
angle (meniscus sign)
–– Associated with hepatic, splenic and
renal injuries CT
–– Splenic injury is associated with rib • High-attenuation fluid (35–70 HU)
fractures in 40% of cases
–– In children there is a strong association
Thoracic duct
with non-accidental injury • Drains lymph from the abdomen and
• Flail chest lower limbs
–– Two fracture sites on each of three or • Arises from the cisterna chyli behind the
more consecutive ribs right crus of the diaphragm
–– Free-floating segment moves • Enters the thoracic cavity through the
paradoxically with respiration aortic hiatus
–– Associated with massive underlying • Ascends anterior to the vertebral column
lung contusion between the azygous vein and the aorta
–– Impairs mechanics of ventilation and • The posterior intercostal arteries and the
requires ventilatory support hemiazygous vein cross behind it
• At the level of the carina (T5) it deviates to
Sternal fracture the left of the oesophagus
• Uncommon injury usually caused by a • Ascends into the neck and passes behind
direct blow the left subclavian vessels
• Displaced fractures associated with • Arches anteriorly over the left lung apex to
myocardial contusion drain into the left internal jugular vein at
• Anterior mediastinal haematoma is a its junction with the subclavian vein
common finding
Chylothorax
Thoracic spine fracture • Can occur following chest trauma or in
• Commonest at the mobile thoracolumbar patients with lymphangioleiomyomatosis
junction region
Imaging features
• Usually a hyperflexion injury or an axial
compression (burst) injury CXR
• Associated with spinal cord injury in 40% • Low-attenuation fluid (negative HU value)
of cases

Ch-01.indd 50 8/7/2010 3:33:59 PM


Chest trauma 51

Pneumothorax Imaging features


• Occurs in 30–40% of cases of major chest CXR
and abdominal trauma • Contralateral mediastinal shift
• Usually caused by a rib fracture or • Depression of ipsilateral hemidiaphragm
penetrating injury • Widening of intercostal spaces
• Chest drain indicated if clinical signs of
tension are present Pulmonary contusion
• Unilateral oedema (re-expansion) is well • Commonest lung injury in blunt chest
recognised trauma (occurring in 30–70% of cases)
• If pneumothorax does not respond to • Capillary haemorrhage into alveolar and
the insertion of a chest drain, consider a interstitial spaces
malpositioned drain, a tracheobronchial • Radiographically apparent within 6 hours
injury or a bronchopleural fistula of injury
• Haemoptysis is most frequent clinical
Causes of pneumothorax symptom
Imaging features
Spontaneous pneumothorax:
• Rupture of a subpleural bleb CXR
• Paraseptal emphysema • Non-segmental air space consolidation
• Infections, e.g. Pneumocystis carinii CT
pneumonia • Parenchymal opacification with subpleural
• Cystic lung disease e.g. sparing
lymphangioleiomyomatosis • Imaging hallmark is rapid resolution
• Osteosarcoma metastases (usually within 72 hours)

Traumatic pneumothorax:
• Rib fracture with laceration
Diaphragm
• Chest compression injury Anatomy
• Dome-shaped sheet separating the
Iatrogenic pneumothorax: thoracic and abdominal cavities
• Central line insertion • Peripheral fibromuscular bands converge
• Lung biopsy on to a central tendon
• Thoracic surface lined by parietal pleura
• Abdominal surface lined by peritoneum
Imaging features except over the bare area
Erect CXR • Anteriorly attaches to the xiphisternum,
• Thin line of visceral pleura with absent laterally to the lower six costal cartilages
peripheral lung markings and posteriorly to the crura and arcuate
• Appearances become more pronounced ligaments
on expiratory film • Medial arcuate ligament is a fascial
thickening over the psoas muscle
Supine CXR • Lateral arcuate ligament is a fascial
• Deep costophrenic sulcus sign thickening over the quadratus lumborum
Tension pneumothorax muscle
• The crura are attached between vertebral
• Progressive build-up of air due to a one-
bodies L1 and L3
way valve mechanism
• The median arcuate ligament lies between
• Compression of SVC leads to rapid
the two crura – the aorta, azygous vein
circulatory collapse
and thoracic duct pass behind it at the T12
level

Ch-01.indd 51 8/7/2010 3:33:59 PM


52 Cardiothoracic and vascular system

• Openings • Tears seen as a defect in this low-signal


–– Oesophagus at the T10 level line
–– IVC at the T8 level
• The right phrenic nerve passes through
the IVC hiatus. Lymphatics and branches
Mediastinal injury
of the left phrenic nerve pierce the Blunt traumatic aortic injury
diaphragm • Shearing injury caused by sudden
• A small posterolateral defect (Bochdalek deceleration (usually as a result of a road
hernia) and areas of marked thinning traffic accident)
(eventration) occur in up to 5% of the • 90% of tears occur at the relatively ‘fixed’
population isthmus; 4–5% occur at the arch of the
Diaphragm rupture aorta; 5–9% occur in the ascending aorta;
1–3% occur at the diaphragmatic hiatus
• Occurs in 1–8% of patients who survive • 80–90% of cases involve laceration through
major chest or abdominal trauma all three layers of the aortic wall, resulting
• Blunt trauma causes a sudden rise in intra- in exsanguination and death at the scene
abdominal pressure • The incomplete type of injury may not be
• Left-sided tears are seen in 70% of cases, recognised in clinically stable patients and
right-sided tears in 30% (because of the may evolve into a pseudoaneurysm with
protective effect of the liver) a high risk of late rupture; CT has lead to
• The posterolateral region is commonest improved detection of these injuries
location • Endovascular repair is the current gold
• Defects are usually large (> 10 cm) in blunt standard for pseudoaneurysm
trauma; penetrating trauma usually causes • Occasionally may present years later
small focal tears (< 2 cm) with incidental CXR finding of a calcified
• Associated with splenic, liver and thoracic mediastinal mass
spine injuries • Imaging involves demonstration of
• Potentially devastating complications if aortic wall abnormality (direct sign) or
not repaired indirect evidence such as a mediastinal
• Negative pressure pulls abdominal haematoma
contents into the thoracic cavity • A mediastinal haematoma which does
• Visceral strangulation has a > 50% not abut the aorta or great vessels usually
mortality represents venous bleeding
Imaging features Imaging features
CXR CXR
• Unreliable for evaluation of diaphragmatic • A normal CXR has a high negative
injury predictive value (98%) for aortic injury but
• May see intra-thoracic abdominal viscera does not exclude it
or the tip of a nasogastric tube in the chest • Indirect signs of mediastinal haematoma
CT –– Superior mediastinal widening
• Multiplanar reconstructions improve –– Indistinct aortic arch outline
detection –– Trachea and oesophageal deviated to
• Collar sign the right
• Constriction of stomach or other viscera by –– Depression of the left main bronchus
the defect –– Widened left paraspinal stripe
• Dependent viscera sign –– Widened right paratracheal stripe
• Stomach or spleen contacting posterior –– Left apical pleural cap
chest wall (on axial sections) CT
MRI • Aortic wall contour abnormality or defect
• T1 sequence: a dark line with bright fat on • Adjacent mediastinal haematoma
either side is normal

Ch-01.indd 52 8/7/2010 3:33:59 PM


Chest trauma 53

• Intraluminal clot at the site of intimal • Associated with high rib fractures and
disruption multiple organ injury
• Pseudoaneurysm or aortic dissection • Bronchial tear more common than
(rare) tracheal injury
• Pericardial haematoma and coronary • Right main bronchus is the most
artery extension may be seen if the commonly injured site
ascending aorta is involved • 80% of cases occur within 2.5 cm of the
Aortogram carina
• Reserved for problem solving in equivocal • Signs of pneumomediastinum,
cases and for guiding endovascular stent pneumothorax and surgical emphysema
graft placement • May be indicated by a pneumothorax that
fails to respond to chest tube placement
• Lung collapses peripherally as a result
Tracheobronchial tear of disruption of the normal central
attachments holding it to the mediastinum
• Uncommon injury, occurring in 1–3% of (‘fallen lung’ sign)
cases of major blunt chest trauma

Ch-01.indd 53 8/7/2010 3:33:59 PM


1.11 Mediastinum

Anatomy Imaging features


• Extends from thoracic inlet to diaphragm. CT
Divided into two parts • Mediastinal thyroid mass
–– Superior mediastinum –– Continuous with the thyroid gland
–– Inferior mediastinum, which is further above
divided into anterior, middle and –– High attenuation on non-contrast scan
posterior sections (because of the high iodine content of
the thyroid)
–– May contain punctuate calcifications
Superior mediastinum –– Displays marked contrast enhancement
• From thoracic inlet to an imaginary line
drawn between the T4 vertebral body and Anterior mediastinum
manubriosternal junction
• Contents • Space between sternum and pericardium
–– The arch of the aorta and its branches • Contents are the thymus, internal
–– The SVC and the brachiocephalic veins mammary vessels and lymph nodes
–– The trachea • Causes of an anterior mediastinal mass
–– The oesophagus –– Thymic lesions
–– The thoracic duct and lymph nodes –– Teratoma
• Causes of a superior mediastinal mass –– Thyroid goitre/tumour
–– Goitre –– Terrible lymphoma
–– Cystic hygroma
–– Lymphoma Normal thymus

Imaging features • Bi-lobed gland with an arrowhead


CXR appearance
• Widened paratracheal stripe • Undergoes fatty replacement or
• Tracheal deviation involution with age
• Negative cervicothoracic sign: a mass • Normally visualised on CT in < 50% of
extending above the clavicle with no lung people over the age of 40 years
tissue between the mass and the neck • Normal anteroposterior diameter in
adults < 13 mm
Thyroid masses • CT shows homogeneous soft tissue
• Normal thyroid extends to just below level attenuation
of cricoid cartilage • MRI shows a mildly high T1 signal and a
• Thyroid goitres and rarely carcinomas can very high T2 signal
extend retrosternally
• Anaplastic carcinoma is the commonest
type of thyroid mass to do this
• Extension is usually anterior to the
Thymic hyperplasia
brachiocephalic vessels • Gland retains its normal shape
• Rarely can extend behind the oesophagus • Gland retains homogeneous CT
• Tracheal deviation is common attenuation and MRI signal
• Tracheal and oesophageal compression is • Causes
recognised –– Hyperthyroidism
–– Acromegaly

Ch-01.indd 54 8/7/2010 3:33:59 PM


Mediastinum 55

–– Addison’s disease Imaging features


–– Rebound after chemotherapy, burns or
CT
a course of corticosteroids
• Ill-defined mass encasing or invading
Thymoma mediastinal structures
• Drop metastases seen as areas of nodular
• Commonest tumour of the anterior
pleural thickening
mediastinum
• Peak age 50 years Thymic cyst
• Often an incidental CXR finding (in 50% of
• Congenital cyst
cases)
–– Arises from remnants of the
• Paraneoplastic symptoms are common
thymopharyngeal duct
–– Myasthenia gravis (in 50% of cases)
–– CT shows a thin-walled, unilocular cyst
–– Hypogammaglobulinaemia (in 10% of
of water attenuation
cases)
–– Can appear more solid if there is a high
–– Pure red cell aplasia (in 5% of cases)
protein content
–– MRI shows a low T1 signal and a high
Thymic lesions in myasthenia gravis
T2 signal
• Acquired cyst
• 20% will have a thymoma –– Seen in thymic tumours and in HIV-
• 65% will have thymic hyperplasia positive patients
–– Multilocular cyst with variable wall
CT is routinely performed in all patients thickness
with myasthenia gravis
Thymolipoma
• Rare benign tumour composed of fat and
Benign thymoma thymic tissue
• Can reach a large size, with compression of
• Accounts for two thirds of thymomas adjacent structures
• Tumour does not breach the thymic
capsule Thymic carcinoma
Imaging features • Rare aggressive malignancy
• Very locally invasive with early lymphatic
CT and distant spread
• Well-defined, lobulated mass distorting • Heterogeneous, ill-defined mass with
the normal thymic contour mediastinal adenopathy and pleural and
• 30% contain areas of necrosis and pericardial effusions
haemorrhage
• 7% contain thin peripheral capsular Thymic carcinoid
calcifications • Neuroendocrine tumour arising from the
• Surrounding fat plane is preserved (APUD) cell line
• Displays mild contrast enhancement • Associated with multiple endocrine
Invasive thymoma neoplasia (MEN) type 1
• Carcinoid syndrome is very rare
• Accounts for one third of thymomas • 40% secrete ACTH, causing Cushing’s
• Tumour breaches the thymic capsule syndrome
and spreads contiguously through the • Large heterogeneous invasive mass
mediastinum and into the pleural space,
where it disseminates via drop metastases Germ cell tumour
• Trans-diaphragmatic spread can occur via • Mediastinum is the commonest
the retrocrural space to involve the upper extragonadal site
abdominal organs and retroperitoneum

Ch-01.indd 55 8/7/2010 3:33:59 PM


56 Cardiothoracic and vascular system

• Arises from mediastinal cell rests Imaging features


• Accounts for 10% of anterior mediastinal
CXR
masses
• Filling in of AP window
• Commonest between the second and
• Displaced azygo-oesophageal recess
fourth decades
• Teratoma is the commonest subtype Bronchogenic cyst
Teratoma • Arises from abnormal budding of the
• Tissue elements from more than one germ ventral foregut
cell layer • Lined by ciliated columnar respiratory
• Majority are benign (< 3% become epithelium
malignant) • Mediastinal bronchogenic cyst
• Rarely rupture into the pleural or –– Accounts for two thirds of bronchogenic
pericardial space cysts
–– Most often in a subcarinal or right
Imaging features: CT paratracheal location
• Multicystic mass with enhancing septae –– Can cause stridor, dysphagia and
• 60% contain fat (fat-fluid level is intractable cough
diagnostic) –– Can rarely compress the main stem
• Commonly contains foci of calcification coronary artery
Ectopic parathyroid adenoma • Intrapulmonary bronchogenic cyst
–– Accounts for one third of bronchogenic
• 20% of parathyroid adenomas are ectopic
cysts
• Anterior mediastinum is a common site
–– Predilection for the lower lobes
• Preoperative localisation via MRI or
–– Commonly present with secondary
technetium-99m MIBI scan, with a
infection
combined accuracy of > 90%
Imaging features
Imaging features
CXR
MRI • Rounded, fluid-density mass near the
• Well-defined, rounded mass of 1–2 cm
carina
diameter
• High T2 signal (‘light bulb’ sign) CT
• Sharply marginated mass with a smooth
Technetium 99m MIBI border
• Adenoma shows tracer accumulation
• 50% show water attenuation, 50% higher
attenuation (because they contain protein
Middle mediastinum or blood)
• 1–2% have calcified walls or milk of
• Contents
calcium within cyst fluid
–– The heart
• May display a thin rim of mural
–– The great vessels
enhancement
–– Lymph nodes
–– The oesophagus MRI
–– The trachea • Variable T1 signal, uniformly high T2
–– The bronchi signal
• Causes of a middle mediastinal mass
–– Lymphadenopathy Pericardial cyst
–– Aortic arch vascular anomalies • See below (Section 2.14)
–– Azygous vein anomalies
–– Mediastinal bronchogenic cyst Posterior mediastinum
–– Oesophageal tumours, duplication cyst
–– Pericardial cyst • Space between the posterior aspect of the
heart and the vertebral column

Ch-01.indd 56 8/7/2010 3:33:59 PM


Mediastinum 57

• Contents • Associated vertebral body destruction and


–– Descending thoracic aorta disc space narrowing
–– Parasympathetic chain
–– Nerve roots Extramedullary haemopoiesis
–– Lymph nodes • Haemopoietic activity occurring at sites
–– Vertebrae other than bone marrow
• Causes of a posterior mediastinal mass • Seen in severe anaemia or extensive
–– Neurogenic tumour marrow infiltration
–– Lymphoma • Commonly involves the liver, spleen and
–– Paraspinal abscess lymph nodes
–– Bochdalek’s hernia • Can cause well-defined bilateral
–– Extramedullary haemopoiesis paravertebral masses
Imaging features
CXR
Mediastinitis
• Widened paratracheal stripe Acute bacterial mediastinitis
• Positive cervicothoracic sign: a mass • Life-threatening condition with a 50%
extending above the clavicle with lung mortality
tissue between the mass and the neck • Most commonly caused by oesophageal
Neurogenic tumours perforation
• Rarely a complication of cardiac surgery
Schwannomas and neurofibroma or from direct spread of retropharyngeal
• Benign nerve sheath tumour that grows infection
along parent nerve
• 50% of neurofibromas occur in patients
Imaging features
with neurofibromatosis type 1 CXR
• Rounded mass orientated along axis of • Widened mediastinal outline
intercostal nerve • Pneumomediastinum and subcutaneous
• May have an intraspinal component emphysema
(‘dumbbell-shaped’) • Air–fluid levels with abscess formation
• MRI shows a high-signal T2 rim with a CT
low-signal centre (‘target’ sign) • Extraluminal mediastinal air and peri-
Sympathetic ganglia tumour oesophageal fluid collections
• Ganglioneuroma (in young adults) and
neuroblastoma (in children)
Oesophageal perforation
• Oval, paraspinal mass vertically orientated
along the spine Distal (iatrogenic or due to foreign body,
malignancy, Boerhaave’s syndrome)
Parasympathetic tumour • Left-sided pleural effusion
• Extra-adrenal phaeochromocytoma • Normal mediastinal outline
• Commonest in the aortopulmonary
window and paravertebral region Proximal (due to blunt chest trauma)
• MRI shows heterogeneous mass lesions • Right-sided pleural effusion
that enhance avidly • Widened mediastinum
Paraspinal abscess Contrast swallow studies show
• Develops from spread of osteomyelitis in a extraluminal contrast extravasation
vertebral body and are 90% accurate for detecting a
• Most commonly caused by tuberculosis perforation
• Rim enhancement with non-enhancing
central pus collection

Ch-01.indd 57 8/7/2010 3:33:59 PM


58 Cardiothoracic and vascular system

Fibrosing mediastinitis • It then ascends to the right of the aorta


and the thoracic duct, entering the thorax
• Progressive hilar and mediastinal
behind the median arcuate ligament
infiltration by fibrous tissue
• The right second to 12th intercostal veins
• Idiopathic, or secondary to tuberculosis,
drain into it (the right first intercostal
histoplasmosis, sarcoidosis, radiotherapy,
vein drains directly into the right
drug reaction, retroperitoneal fibrosis
brachiocephalic vein)
• Causes progressive encasement of
• The right posterior intercostal arteries
mediastinal structures, with narrowing
cross behind it
of the trachea, main bronchi, SVC,
• At the T5 level the azygous vein arches
pulmonary veins, pulmonary artery, left
anteriorly over the right main bronchus to
atrium and brachiocephalic veins
drain into the SVC
• Often presents with symptoms of airway
• The hemiazygous vein commences
compromise or SVC obstruction
anterior to the L2 vertebral body as
Imaging features a branch of the left renal vein or left
ascending lumbar vein
CXR
• It ascends to the left of the aorta and the
• Non-specific, may show calcified hilar and
thoracic duct
mediastinal nodes
• At the T9 level it crosses laterally, draining
CT into the azygous vein
• Localised or diffuse soft-tissue mediastinal • The accessory hemiazygous vein receives
mass the left fourth to eighth intercostal veins
• Commonly contains calcifications (the left first to third intercostal veins
• Obliterates fat planes and compresses drain directly into the left brachiocephalic
adjacent structures vein) and descends on the left side of the
vertebral column
Pneumomediastinum • At the T8 level it crosses laterally draining
into the azygous vein
• Causes
–– Asthma (commonest cause) SVC obstruction
–– Oesophageal perforation
–– Intraperitoneal or retroperitoneal free Causes of SVC obstruction
air, which can track superiorly
–– Bronchial perforation Malignant (80% of cases)
Imaging features • Bronchogenic carcinoma
• Lymphoma
CXR • Mesothelioma
• Streaky translucencies, best seen along the
left heart border Benign (20% of cases)
• Subcutaneous emphysema in the neck • Central venous catheters
• Continuous diaphragm sign • Fibrosing mediastinitis
• Air outlines thymus (‘thymic sail’ sign) • Constrictive pericarditis
seen in children • Ascending aortic aneurysm

Venous pathology • Presents with upper extremity swelling and


Anatomy dyspnoea, and dilated chest wall collateral
veins
• The azygous vein commences anterior
• Blood drains to right heart via azygous vein
to the L2 vertebral body as a branch of
and other collaterals
the IVC or a continuation of the right
• Treatment of malignant causes is with
ascending lumbar vein

Ch-01.indd 58 8/7/2010 3:34:00 PM


Mediastinum 59

radiotherapy, chemotherapy or placement • Venous blood reaches heart via the


of self-expanding stents azygous or hemi-azygous veins
• Benign causes are more difficult to traverse • Hepatic veins drain directly into right
with stents atrium

Persistent left SVC Imaging features


• Commonest congenital venous anomaly CXR
• Failure of obliteration of the left anterior • Dilated azygous vein
cardinal vein • Widened paravertebral stripes
• Occurs in 0.5% of the general population CT
but in up to 4% of patients with congenital • Dilated azygous veins and azygous arch
heart disease
• 90% connect to the right atrium via the Causes of azygous vein
coronary sinus enlargement (> 1 cm)
• 10% connect to the left atrium (right-to-left
shunt)
• Inspiration
Azygous continuation of IVC • Right heart failure
• Associated with total anomalous • Portal hypertension
pulmonary venous drainage, polysplenia • Constrictive pericarditis
and rarely asplenia • SVC or IVC obstruction
• Suprarenal portion of the IVC is absent • Azygous continuation of IVC

Ch-01.indd 59 8/7/2010 3:34:00 PM


1.12 Aorta

Anatomy • The upper two pairs arise from the


costocervical trunk of the subclavian artery
Thoracic aorta • The anterior intercostal arteries arise from
• Composed of the aortic root and the internal mammary arteries
ascending, arch and descending parts • The descending aorta passes through
• Originates at the aortic valve (which lies diaphragm behind the median arcuate
posterior to pulmonary trunk) ligament at T12 to become the abdominal
• The coronary arteries arise from the three aorta
sinuses of Valsalva above the valve cusps
• Left coronary ostia from left sinus, right
Abdominal aorta
coronary from right sinus. The posteriorly • Retroperitoneal course from the hiatus to
placed non-coronary sinus does not the bifurcation at L4
normally give rise to a coronary artery • Midline unpaired branches
• The left coronary artery arises from the left –– Coeliac artery (at T12 level)
sinus and the right coronary artery from –– superior mesenteric artery (at L1 level)
the right sinus. No vessel arises from the –– inferior mesenteric artery (at L3 level)
non-coronary cusp (the most posterior • Lateral paired branches
cusp) –– Inferior phrenic arteries (at T12 level)
• The thoracic aorta arches posteriorly from –– Middle adrenal arteries (at L1 level)
right to left over the pulmonary trunk, left –– Renal arteries (at L2 level)
main bronchus and left pulmonary artery –– Gonadal arteries (at L3 level)
• The origin is invested in a pericardial –– Lumbar arteries (two pairs)
sheath with the pulmonary trunk
• The aortic arch lies at the level of T4 and Congenital anomalies of
gives off the innominate, left common
carotid and left subclavian arteries
the aortic arch
(variations are common) Left aortic arch branch
• These are crossed anteriorly by the left patterns
brachiocephalic vein
• Together account for up to 99% of cases
• The left superior intercostal vein passes
• Classical pattern (innominate, left carotid,
anterior to arch as it drains into the left
left subclavian arteries) (70% of cases)
brachiocephalic artery (the ‘aortic nipple’
• Left common carotid common artery
on CXR)
origin with the innominate artery (12% of
• The aortic arch becomes the descending
cases)
aorta at the isthmus where the ligamentum
• Left common carotid artery arises from
arteriosum attaches, making this part of
the innominate artery (bovine arch) (9% of
aorta prone to deceleration injuries
cases)
• The descending aorta courses inferiorly
• Left vertebral artery arises directly from
through the posterior mediastinum with
the arch (5% of cases)
the spinal column to its right and the lung
• Aberrant right subclavian artery (0.4–2% of
to its left
cases)
• The descending aorta gives off nine pairs
• Common origin of left common carotid
of posterior intercostals arteries (the third
and subclavian arteries (1% of cases)
to 12th intercostal arteries)
• Separate origin of right common carotid
and subclavian arteries (1% of cases)

Ch-01.indd 60 8/7/2010 3:34:00 PM


Aorta 61

Right aortic arch branch side of the trachea; the right arch is higher
than left
patterns • Each arch gives a single subclavian and
• Together account for up to 1–2% of cases common carotid artery
• Mirror-image branching (in order: left • The subclavian arteries arise posterior to
innominate artery, right common carotid the carotids
artery, right subclavian artery (65% of • The right arch crosses behind the
cases) oesophagus to join the left arch as the
• Aberrant left subclavian artery (in order: descending thoracic aorta
left common carotid artery, right common
carotid artery, right subclavian artery, Imaging features
aberrant left subclavian artery (35% of CT and MRI
cases) • Four vessels evenly spaced around trachea
– four artery’ sign on axial image
Double aortic arch
• A rare anomaly Contrast swallow
• Lateral view shows posterior indentation
Left arch with aberrant right • Frontal view shows bilateral indentations
subclavian artery (‘reverse S-shape’)
• Right subclavian artery arises as last Coarctation of the aorta
branch of the aortic arch
• Eccentric luminal narrowing caused by
• Associated with congenital heart defects in
infolding of the aortic wall
10–15% of cases
• Most commonly a focal narrowing distal to
• Usually asymptomatic, can rarely cause
the origin of the left subclavian artery
‘dysphagia lusoria’
• Blood flow to the descending aorta via
Imaging features intercostal collateral vessels
• Rarely a long segment of tubular
CXR
hypoplasia, which presents in neonates
• Right paratracheal opacity
with heart failure and congenital cardiac
• Opacity posterior to trachea on lateral CXR
anomalies
CT
• Last and most posterior vessel to come off Associations in tubular hypoplasia
aortic arch type
• Origin is commonly dilated (Kommerell’s
diverticulum) • Bicuspid aortic valve
• Traverses the mediastinum • Intracardiac malformations
–– Posterior to oesophagus in 80% of cases • Turner’s syndrome (45XO)
–– Between oesophagus and trachea in • Berry aneurysms
15% of cases
–– Anterior to trachea in 5% of cases

Double aortic arch Imaging features


• Most common and serious vascular ring CXR
• Complete vascular ring around trachea • ‘Figure three’ sign of pre- and post-stenotic
and oesophagus dilatation
• Presents in neonates or children with • Inferior rib notching (of third to ninth ribs)
respiratory distress and stridor from dilated intercostal collaterals
• The descending aorta is left-sided in 75% • Unilateral, right-sided rib notching in the
of cases right aortic arch with coarctation proximal
• The ascending aorta divides into separate to an aberrant left subclavian artery
right and left arches, which pass on either

Ch-01.indd 61 8/7/2010 3:34:00 PM


62 Cardiothoracic and vascular system

• Unilateral left-sided rib notching in the left


aortic arch with coarctation proximal to an Risk factors
aberrant right subclavian artery
• Hypertension (60–90% of cases)
CT angiogram
• Pregnancy
• Modality of choice for follow-up after stent
• Coarctation
insertion
• Bicuspid aortic valve
MRI (modality of choice pre-stent and • Adult polycystic kidney disease
postsurgery) • Marfan’s syndrome
• Modality of choice before and after stent • Ehlers–Danlos syndrome
insertion • Vasculitis, e.g. systemic lupus
• Clearly depicts extent of coarctation, erythematosus, rheumatoid arthritis,
quantifies the shunt and shows the Takayasu’s arteritis
collateral vessels
• Complications include re-coarctation and
aneurysm formation
Type A dissection
Pseudocoarctation • High early mortality if not treated promptly
• Requires surgical intervention (surgical
• Elongation of the ascending aorta mortality is 10–35%)
(‘high aortic arch’) • Endovascular approach is a new treatment
• Arch buckles at ligamentum arteriosum option being trialled
insertion • Complications
• No pressure gradient across buckle –– Dissection extension into the coronary
• No rib notching arteries (causes myocardial infarction)
• Anteromedial deviation of aorta and –– Dissection extension into the common
oesophagus carotid arteries (causes stroke)
• Associated with hypertension, bicuspid –– Rupture into the pericardial sac (causes
aortic valve, patent ductus arteriosus, cardiac tamponade)
atrial septal defect, ventricular septal –– Aortic valve disruption (causes acute
defect and a single ventricle aortic regurgitation in 60% of cases)

Type B dissection
• Best treated initially with blood pressure
control
Aortic dissection • Often become aneurysmal over time,
• Caused by an intimal tear; blood dissects requiring endovascular stenting or surgery
into the tunica media, creating a false • Endovascular or surgical intervention
lumen is needed if the dissection becomes
• Central tearing chest pain radiating symptomatic secondary to distal
between the scapulae compromise, i.e. mesenteric, renal or limb
• Asymmetric peripheral pulses and blood ischaemia
pressures (in 60% of cases)
• Stanford classification
Imaging of dissection
–– Type A (70% of cases): involves the CXR
ascending aorta with or without • Normal in 25% of cases
involvement of the arch • Mediastinal widening in 60% of cases
–– Type B (30% of cases): begins distal to • Ill-defined aortic arch contour
the origin of the left subclavian artery • Inward displacement of intimal
and involves only the descending aorta calcification (> 1 cm)

Ch-01.indd 62 8/7/2010 3:34:00 PM


Aorta 63

Multidetector CT –– Pacemaker leads, surgical clips


• > 95% accurate –– Curvilinear aortic motion artefacts
• Modality of choice in the acute setting (ECG-gating improves this)
• Contrast-enhanced acquisition shows • Structures adjacent to ascending aorta
dissection as two opacified channels –– Aortic valve cusps
separated by an intimal flap –– Superior pericardial recess
• ECG-gating is technique of choice for –– Residual thymic tissue
assessing extension into the aortic root • Structures adjacent to descending aorta
and coronary arteries –– Left superior intercostal vein
• Features of false lumen –– Left inferior pulmonary vein
–– Larger than true lumen –– Ductus diverticulum
–– Anterior in the ascending aorta, –– Kommerell’s diverticulum
posterolateral in the descending aorta –– Atelectatic lung
–– Rarely thrombosed in the acute setting –– Aneurysm with intraluminal thrombus
–– Delayed enhancement compared with
true lumen Variants of aortic dissection
–– Partial luminal thrombosis is an Intramural haematoma
adverse prognostic sign on follow-up of • Haemorrhage of the vasa vasorum into the
type B dissection media layer
MRI • Can progress to an intimal tear and the
• T1 spin-echo shows blood as black; flap classic dissection pattern
seen as a high-signal line • Non-contrast CT shows a crescent of high
• Post-gadolinium (superior to black blood attenuation within the aortic wall
sequence): contrast is bright, flap seen as • Cannot be diagnosed with aortography
dark line • Requires emergency surgical repair if it
involves the ascending aorta
Transoesophageal echocardiogram
• Usually managed conservatively if it
• > 90% accurate
involves the descending aorta; it can
• High resolution imaging which clearly
disappear following antihypertensive
depicts the intimal flap
treatment
• Aortic valve, ascending and descending
• Differential diagnosis is thrombosed
aorta are well seen
false lumen of dissection (intramural
• Aortic arch and branches often obscured
haematoma does not spiral around aorta)
by trachea – ‘blind spot’
Arch aortogram Penetrating atherosclerotic ulcer
• Traditional technique but currently only • Atherosclerotic lesion with ulceration
used prior to endovascular repair penetrating the intima layer and
haematoma formation within the media
CT pitfalls in aortic dissection • Occurs in elderly patients with
False negatives hypertension and atherosclerosis
• Most commonly involves the descending
• Insufficient contrast enhancement of
thoracic aorta
aortic lumen
• CT shows a focal ulcer with subintimal
• Too slow an injection rate, poor timing of
haematoma
contrast bolus
• May progress to transmural rupture (in
False positives 40% of cases) or aortic dissection
• Streak artefacts • Usually treated with an endovascular
–– Contrast in the SVC aortic stent graft
–– Contrast in left brachiocephalic vein
(left arm injection)

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64 Cardiothoracic and vascular system

Thoracic aortic aneurysm • Mainly affects the aorta and its major
branches
• Aetiology • May also involve the pulmonary, coronary
–– Atherosclerosis and renal arteries
–– Cystic medial necrosis (in Marfan’s
syndrome or Ehlers–Danlos syndrome) Imaging features
–– Infective (bacteria or syphilitic) Catheter angiogram
–– Aortopathy (bicuspid aortic valve) • Patchy stenoses and occlusions
• Fusiform dilatation (saccular dilatations
CT and MRI
are mycotic or traumatic)
• Enhancing mural thickening and luminal
• Most commonly involves the distal arch or
narrowing
the descending aorta
• Progressive dilatation with aortic
regurgitation, rupture Abdominal aortic aneurysm
• Predisposes to aortic dissection (AAA)
• Local compressive effects produce
dysphagia and stridor • True aneurysm involving all three layers of
• Surgical or endovascular repair is the arterial wall
indicated if diameter > 5.5 cm • Affects 5% of men and 1% of women over
• Risk of rupture if diameter > 6 cm is 10% the age of 65
per year • Defined as aortic diameter > 3 cm or
double the normal size
Imaging features • Elective repair considered if diameter
CXR > 5 cm
• Mediastinal mass with curvilinear • 90% arise below the origin of the renal
calcifications arteries (infra-renal)
• 80% have occlusion of the inferior
CT mesenteric artery and lumbar arteries
• Mural thrombus and calcifications
• Majority contain mural thrombus, which
may protect it from rupture
Mycotic (non-syphilitic) aneurysm • Endovascular repair is standard technique
in majority (65%) of cases
• Infective destruction of intima and
media CT signs of AAA rupture
• Bacterial seeding via the vasa vasorum • Adjacent retroperitoneal haematoma
• Risk factors include septicaemia from (typically left sided)
bacterial endocarditis, intravenous • Active extravasation of contrast agent
drug use and immunosuppression
• Majority occur in the ascending aorta Signs of impending AAA rupture
near the sinus of Valsalva
• High risk of rupture • Diameter > 7 cm
• Growth > 5 mm in 6 months
• Abdominal pain
• Crescent of high attenuation within
Takayasu’s arteritis wall
• Chronic, granulomatous, large-vessel
vasculitis
• Most common in young Asian females Aortoenteric fistula
• Intimal proliferation leading to stenoses • Late complication of AAA or aortic
and occlusions reconstructive surgery (about 10 years
• Diminished or absent pulses postoperative)

Ch-01.indd 64 8/7/2010 3:34:00 PM


Aorta 65

• Most fistulas involve the third part of the • > 50% of patients with AAA are suitable for
duodenum EVAR
• Present with abdominal pain, • Endograft is used to exclude blood flow
haematemesis and melena from the aneurysm sac
• Procedure performed via bilateral femoral
Imaging features artery cut-downs
CT • Graft is preloaded on a special delivery
• Intraluminal and periaortic extraluminal system
gas • Deployed under fluoroscopic guidance
• Contrast extravasates into the bowel, and above and below the sac
therefore contrast agent should not be • EVAR has a lower 30-day mortality (1.6%)
administered before CT scanning than surgical repair (4.6%)
• At 4 years it has a high re-intervention rate
Inflammatory aneurysm (up to 40%)
• AAA with surrounding inflammation and • EVAR is more expensive than conventional
fibrotic adhesions surgical repair
• Caused by an exaggerated immune
response to atherosclerosis EVAR exclusion criteria
• Associated with hypertension, smoking
and diabetes • Neck angulation > 60°
• Accounts for 5–10% of all AAAs • Neck length < 15 mm
• Majority are infra-renal • Iliac arteries diameter < 6 mm
• Males affected more often than females
• Median age of 60
• Raised ESR Complications
• Majority are symptomatic, causing fever, • Endoleak (blood leak into old aneurysm
back pain and weight loss sac)
• Low risk of rupture compared with –– Type 1: persistent perigraft channel
atheromatous aneurysm (because of ineffective seals at top and
• Adhesions most often involve the bottom); requires urgent intervention
duodenum, IVC or left renal vein –– Type 2: retrograde flow into sac via
• 10–20% have obstructive uropathy from the inferior mesenteric artery or
ureteric involvement lumbar arteries; usually managed
Imaging features conservatively
–– Type 3: a major defect in graft fabric
Ultrasound (mechanical failure); treated with an
• Sonolucent halo surrounding aorta additional graft
CT –– Type 4: a minor leak seen through
• Thick enhancing tissue rind around porous graft fabric; usually managed
aneurysm, with or without calcification conservatively
• Adjacent inflammation subsides following –– Type 5: sac increasing in size but no
surgical repair endoleak visible (endotension)
• Other complications
Endovascular aneurysm –– Graft thrombosis
–– Graft kinking
repair (EVAR) –– Graft infection
• An alternative to surgical repair of AAA –– Shower embolism
• Requires favourable vascular anatomy –– Colonic necrosis

Ch-01.indd 65 8/7/2010 3:34:00 PM


1.13 Vascular disease

Peripheral vascular disease artery is not suitable), followed by wire and


catheter insertion: Seldinger’s technique
• Usually results from atherosclerosis; • Contrast is injected via the catheter by
multiple risk factors including genetics, hand or pump, and images are acquired
smoking and diet • Computer subtracts an initial non-contrast
• Atherosclerosis is an arterial disease but ‘mask’ image from a subsequent contrast-
may also effect veins exposed to arterial enhanced image
pressures • Contrast resolution is superior to screen-
• Involvement of vessels is usually film arteriography as bone, soft tissue and
segmental and progressive gas shadows are removed
• Can cause stenoses, occlusions and • 1024 × 1024 matrix has a spatial resolution
aneurysms of three line pairs per millimetre
• Clinical presentation is with intermittent • Patients must remain still and hold
claudication, rest pain or ulcer disease their breath for imaging of the
• Commonest cause of claudication is thoracoabdominal and pelvic vessels
aortoiliac disease, and the treatment of • Buscopan is routinely used to prevent
choice is balloon angioplasty followed by peristalsis
stenting • Very slight movements can be corrected
• Femoropopliteal occlusive disease is using ‘pixel shifting’
more common than iliac disease, and the • Contrast volumes and flow rates vary
treatment of choice is angioplasty depending on the territory being assessed
• Angioplasty for infrapopliteal disease is –– Aortic arch: 25–40 mL at 15–20 mL/
usually reserved for ischaemic foot ulcers second
or rest pain –– Abdominal aorta: 25 mL at 12–15 mL/
• In multilevel vascular occlusive disease, second
treatment should commence at the highest –– Coeliac artery or superior mesenteric
level lesion to improve ‘in-flow’ artery: 15 mL at 8–10 mL/second
• High angioplasty success rates are • Demonstration of common iliac vessel
achieved by treating short segment disease bifurcation is best achieved with
with good ‘run-off’, i.e. 3 cm for iliac contralateral oblique views, e.g. right
arteries and < 10 cm for femoral arteries anterior oblique view for left iliac arteries
• Stenoses longer than 10 cm give less • Demonstration of femoral vessel
favourable results bifurcation is best achieved with ipsilateral
• 20% of patients with claudication who oblique views, e.g. left anterior oblique
undergo angioplasty eventually require view for left femoral arteries
amputation • Demonstration of distal vessels of the
foot is best achieved by using biplane
Diagnostic angiography arteriography (lateral and posteroanterior
projections)
Digital subtraction • Pseudo-occlusions can be produced by
angiography excessive plantar flexion and external
• Access is via puncture of the common compression
femoral artery (or the brachial, radial or • Peripheral vasodilators may be used to
popliteal artery if the common femoral vasodilate for pressure gradient recordings

Ch-01.indd 66 8/7/2010 3:34:00 PM


Vascular disease 67

• Stenosis causes an increase in velocity and


Catheters spectral broadening
• A significant stenosis causes at least a
• High flow with side holes for central doubling of velocity
vessels • Loss of triphasic waveform downstream
• Low flow with end hole for selective of flow can be used to make an indirect
vessels inference about upstream stenosis or
• Higher flow rates can also be achieved occlusions
using a shorter catheter with a larger
CT angiogram
diameter
• The current method of choice for imaging
the thoracoabdominal aorta, common
Types
femoral arteries and carotid arteries
• Pigtail, for the aorta and pulmonary
• Used both in planning and in surveillance
artery
of abdominal aortic aneurysm repair
• Cobra, for mesenteric, renal and
• Can assess in-stent stenosis in vessels
contralateral iliac arteries
> 3 mm in diameter
• Simmons, for the mesenteric arteries
• Increasingly used in assessing peripheral
and arch vessels
vascular disease. Isotrophic high spatial
• Microcatheters, for coaxial subselection
resolution is achievable with 16-detector
• Straight, for runoff vessels
scanners and above, which enables
assessment of pedal vessels
• Presence of calcium causes blooming
Imaging features artefact and makes accurate quantification
MR angiogram of stenoses difficult
• Contrast-enhanced MR angiography is Endovascular ultrasound
a widely used technique for peripheral • Expensive and needs larger arteriotomy;
vascular and carotid imaging not commonly used
• It is faster and has a better signal-to-noise • Miniature ultrasound probe is attached to
ratio than time of flight angiography; a catheter tip and positioned in the target
however, it tends to overestimate stenosis vessel lumen using a guide wire
severity • Catheter is slowly slid backwards as images
• Uses T1-shortening effects of gadolinium- are acquired
based contrast agents • Can be used to assess atheromatous
• Most metallic stents (except the Nitinol plaque volume and morphology, the
type) exhibit artefacts that preclude effects of angioplasty and stenting, and in
MR angiographic evaluation of in-stent planning endovascular treatments
patency • Can underestimate intimal flaps by
• Imaging using contrast kinetics is used in re-opposing them to the wall
evaluating run-off vessels and vascular
malformations General principles of
• Increasing incidence of nephrogenic percutaneous angioplasty and
systemic fibrosis from free gadolinium has stenting
lead to emergence of new non-contrast
• French size refers to outer circumference
enhanced sequences such as steady state
in catheters, inner circumference in
free precession (bright blood imaging)
sheaths
and ECG-gated flow spoiled (fresh blood
• Angiograms are initially performed to act
imaging)
as a ‘road map’
Peripheral vascular ultrasound • Stenotic lesion is crossed using a wire
• Normal peripheral arterial waveform is • Heparin in a single dose of 5000 IU is
triphasic routinely administered

Ch-01.indd 67 8/7/2010 3:34:00 PM


68 Cardiothoracic and vascular system

• Angioplasty balloon catheter of an disease to help in healing because the


appropriate size is railroaded over wire duration of patency is shorter
and across the stenosis • Self-expanding stents with improved
• The balloon or stent diameter is chosen to flexibility are sometimes used for calcified
be 5–10% oversized or long lesions
• Balloon is inflated for 30 seconds to
1 minute (for up to 2 minutes if there is Guide to approximate balloon and
resistance) stent sizes
• Residual stenosis < 20% is considered a
technical success • Aorta: 12–15 mm
• Higher grades of residual stenosis or • Renal arteries: 4–6 mm
angioplasty complications are indications • Common iliac arteries: 8–10 mm
for stent placement • External iliac arteries: 7–8 mm
• Non-covered, self-expanding or balloon- • Common femoral and superficial
mounted stents are commonly used femoral arteries: 5–6 mm
• Stents re-occlude acutely via in-stent • Popliteal arteries: 4–5 mm
thrombosis and subacutely by the process • More distal vessels: 2–3 mm
of neointimal hyperplasia
Choose a diameter of 10% more than the
Iliac artery angioplasty and vessel diameter
stenting
• Iliac artery percutaneous transluminal
angioplasty (PTA) is technically successful
in around 95% of patients
Intra-arterial thrombolysis
• Short (< 3 cm), non-calcified stenotic • Indications are an ‘acute white leg’ caused
segments are more effectively treated than by emboli from a distant source or an in
occlusions situ thrombus
• Percutaneous transluminal angioplasty • Contraindications (absolute) are surgery
and stenting is the treatment of choice for in previous 6 weeks, a recent stroke and a
iliac artery disease, with outcomes similar known bleeding disorder
to surgery • Alteplase or recombinant tissue
• The addition of stenting improves long- plasminogen activator (rt-PA) is used
term patency rates (90% patent at 1 year • Side effects include bleeding, allergic
versus 75%; 80% patent at 4 years versus reaction, nausea and vomiting, and
65%) reperfusion injury with compartment
• Success rates of angioplasty and stenting syndrome in legs or arms
for stenoses below the inguinal ligaments Delivery technique
are lower than for those in the iliac arteries • Routine angiographic approach: place
Femoropopliteal angioplasty multi-hole catheter in thrombus or clot
• Inject a bolus dose (5–10 mg) of alteplase
and stenting with short bursts in length of clot to give an
• Common femoral artery disease is best extra mechanical effect
treated by endarterectomy but if necessary • Re-site the catheter into the clot and
it can be done via a contralateral approach continue slow rate infusion with check
• Superficial femoral artery disease angiograms every 4–6 hours
is treated as per the TransAtlantic • If bleeding occurs, stop the infusion and
InterSociety Consensus (TASC) guidelines, call for help
and angioplasty is best • If check angiogram shows clearance,
• Subintimal angioplasty can be performed look for a local cause such as stenosis or
in long occlusions in patients with ulcer occlusion, and treat appropriately

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Vascular disease 69

• In cases of popliteal aneurysm, surgery or • Presents with the ‘five Ps’: pain,
endovascular stent graft are indicated paraesthesia, pulselessness, pallor,
• If no local cause is found, assess for a perishing with cold
central distal cause such as from the aorta • High risk of limb loss without emergency
or perform an echocardiogram to look for treatment
a cardiac cause • Treatment is with surgery (thrombectomy
or bypass grafting) or using intra-arterial
Complications of PTA and thrombolysis (involving an infusion for up
to 48 hours)
stenting
• Puncture: haematoma, pseudoaneurysm,
Renal failure induced by
arteriovenous fistula, infection contrast material
• Contrast-related: renal failure, allergic • Increased risk with pre-existing renal
reaction insufficiency or diabetes
• Catheter-related: embolism, stroke, • Risk can be reduced by using non-ionic
dissection contrast media or carbon dioxide contrast
• Therapy-related: haemorrhage and by pre-procedural hydration
• Mortality is 0–1.2%
• Stent infection is rare
Contrast reactions and
treatment
Femoral artery
Urticaria
pseudoaneurysm • Supportive treatment if scattered or
• A false aneurysm, i.e. an aneurysm that transient
does not involve all three layers of the • For a protracted reaction consider
artery wall antihistamine (intramuscularly, orally or
• High-pressure leak contained by adjacent intravenously)
tissues and haematoma • If profound consider adrenaline (1:1000)
• Occurs in < 1% of diagnostic angiograms 0.1–0.3 mg intramuscularly
and in 3–5% of interventional procedures;
higher risk with large-bore catheters Bronchospasm
• Ultrasound-guided compression or • Oxygen by mask (6–10 L/minute)
thrombin injection can be used to treat • Beta-2-agonist metered dose inhaler (two
narrow-necked aneurysms or three deep inhalations)
• If normotensive, give adrenaline (1:1000)
Imaging features 0.1–0.3 mg intramuscularly
Ultrasound • If hypotensive, give adrenaline (1:1,000)
• Echo-poor ‘sac’ seen adjacent to the 0.5 mg intramuscularly
femoral artery
• The sac may contain internal echoes Laryngeal oedema
(because of partial thrombosis) • Oxygen by mask (6–10 L/minute)
• Swirling blood flow seen within the sac • Adrenaline (1:1000) 0.5 mg
using colour Doppler with inflowing and intramuscularly, repeated as needed
outflowing half-moons (‘ying–yang’ sign) Hypotension with bradycardia
• Communicating ‘neck’ links parent artery
(vasovagal reaction)
to the sac
• Elevate patient’s legs
Acute limb ischaemia • Oxygen by mask (6–10 L/minute)
• Secondary to embolisation of plaque • Atropine 0.6–1.0 mg intravenously,
debris, which tends to lodge at arterial repeated if needed after 3 minutes (up to a
bifurcations or areas of pre-existing maximum of 3 mg in total)
narrowing • Intravenous fluids rapidly (normal saline
or lactated Ringer’s solution)

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70 Cardiothoracic and vascular system

Generalised anaphylactoid reaction minutes before procedure; initially 2 mg


• Call for resuscitation team increased in steps of 1 mg
• Suction airway if needed • Cautions: cardiac or respiratory disease,
• Elevate patient’s legs if hypotensive myasthenia gravis
• Oxygen by mask (6–10 L/minute) • Contraindications: unstable myasthenia
• Adrenaline (1:1,000) 0.5 mg gravis, severe respiratory depression, acute
intramuscularly pulmonary insufficiency
• Histamine-1 (H1) blocker, e.g. • Side effects: hypotension, arrhythmias,
diphenhydramine 25–50 mg intravenously convulsions, confusion, urinary retention,
injection site reactions
Contrast medium extravasation
• Elevate affected limb Glyceryl trinitrate
• Apply ice packs to affected area • Indication: local vasodilatation
• If symptoms do not resolve quickly, admit • Mechanism of action: relieves smooth
and monitor muscle spasm (both venous and arterial)
• If skin or soft tissues are threatened • Dose: 100–200 μg boluses; intra-arterial
consider surgical treatment infusion rate, 15–20 μg/minute
• Cautions: monitor blood pressure
Pulse oximetry monitoring • Contraindications: hypotension, severe
• Causes of a low reading aortic stenosis, recently taken nitrate-
–– Low PaO2 based medication for erectile dysfunction
–– Cold extremities
–– Hypotension Glucagon
–– Dyshaemoglobinaemia • Indication: reduces intestinal peristalsis
–– Skin pigmentation, e.g. jaundice • Mechanism of action: smooth muscle
–– Nail varnish relaxation (cyclic AMP-mediated)
–– Motion • Dose: 1 mg
• Cautions: insulinoma,
Pharmacology phaeochromocytoma (may induce a
hypoglycaemic or hypertensive crisis)
Lignocaine
Buscopan (hyoscine
• Indication: local anaesthesia
• Mechanism of action: reversible blockage
butylbromide)
of nerve conduction • Indication: antispasmodic agent, reduces
• Dose: 10 mg/mL (1%); maximum dose: intestinal motility
20 ml • Mechanism of action: antimuscarinic
• Cautions: allergy, impaired cardiac agent
conduction • Dose: 20 mg/mL; maximum dose: 160 mg
• Contraindications: hypovolaemia, • Contraindications: myasthenia gravis,
complete heart block paralytic ileus, pyloric stenosis, prostatic
• Side effects: confusion, respiratory enlargement
depression, convulsions, hypotension and • Side effects: antimuscarinic
bradycardia
Atropine
Midazolam • Indication: treatment of bradycardia
• Indication: conscious sedation • Mechanism of action: inhibits
• Mechanism of action: facilitates action of acetylcholine
gamma-aminobutyric acid(GABA), the • Dose: 0.6–1.2 mg intravenously; maximum
major inhibitory neurotransmitter; half- dose: 3 mg
life of 2 hours • Contraindications: closed-angle glaucoma
• Dose: slow intravenous injection • Side effects: anticholinergic
(approximately 2 mg/minute) 5–10

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Vascular disease 71

Peripheral vascular Popliteal artery


anatomy • The popliteal artery descends in the floor
of the popliteal fossa deep to the popliteal
Iliac artery vein
• The aorta bifurcates into the right and left • It has several genicular branches, which
common iliac arteries serve as collateral pathways when the
• Within the pelvis each common iliac artery femoral artery is occluded
divides into the internal and external iliac • It divides at the lower border of
arteries popliteus muscle into anterior tibial and
• The internal iliac artery supplies the tibioperoneal trunks
organs of the pelvis and buttock
• Reduced blood flow via the internal iliac
Run-off vessels
artery leads to buttock claudication in • The anterior tibial artery descends anterior
patients with poor collateralization; it can to the interosseous membrane deep to the
also present as erectile dysfunction tibialis anterior and extensor digitorum
• The external iliac artery gives off the muscles; it passes in front of the ankle joint
deep circumflex iliac artery and the deep to become the dorsalis pedis artery
external pudendal artery • The tibioperoneal trunk divides after a few
centimetres into the posterior tibial and
Femoral artery peroneal arteries
• The external iliac artery exits the pelvis • The posterior tibial artery descends
to become the common femoral artery between deep and superficial muscle
as it crosses beneath the midpoint of the compartments then passes behind the
inguinal ligament medial malleolus deep to the flexor
• The femoral vein lies medial to the retinaculum, dividing into the medial and
common femoral artery; the femoral nerve lateral plantar arteries
lies laterally to the common femoral artery • The peroneal artery descends close to
• The common femoral artery descends the fibula. It supplies branches to the
superficially in the femoral triangle and lateral compartment before dividing into
gives three superficial branches: the calcaneal and lateral malleolar branches
inferior epigastric artery, the external
pudendal artery and the circumflex iliac Non-atherosclerotic
artery
• After a short distance it gives a deep Pathology
branch, the profunda femoris artery, Buerger’s disease
which leaves through the floor of the
• Not common among Caucasians
triangle
• Non-necrotising panarteritis of the small
• The profunda femoris artery has medial
and medium-sized peripheral arteries, in
and lateral circumflex femoral branches
which there is luminal occlusion by highly
and deep perforating branches supplying
cellular thrombus
adductor and flexor compartments
• Strong association with smoking
• The common femoral artery continues as
• < 2% of cases occur in women
the superficial femoral artery
• Presents with claudication symptoms in
• At the apex of the femoral triangle the
young patients
superficial femoral artery enters the
adductor canal anterior to the femoral vein Imaging features
and winds medially round the thigh
Angiography
• It becomes the popliteal artery at the
• Multifocal stenoses and occlusions of the
adductor hiatus
calf and forearm arteries
• ‘Corkscrew’ collaterals are characteristic

Ch-01.indd 71 8/7/2010 3:34:01 PM


72 Cardiothoracic and vascular system

Raynaud’s syndrome • Medial head of the gastrocnemius muscle


arises laterally on femoral condyle
• Episodic pain affecting the fingers,
toes and extremities with phasic colour Polyarteritis nodosa
changes (white, blue, red)
• Pan-mural necrotizing vasculitis affecting
• Primary Raynaud’s is a cold-induced
small and medium-sized arteries, thought
spasm of smooth muscle in the arterial
to be a result of immune complex
wall
deposition
• Secondary Raynaud’s is a stenotic process
• Can be associated with hepatitis B and HIV
that produces arterial occlusion
infection
• Causes of secondary Raynaud’s syndrome
• Clinical symptoms of ischaemia from
–– Atherosclerosis (the commonest cause)
arterial branch occlusions
–– Thoracic outlet syndrome, e.g. a cervical
• Renal involvement in 70–80% of cases
rib
• Central nervous system involvement in
–– Connective tissue diseases, e.g.
10% of cases
scleroderma
• Treatment is with systemic corticosteroids;
–– Vasculitis, e.g. Takayasu’s arteritis,
aneurysms of polyarteritis nodosa may
systemic lupus erythematosus
resolve over time as remission occurs
–– Drugs, e.g. methysergide
• Prophylactic treatment of large aneurysms
–– Buerger’s disease
by covered stents or catheter embolisation
–– Trauma, e.g. ‘vibration white finger’
is considered to prevent rupture
–– Polyvinyl chloride exposure
Imaging features
Popliteal artery entrapment
Angiogram
syndrome • Multiple visceral organ arterial ectasias
• Caused by an anomalous anatomic and microaneurysms
relationship between the popliteal artery • Arterial occlusive lesions (in > 90% of
and surrounding musculotendinous cases)
structures
• Arterial compression by these structures Causes of small and medium-sized
occurs with plantar flexion and causes vessel vasculitis
intermittent claudication symptoms
• Compression most often caused by the
• Polyarteritis nodosa
medial head of the gastrocnemius muscle,
• Behçet’s syndrome
less commonly by the popliteus muscle or
• Churg–Strauss syndrome
a deep fibrous band
• Microscopic polyangiitis
• Usually affects healthy young men 20–40
• Kawasaki’s disease
years of age
• Rheumatoid vasculitis
• Aneurysmal dilatation can lead to
• Systemic lupus erythermatosus
thrombosis with distal embolisation and
vasculitis
acute limb ischaemia
• Wegener’s granulomatosis
Imaging features
Doppler, MR angiogram and catheter
angiogram Causes of large vessel vasculitis
• Medial deviation or angulation of the
artery in the popliteal region • Giant cell arteritis
• Mid-portion narrowing, which is most • Takayasu’s arteritis
marked in plantar flexion
• Occasionally post-stenotic dilatation

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Vascular disease 73

Vascular calcifications intermittent, and imaging tests are


sensitive only when there is evidence of
• Causes
active haemorrhage, such as tachycardia
–– Atherosclerosis
or systolic blood pressure < 100 mmHg
–– Diabetes
–– Chronic renal failure Imaging methods to identify
–– Hyperparathyroidism bleeding source
–– Hyperlipidaemia
–– Haemochromatosis (‘bronze diabetes’) Radionuclide scintigram
• Colloid sulphur or labelled red blood cell
scan
Acute gastrointestinal • Detects bleeding at rates as low as 0.1 mL/
haemorrhage minute
• Can image for up to 24 hours after
Upper gastrointestinal injection
bleeding CT angiogram
• Proximal to the ligament of Treitz • No oral contrast agent
• Accounts for 75% of cases • Thin-section, three-phase (pre-, arterial
• Peptic ulcer disease accounts for > 50% of and venous) scan
cases • Detects bleeding at rates as low as 0.3 mL/
• Other common causes are varices and minute
Mallory–Weiss tears • Hyperdense intraluminal contrast
• Presents with haematemesis and melaena extravasation
• Upper gastrointestinal endoscopy
Mesenteric angiogram
identifies bleeding source in 90% of cases
• Detects bleeding as low as 0.5 ml/minute
Lower gastrointestinal • Better sensitivity with carbon dioxide
bleeding contrast, vasodilators and anticoagulants
• Contrast blush or intraluminal
• Distal to the ligament of Treitz extravasation
• Accounts for 25% of cases
• Diverticular disease is the commonest
cause Embolisation
• Other common causes are angiodysplasia, • Deliberate occlusion of a vessel
neoplasia and colitis • Various agents are used
• Haemorrhoids account for 10% of cases • Permanent agents
• Haematochezia (red blood per rectum) –– Coils (provide a large thrombogenic
• Sigmoid or colonoscopy identifies surface)
bleeding source in 90% of cases, but –– Detachable balloons
colonic preparation is essential –– Absolute alcohol
–– Glue
Diagnosis and management • Temporary agents
• 80% of gastrointestinal bleeds will cease –– Autologous clot
spontaneously –– Gelfoam
• In the remaining 20% intervention is –– Polyvinyl alcohol (a particulate agent
required that causes thrombosis)
• Endoscopy should be the first-line • Coils should be slightly bigger than the
diagnostic and therapeutic procedure and lumen of the target vessel
can identify a bleeding source in > 90% of • Unintentional distal embolisation of
cases systemic vessels is more common at the
• Gastrointestinal bleeding is typically end of the procedure than the beginning

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74 Cardiothoracic and vascular system

Acute gastrointestinal • Failure (in 10% of cases)


• Premature menopause
haemorrhage • Hysterectomy
• Selective coil embolisation is the preferred
method Bronchial artery embolisation
• Has a high technical success rate for • The bronchial arteries originate directly
cessation of bleeding from the descending aorta, most
• Complications include failure and bowel commonly between the T4 and T6 levels
infarction needing surgery • Classically there are two arteries on the
left and one on the right, but variations are
Varicocele embolisation numerous
• 90% of varicoceles are left-sided • They supply the tracheobronchial tree
• Embolisation is used for symptom relief or • The left main bronchus marks their origin
associated infertility in most cases
• Access is via the right common femoral • A descending thoracic aortogram is
vein or the internal jugular vein performed prior to selective bronchial
• Left testicular vein drains into left renal angiography
vein • Polyvinyl alcohol (particles > 250–300
• Right testicular vein drains into the IVC, micrometres, to avoid passage into spinal
which is more difficult to access vessels and the bronchopulmonary
• Coils are deployed distal to the first joining anastomosis causing spinal cord and lung
branch at level of inguinal canal infarction) is the agent of choice
• If coils are too small they will embolise into
pulmonary arteries Complications
• Testicular vein spasm is a frequent • Chest pain (commonest)
problem • Dysphagia, caused by occlusion of
oesophageal branches
Uterine fibroid embolisation • Spinal cord ischaemia, caused by
• Widely accepted as a first-line treatment occlusion of radicular branches
for symptomatic fibroid disease as
an alternative to hysterectomy or Hepatic artery
myomectomy chemoembolisation
• Not suitable to treatment of pedunculated • Used in the treatment of hepatocellular
fibroids carcinoma, carcinoid and some liver
• Requires bilateral uterine artery metastases
embolisation (via the first or second • Lipoidal emulsified with cisplatin or
branches of the anterior division of the doxyrubicin, or polyvinyl alcohol is used
internal iliac arteries) to induce ischaemic • Deprives tumour of its nutrient source,
infarction causing ischaemic necrosis
• Polyvinyl alcohol is typically used (particle • Hepatic arteriography and portography are
size 300–500 micrometres) mandatory to evaluate vascular anatomy
• Angiographic endpoint is to-and-fro flow and patency prior to treatment
within the uterine artery • Portal vein thrombosis is not a
• Complete embolisation of both arteries is contraindication per se but selective
undesirable, since this will cause uterine arterial embolisation must be performed
necrosis in this setting
Complications • Non-target organ complications include
ischaemic cholecystitis, splenic infarction,
• Postoperative pain (severe for up to 24
bowel ischaemia, pulmonary embolism
hours)
and spinal cord ischaemia
• Post-embolisation syndrome
• Sepsis

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Vascular disease 75

Renal artery stenosis bifurcates at the C4 level into the internal


carotid artery (ICA) and the external
• Accounts for 2–5% of all cases of carotid artery (ECA)
hypertension • The ECA has no branches in the neck
• Haemodynamically significant when
> 70% luminal narrowing Branches of the ECA
• Causes reduced perfusion pressure at
the glomerulus, which stimulates renin
• Superior thyroid artery
production leading to a rise in angiotensin
• Ascending pharyngeal artery
and thus a rise in blood pressure
• Lingual artery
• Causes hypertension refractory to drug
• Facial artery
treatment
• Occipital artery
• Atherosclerosis is the cause in 70% of cases
• Posterior auricular artery
–– Commonest site is within 2 cm of the
• Temporal artery
ostium
• Maxillary artery (first branch is the
–– Stents are used because there is a high
middle meningeal artery, terminal
recurrence rate with angioplasty alone
branch is the sphenopalatine artery)
–– Quoted risk of renal artery rupture is 1%
• Fibromuscular dysplasia is the cause in
30% of cases
–– Mid- and distal vessel disease Atherosclerotic ICA stenosis
–– Good results with balloon angioplasty • Stenosis between 70% and 99% has
alone been shown in the North American
–– Indications for intervention are Symptomatic Carotid Endarterectomy
hypertension that is resistant to best Trial (NASCET) to benefit from
medical therapy, flash pulmonary endarterectomy surgery
oedema and acute or acute-on-chronic • Methods of assessing stenosis are
renal failure in patients with single Doppler ultrasound, MR angiography, CT
kidney angiography and catheter angiography
Imaging features Carotid Doppler ultrasound
Ultrasound
• Unilateral small kidney
Features of a normal ECA waveform
• MR angiography peak systolic velocity • ECA arises anteromedial to the ICA and
> 180 cm/second has side branches in the neck
• Ratio of peak systolic renal velocity to • Demonstrates the ‘temporal tapping’
aortic velocity > 3.5 phenomena
• ‘Parvus tardus’ interlobar artery Doppler • Has a high resistance waveform, i.e. low
waveform (acceleration time > 0.07 end-diastolic flow
second); this has a high specificity but a • Waveform has a characteristic notch and
low sensitivity for renal artery stenosis may dip below the baseline (diastolic flow
reversal)
MR angiogram and catheter angiogram
• Atherosclerosis (proximal stenosis) Features of a normal ICA waveform
• Fibromuscular hyperplasia (focal disease) • No side branches in the neck
• Can affect vessels at all levels, with a • No ‘temporal tapping‘ phenomena
stenotic or ‘beaded’ appearance and • Has a low resistance waveform, i.e. high
aneurysm formation end-diastolic flow
Features of a normal CCA waveform
Carotid imaging • Intima–media thickness, measured just
proximal to carotid bulb, is normally
Anatomy < 0.8 mm
• The common carotid artery (CCA)

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76 Cardiothoracic and vascular system

• Thickening is recognised as an • Accurate assessment is possible because it


independent risk factor for cardiovascular can evaluate plaque composition
events • Improved spatial resolution for depicting
• CCA waveform is higher resistance than dissection, intramural haematoma and
the ICA waveform but lower than the ECA aneurysm
waveform • Postoperative complications following
• Can normally dip below the baseline carotid endarterectomy are best imaged
by CT angiography, including in-stent
Criteria for diagnosing ICA stenosis stenosis
• Direct signs
–– Peak systolic velocity in the ICA Catheter angiography
> 230 cm/second • Definitive imaging test for distinguishing
–– ICA/CCA peak systolic velocity between high-grade stenosis and
gradient > 4 occlusion of the ICA
• Indirect signs • Carries an approximately 1% risk of stroke
–– Spectral broadening of ICA waveform
(‘filling in’ under the curve) Carotid dissection
–– ECA waveform shows increased • Accounts for 10–25% of strokes in younger
diastolic flow (‘ICA-like’) adults (< 45 years age)
Limitations of ultrasound • Increased risk with fibromuscular
dysplasia, Ehlers–Danlos syndrome,
• Operator-dependent and not reproducible
Marfan’s syndrome and homocystinuria
• Calcified plaques interfere with Doppler
• Most commonly occurs spontaneously or
interrogation
following minor, unrecalled trauma. Less
• Inability to visualise ICA lesions near skull
commonly caused by major trauma or
base
penetrating injuries
• Inability to interrogate origins of the great
• Presents with headache, stroke or an
vessels
ipsilateral painful Horner’s syndrome
• Problems distinguishing high-grade
stenosis from occlusion Imaging features
MR angiography MR angiogram, CT angiogram and
catheter angiogram
• Contrast-enhanced MR angiography is
• Commonest location is the cervical ICA at
technique of choice
C1–C2 level
• This is an accurate non-invasive technique
• Tapered narrowing of vessel over a few
for evaluating the extracranial carotid
centimetres
vessels
• Lumen typically reconstitutes within the
• Can image their origins at the aortic arch
bony carotid canal
• Has a tendency to overestimate the degree
• Intracranial extension is rare
of stenoses
Carotid stenting
Residual ‘trickle’ flow in high-grade
• Endovascular procedure that is an
stenosis
alternative to carotid endarterectomy
• Currently used in high or low surgically
• Residual flow: suitable for surgery inaccessible sites, for recurrence
• Absent flow: unsuitable for surgery after carotid endarterectomy and in
postoperative and post-radiotherapy necks
• Especially suited for patients with
CT angiography significant medical co-morbidities who are
• Non-invasive but involves radiation and considered too high an anaesthetic risk
iodinated contrast • Shortened hospitalisation and
convalescence times

Ch-01.indd 76 8/7/2010 3:34:01 PM


Vascular disease 77

• Similar 30-day stroke risk to that of carotid • Used as a measure in patients with portal
endarterectomy (approximately 5%) hypertension and repeated variceal
• Distal embolic protection devices (filters) bleeding
have been developed to reduce the risk of • Low mortality rate (1%) compared with
stroke further surgical shunting
• Can also be used (with a covered stent) to • Complications
treat carotid dissection and blow-out with –– New or worsening hepatic
bleeding encephalopathy (in 5–35% of cases)
–– Shunt thrombosis (rate is 50% at 1 year);
Subclavian steal syndrome treated via thrombolysis (acutely) or
stenting
• Narrowing (partial steal) or occlusion –– In-stent stenosis, needing routine
(complete steal) of the proximal subclavian surveillance (6-monthly Doppler
(or brachiocephalic) artery studies and yearly angiography)
• Blood flows in a retrograde direction down
the ipsilateral vertebral artery to supply
the distal subclavian artery, diverting flow
Percutaneous nephrostomy
away from the vertebrobasilar circulation • Provides temporary drainage of the
• Can cause syncopal episodes, vertigo and urinary tract as well as access for a variety
ataxia, classically induced by exercising of pyeloureteric procedures
the arm on the affected side • Indications
–– Acute hydronephrosis
Imaging features –– Pyonephrosis (infected obstructed
Ultrasound system)
• Normal vertebral artery waveform is low –– Palliation for malignant obstruction
resistance with forward (antegrade) flow –– Ureteric stricture dilatation, stenting or
throughout the cardiac cycle stone removal
• Partial steal • Ultrasound-guided puncture is a safe and
–– Antegrade flow in the vertebral artery reliable technique
during diastole • The posterior calyces in the mid- and
–– Retrograde flow in the vertebral artery lower poles are the best target
during systole • However, access via the lower pole gives
–– Exercising the arm on the affected side an acute angle of entry into the renal pelvis
exaggerates waveform change and can make further tracking of stents
–– Brachial artery has a monophasic difficult
waveform (normally triphasic) • ‘Brödel’s bloodless line’ of incision lies at
• Complete steal the junction of the anterior two thirds and
–– Retrograde flow throughout the cardiac the posterior one third of the kidney
cycle • The colon is retrorenal in 2% of patients,
more often on the left side and in relation
Trans-jugular intrahepatic to the lower pole
• Pus requires at least an 8 F catheter to
portosystemic shunt ensure adequate drainage
• Creation of a path and placement of a • Upper pole calyx puncture is only needed
covered stent (the preferred type) or a bare for nephrolithotomy
metal stent between the hepatic vein and • Ureteric stenting is indicated when long-
the portal vein to reduce portal venous term drainage is required and should be
pressure performed only once any infection has
• Can also create the path between the IVC abated
and the portal vein

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1.14 Cardiac imaging

Anatomy • The mitral valve is bicuspid; the anterior


cusp separates the left ventricular inflow
Cardiac chambers and outflow tracts
• Normal heart lies obliquely with its apex to Left ventricle
the left (levocardia)
• Forms the left border of the heart on
Right atrium frontal CXR
• Forms the right border of the heart on • Thick-walled, finely trabeculated, cone-
frontal CXR shaped cavity
• Receives systemic venous return via the • Circular in cross-section
SVC and the IVC • The mitral valve is in fibrous continuity
• The sinoatrial (SA) node lies at the junction with the aortic valve
of the SVC and the right atrium • Above each of the three aortic valve cusps
• The crista terminalis separates the atrial is a sinus of Valsalva
appendage from the right atrium and it
contains internodal tracts connecting the
Coronary artery anatomy
SA node with atrioventricular (AV) node Left coronary artery
• The atrial appendage is broad-based with a • Arises perpendicular to the aorta above
ridged muscular wall the left coronary cusp
• The fossa ovalis (closed foramen ovale) lies • Mainstem courses behind the pulmonary
in the interatrial septum trunk for 1–3 cm then bifurcates into the
• The coronary sinus drains into the smooth left anterior descending (LAD) and left
posterior wall of the right atrium circumflex (LCx) vessels
• The tricuspid valve has three cusps, each • LAD runs in the anterior interventricular
attached to papillary muscles of the groove giving off diagonal and septal
ventricular wall by the cordae tendinae perforator branches, which supply the
• A muscular fold (conus) separates the anterolateral wall of left ventricle and the
tricuspid valve from the pulmonary valve anterior two thirds of the interventricular
septum
Right ventricle
• LCx runs posteriorly under the left atrial
• Forms the anterior wall of the heart
appendage in the left atrioventricular
• Thin-walled, heavily trabeculated,
groove and gives off obtuse marginal
crescent-shaped cavity
branches
• Convexity of the interventricular septum
• LCx supplies the posterolateral wall of the
normally bulges into it
left ventricle
• Pulmonary valve (which has three cusps)
• LCx gives rise to the posterior descending
is the most anterior and the most superior
artery in 10% of people
heart valve
• Identified also by the muscular Right coronary artery
infundibulum • Arises perpendicular to aorta above the
right coronary cusp
Left atrium
• Courses to the right and descends in the
• Forms the posterior border of the heart on
right atrioventricular groove
lateral CXR
• First branch is the conal artery, supplying
• Not seen on a frontal CXR unless enlarged
the SA node
• Receives paired superior and inferior
• Supplies the right ventricle, the inferior
pulmonary veins
wall of the left ventricle and the posterior
• Tubular appendage projects anteriorly
third of the interventricular septum
from its left superior border

Ch-01.indd 78 8/7/2010 3:34:01 PM


Cardiac imaging 79

• Gives rise to the posterior descending –– Aortic stenosis, hypertension,


artery in 70% of people coarctation of the aorta
–– Hypertrophic cardiomyopathy
Coronary dominance: vessel giving –– Atrial fibrillation (if long-standing)
rise to the posterior descending –– Left atrial myxoma
artery and the AV nodal branch –– Ventricular septal defect (not atrial
septal defect)
• 70% are right coronary artery- –– Patent ductus arteriosus
dominant Imaging features
• 20% have a co-dominant system
• 10% are left coronary artery-dominant CXR
• Straightening of left heart border
• Elevated left main bronchus (splayed
carina)
Coronary artery anomalies • Double right heart border
• 1–2% of people have an anomaly of the
coronary arteries Conditions simulating left atrial
• A huge number of variations have been enlargement
described
–– High take-off above sinotubular • Partial pericardial defect
junction (commonest anomaly) • Pulmonary stenosis (dilated left
–– Trifurcation of left main stem, giving a pulmonary artery)
ramus intermedius branch • Persistence of a left SVC
–– Single coronary arising from left, right • Coronary artery fistula
or posterior cusps • Corrected transposition
–– Separate origin of LAD and LCx vessels

‘Malignant’ coronary artery


anomalies (< 1% of cases)
Left ventricular enlargement
• Causes
–– Ischaemic heart disease, hypertension
• Inter-arterial course: left or right
–– Aortic regurgitation or aortic stenosis
coronary aretery arising from the
–– Cardiomyopathy
contralateral sinus and running
–– Coarctation of the aorta
between both outflow tracts; carries
–– Ventricular septal defect
a risk of exercise-induced arterial
–– High output states, e.g.
compression and is linked with sudden
hyperthyroidism, severe anaemia,
cardiac death in young adults
Paget’s disease, pregnancy,
• Coronary artery arising from
arteriovenous fistulas (anywhere)
pulmonary artery: 90% die in the first
year of life Imaging features
• Coronary artery fistula: can cause
CXR
ischaemia
• Left, downward displacement of the apex
• Rounding of the left cardiac border

Right atrial enlargement


Cardiac chamber • Causes
enlargement –– Tricuspid regurgitation (e.g. pulmonary
hypertension)
Left atrial enlargement –– Ebstein’s anomaly
Causes –– Atrial septal defect
–– Mitral stenosis, mitral regurgitation –– Arrhythmogenic right ventricular
cardiomyopathy

Ch-01.indd 79 8/7/2010 3:34:01 PM


80 Cardiothoracic and vascular system

Right ventricular enlargement Imaging features


• Causes MRI
–– Left heart failure (pulmonary venous • Diffuse myocardial signal and perfusion
hypertension) abnormality
–– Pulmonary arterial hypertension CT
–– Left to right shunts • Cardiac CT is useful in differentiating
–– Outflow tract obstruction, e.g. calcified pericardium from restrictive
pulmonary stenosis cardiomyopathy
Imaging features
Dilated cardiomyopathy
CXR • Causes include alcohol, post-viral changes
• Elevation of cardiac apex (‘coeur en sabot’) and pregnancy
• Ventricular dilatation and global
Cardiomyopathy hypokinesis
• CT coronary angiography is useful in
Hypertrophic cardiomyopathy excluding coronary ischaemia
• Asymmetric hypertrophy of
interventricular septum is the commonest Arrhythmogenic right
subtype ventricular dysplasia
• Other subtypes include apical, mid • Fibrofatty replacement of right ventricular
ventricular and symmetric hypertrophic myocardium
cardiomyopathy (difficult to distinguish • Autosomal-dominant condition typically
from athletic and hypertensive heart affecting young males
disease) • Presents with ventricular arrhythmias and
• 50% hereditary, 50% sporadic right heart failure
• Associated with sudden cardiac death
from arrhythmias and exercise-induced Imaging features
outflow tract obstruction (termed MRI
hypertrophic obstructive cardiomyopathy) • High T1 and T2 signal in the right
ventricular wall, which suppresses with fat
Imaging features
saturation imaging
Echocardiogram and MRI • Focal or global dyskinesia of the right
• Asymmetrical septal hypertrophy ventricular wall
(commonest subtype) • Delayed enhancement of right ventricular
• Increased left ventricular mass wall, outflow tract and papillary muscles
• Small left ventricular cavity with
obliteration in systole
• Low left ventricular end systolic volume
Cardiac myxoma
(‘hyper-dynamic circulation’ with elevated • Commonest benign cardiac tumour
ejection fraction) in > 70% of cases • Can arise from any endocardial surface
• Mitral valve systolic anterior motion or • Majority attached to interatrial septum
prolapse • 80% occur in left atrium and are usually
pedunculated
Restrictive cardiomyopathy • 20% occur in right atrium and are usually
• Infiltrative process of ventricular sessile
myocardium • 20% are calcified (more common in the
• Causes include amyloidosis, sarcoidosis right atrium than the left)
and haemochromatosis • Clinically may mimic mitral stenosis, with
• Diastolic dysfunction (‘stiff myocardium’) a mid-diastolic murmur
• Clinically mimics constrictive pericarditis • Can cause embolic phenomenon (stroke,
pulmonary embolus)

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Cardiac imaging 81

Imaging features • Pulmonary stenosis is progressive;


cyanosis develops around 3 months of age
MRI
• Child often adopts a squatting posture,
• Intraluminal filling defect with a lobulated
which forces blood through the tight
contour
pulmonary valve
• Large tumours may be seen to prolapse
• Multiple associations, e.g. coronary artery
through the mitral valve on cine sequences
and valvular anomalies
• Usually heterogeneous contrast
enhancement (distinguishes a myxoma Treatment
from thrombus) • Blalock–Taussig shunt (from the subclavian
artery to the ipsilateral pulmonary artery)
Congenital heart disease • Surgical closure of the ventricular septal
defect (VSD) and patch repair of the
Fetal circulation pulmonary stenosis
• Oxygenated blood leaves the placenta via a Imaging features
solitary umbilical vein
• Travelling in the umbilical cord, CXR
oxygenated blood reaches the umbilicus • Boot-shaped heart
and then runs in the falciform ligament to • Elevated apex (because of right ventricular
the porta hepatis hypertrophy)
• Here the porta hepatis joins the • Small pulmonary trunk
intrahepatic left portal vein, and most • Right aortic arch in 25% of cases
blood passes via the ductus venosus into • Pulmonary oligaemia
the left hepatic vein and the IVC
• Blood entering the right atrium is directed
Ebstein’s anomaly
by a valve towards the foramen ovale, • Displacement of the tricuspid valve deep
through which the blood enters the left into right ventricular cavity
atrium • Proximal right ventricle is ‘atrialised’ but
• Some blood enters the pulmonary artery contracts synchronously with remainder of
but high lung resistance diverts most of it the ventricle
via the ductus arteriosus to the descending • Impaired ventricular function with
aorta cyanosis and oligaemia
• Each internal iliac artery gives an umbilical • Severe tricuspid regurgitation and massive
artery, which passes deoxygenated blood right atrial dilatation
back to the placenta in the umbilical cord • Associated with other structural and
conduction defects
Cyanosis without pulmonary • Wolf–Parkinson–White syndrome is a
plethora common cause of death
• 50% mortality in first year of life
• Tetralogy of Fallot Imaging features
• Ebstein’s anomaly
CXR
• Tricuspid atresia
• Box-shaped heart
• Dilated azygous vein

Tetralogy of Fallot Tricuspid atresia


• The four features of the tetralogy • Complete agenesis of the tricuspid valve
–– Pulmonary stenosis • Fibrous tissue occupies the cleft between
–– Right ventricular hypertrophy the right atrium and the right ventricle
–– Large ventricular septal defect • Obligatory right-to-left atrial septal defect
–– Aortic valve overriding the ventricular (ASD) or patent foramen ovale to sustain
septum life

Ch-01.indd 81 8/7/2010 3:34:01 PM


82 Cardiothoracic and vascular system

• Most also have a small VSD allowing some


left-to-right flow through a hypoplastic Congenitally corrected
right ventricle to the pulmonary artery transposition
• Severe cyanosis at birth
• Rarely there may be pulmonary plethora • Atrioventricular and ventriculoarterial
(if the VSD is large) discordance
• Ventricles are transposed with
Imaging features morphological right ventricle supplying
MRI or echocardiogram systemic circulation
• Large right atrium and hypoplastic • Aortic root lies anteriorly with respect
ventricle to the pulmonary outflow tract
• May present with left ventricular failure
Surgical shunt procedures in adulthood, owing to failure of the
• Blalock–Taussig shunt in infancy systemic ventricle
• Glenn anastomosis after infancy (from the • Acyanotic lesion
IVC to the pulmonary artery)
• Fontan operation (external graft from right
atrium to pulmonary artery)
Total anomalous pulmonary
Cyanosis with pulmonary plethora venous connection
• Pulmonary veins all drain to the systemic
• Transposition of the great arteries veins or the right atrium
• Total anomalous pulmonary venous • The SVC is the commonest site for the
connection connection
• Truncus arteriosus • Complete left to right shunt: shunt
• Tricuspid atresia with a large VSD dependant (ASD or PFO)
• Tingle (single) ventricle • The pulmonary vein may be obstructed,
causing low cardiac output
Imaging features
Transposition of the great CXR
arteries • ‘Figure-of-eight’-shaped heart
• Systemic and pulmonary circulations are
in parallel Scimitar syndrome
• Aorta arises from the right ventricle, the
pulmonary artery from the left ventricle • Partial anomalous pulmonary venous
• Cyanosis at birth with pulmonary plethora connection
• Shunt-dependent, i.e. relies on ASD, • Associated with a hypogenetic right
VSD or patent ductus arteriosus (PDA) to lung
sustain life • Anomalous vein seen adjacent to the
• Treated with prostaglandin E1 to stop right side of the heart
closure of the ductus arteriosus • Most commonly drains to the
• 90% mortality in first year of without subdiaphragmatic IVC
surgical intervention • Occurs almost exclusively on the right
side
Imaging features
CXR
• ‘Egg-on-side’ appearance of the heart
• Right ventricular hypertrophy
Truncus arteriosus
• Narrow vascular pedicle—pulmonary • Failure of septation of the embryonic
trunk lies posteriorly truncus arteriosus
• A single vessel drains both ventricles,

Ch-01.indd 82 8/7/2010 3:34:02 PM


Cardiac imaging 83

supplying the systemic, pulmonary and aorta, and the pulmonary trunk as far as its
coronary circulations bifurcation
• Moderate cyanosis and pulmonary • Pericardial cavity normally contains
plethora 20–50 mL of serous fluid
• Inner visceral layer is adherent to
Imaging features myocardium or epicardial fat
CXR • Outer parietal layer is attached inferiorly to
• Truncus is larger than a normal ascending the diaphragmatic tendon
aorta • Normal pericardium is seen on CT and
MRI as a thin (< 2 mm) linear band
Acynotic congenital heart • Superior pericardial recess lies posterior to
condisions with pulmonary plethora ascending aorta
• Normal aortopulmonary window contains
• ASD fat
• VSD
• PDA
Congenital absence of the
pericardium and pericardial
sac defect
Left-to-right shunt • Complete absence (10% of cases) is benign
• Has the potential to increase pulmonary condition with a normal life span
blood flow, leading to plexogenic • Partial absence (90% of cases)
arteriopathy and pulmonary hypertension –– More common on the left side and in
if untreated males
• Large shunts (e.g. VSD, PDA) cause –– Associated with bronchogenic cyst,
pulmonary hypertension within the first VSD, PDA, ASD, tetralogy of Fallot,
few years of life diaphragmatic hernia and pulmonary
• Smaller shunts (e.g. ASD) will often not sequestration
present until adulthood –– Herniation through the defect can
• Shunt reversal occurs when pulmonary cause arrhythmia and sudden death
resistance rises above systemic resistance Imaging features
(Eisenmenger’s syndrome); pulmonary
vessels become pruned and central CXR
pulmonary arteries and conus remain • Radiolucent cleft in the aortopulmonary
enlarged, and left atrial size decreases in window
VSD. Patients are cyanosed • Straight left heart border (bulging left
atrium through defect)
Imaging features • Absence of left pericardial fat pad
CXR • Increased distance between the heart and
• Enlarged pulmonary conus sternum on lateral view
• Enlarged left and right pulmonary arteries CT
• Enlarged pulmonary vessels (plethora) • Lung tissue interposed between the aorta
• Enlarged left atrium in VSD; normal left and pulmonary trunk
atrium in ASD • Pulmonary trunk deviated to the left

Pericardial effusion
Pericardium • > 50 mL of fluid in the pericardial cavity
Anatomy • Causes
• Fibroserous sac consisting of visceral and –– Infection (viral, bacterial, tuberculous)
parietal layers –– Heart failure
• Envelops the four cardiac chambers, the –– Uraemia
first few centimetres of the ascending –– Hypothyroidism

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84 Cardiothoracic and vascular system

–– Connective tissue disorders • Clinically mimics restrictive


–– Malignancy (of breast or lung, or cardiomyopathy
lymphoma) • Commonest causes are radiation therapy
–– Myocardial infarction (Dressler’s and cardiac surgery
syndrome) • Can be secondary to any cause of
–– AIDS pericarditis
–– Trauma
Imaging features
Imaging features CXR
CXR • Small or normal-sized heart
• Enlargement of the cardiopericardial • Linear pericardial calcifications in > 50% of
shadow cases
• Clear cardiac margins (‘pencil line’) • Dilated azygous vein
• ‘Water bottle’ heart CT/MRI
CT • Pericardial thickening (> 4 mm) and
• Water attenuation with simple effusions calcifications
• Higher attenuation with blood, • Narrow tubular right ventricular cavity
malignancy, infective conditions and • Contrast reflux into the hepatic veins,
hypothyroidism coronary sinus and IVC
• Nodular enhancing pericardium suggests
malignant involvement Pericardial cyst
• Unilocular, mesothelial-lined cyst
MRI
• Outpouching of parietal pericardium
• Low T1 signal, high T2 signal seen with
• Does not communicate with the
simple effusions
pericardial space
• High T1 signal is characteristic of blood,
• Usually an asymptomatic incidental
commonly heterogenous
finding
Cardiac tamponade • Right cardiophrenic angle is the
• Haemodynamically significant cardiac commonest location
compression from pericardial contents Imaging features
causing impaired diastolic filling and
reduced stroke volume
CXR
• Lobulated mass at the cardiophrenic angle
• Requires urgent pericardiocentesis (echo-
or CT-guided) CT
• Can be caused by rapid accumulation of as • Round, thin-walled, water-attenuation cyst
little as 100 mL of fluid • The wall may calcify
• Pulsus paradoxus (inspiratory drop in MRI
blood pressure) • Low T1 signal, high T2 signal
Imaging features
Echocardiogram, MRI and ECG-gated CT Nuclear cardiology
• Diastolic right ventricular/atrial collapse • SPECT scans provide a three-dimensional
Constrictive pericarditis display of radionuclide distribution
• Images acquired over a 180° arc from right
• Fibrous pericardial thickening, which anterior oblique to left posterior oblique
prevents normal diastolic ventricular views
expansion • More accurate than older planar imaging
• Kussmaul’s sign (paradoxical rise of technique
jugular venous pressure on inspiration)

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Cardiac imaging 85

Radiopharmaceutical agents are used to induce coronary artery


vasodilation and tachycardia
Thallium-201 • Imaging can be with SPECT or MRI (and
• Intravenous injection at maximal heart more recently CT perfusion imaging has
rate (‘stress study’) been described)
• Decays to mercury-201 by electron capture • Sensitivity for detecting > 70% coronary
• Emits both gamma-rays and X-rays stenosis
• 90% is cleared from the blood after its first –– ECG stress test: 65% sensitivity
circulation –– Myocardial perfusion imaging: 80–90%
• 4% is taken up by the heart at rest and 10% sensitivity
during maximal exercise
• Enters cardiac myocytes via the sodium– Patterns of abnormality
potassium ATPase pump • Reversible defect
• Distribution through the myocardium is –– Perfusion defect at stress but normal
proportional to perfusion at rest
• Images are acquired immediately after –– Implies inducible ischaemia
injection • Fixed defect
• Booster injection and repeat imaging at –– Perfusion defect at stress that persists
2–4 hours for a ‘rest study’ at rest
• Repeat imaging at 24–48 hours to assess –– Implies myocardial scar tissue, e.g. old
hibernating myocardium myocardial infarction
• Lung uptake
Limitations of thallium –– A little lung uptake is a normal finding
but a lung–myocardial uptake ratio
• High radiation dose (18-22 mSv) > 0.5 signifies impaired left ventricular
• Low signal-to-noise ratio in obese function
patients –– Lung uptake is also seen with
bronchogenic carcinoma and
lymphoma
Technetium-99m False positive cardiac SPECT
• Labelled with perfusion tracer, e.g.
sestamibi
• Cardiomyopathies
• Passive diffusion into cardiac myocytes
• Coronary spasm
• No redistribution; retained in myocardium
• Infiltrative disease e.g. sarcoidosis
for up to 48 hours, and hence the need for
separate rest and stress imaging
• Stress study is done first for ischaemia; rest
study is done first to assess viability False negative cardiac SPECT
• Lower dose (8 mSv) than thallium but not
as good as thallium for assessing viability
• ‘Balanced’ triple-vessel disease
Stress imaging • Inadequate stress
• Myocardial stress response is critical in
patient assessment
• Perfusion defects are only seen at rest with
> 90% stenosis Cardiac MRI
• Heart rate achieved should ideally be
> 85% maximum predicted Clinical applications
• Dynamic exercise (treadmill) or • Congenital heart disease
pharmacological stress whereby • Myocardial viability assessment
adenosine, dipyridamole or dobutamine • Cardiomyopathy

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86 Cardiothoracic and vascular system

• Valvular assessment (including prosthetic) • Mainly used to identify coronary artery


• Pericardial disease anatomical variants and to assess for
proximal vessel patency
Basic sequences
Spin-echo sequence (black blood)
Myocardial perfusion imaging
• Dynamic first-pass imaging following a
• Double inversion recovery pulse
gadolinium contrast bolus
suppresses signal from moving blood
• Rapid image acquisition (high temporal
• Mainly used for anatomical and
resolution) is achieved via echoplanar
morphological overview
sequences and segmented k-space filling
• Can be used to assess for aortic dissection
• Normal myocardium shows a uniform
and coarctation
‘contrast blush’
• Parameters can be adjusted to obtain both
• Delayed ‘wash-in’ is seen in areas of
T1WI and T2WI
relative hypoperfusion
• Fat saturation sequences are useful
• Stress images are acquired using
for diagnosing arrhythmogenic right
intravenous vasodilators (adenosine) to
ventricular dysplasia
induce vasodilation and increase oxygen
Gradient-echo sequence demand
(bright blood) • With significant stenosis (> 70%), flow
• Rapid acquisition using balanced steady- cannot be sufficiently increased, which
state free, precession (SSFP) sequence causes hypoperfusion in an arterial
with low flip angles territory
• Image contrast depends on ratio of T2/T1 • Study is later repeated at rest for
times comparison
• Excellent inherent contrast between • MR perfusion imaging is similar to PET
myocardium (dark) and luminal blood and catheter angiographic studies in terms
(bright) of detecting myocardial ischaemia
• Used to create cine-images for assessing Delayed enhancement imaging
wall motion abnormalities, valvular
function and turbulent flow (signal loss) • Technique of choice for distinguishing
viable myocardium from non-viable
Basic scanning method myocardium
• Accurately sizes regions of myocardial
necrosis
• Phased array cardiac surface coil • More sensitive than SPECT, PET and
• ECG gating and/or respiratory gating dobutamine stress echocardiography
• R wave chosen as gating signal for detection of subendocardial infarcts
reference point (improved spatial resolution)
• Myocardial scar tissue shows delayed
uptake of contrast, which washes out more
MR coronary artery slowly than in normal myocardium
angiography • Delayed images acquired 10–20 minutes
after gadolinium administration
• ECG trigger delayed to acquire images in
• An inversion recovery pulse is used to
end-diastole
null the myocardial signal and make
• SSFP sequence with respiratory (navigator)
gadolinium accumulation stand out
gating
• No contrast media required Myocardial infarction
• Limited by poor spatial resolution • Subendocardial or transmural delayed
(1–2 mm) at 1.5 Tesla hyperenhancement corresponding to an
epicardial arterial territory

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Cardiac imaging 87

• < 25% thickness: good functional recovery • Hyperenhancement is globally diffuse, and


likely with revascularisation typically subendocardial
• > 75% thickness: poor functional recovery
likely with revascularisation Cardiac CT
Hibernating myocardium • Mainly used for non-invasive coronary
• This is contractile dysfunction caused by artery evaluation
chronic hypoperfusion
• Absence of delayed hyperenhancement Coronary CT angiography
• Shows good functional recovery with • High negative predictive value for
revascularisation exclusion of haemodynamically significant
coronary artery disease in selected patient
Delayed enhancement in other groups
pathologies • Calcified coronary artery plaque burden is
• Many non-ischaemic pathologies are a predictor of future cardiovascular events,
associated with delayed gadolinium but absence does not rule it out
enhancement • The amount of calcium underestimates the
• The pattern typically does not correspond true extent of atherosclerotic disease
to an arterial territory which helps with • Technique
distinction –– Performed using ECG gating
–– Non-contrast study is usually
Hypertrophic cardiomyopathy performed first for assessment of
• Patchy hyperenhancement within coronary artery calcium burden
hypertrophied regions –– Target heart rate is 50–60 beats per
• Correspond to areas of minor infarcts or minute (intravenous beta-blockers are
fibrosis administered to achieve this rate)
• Junction of the right ventricular free wall –– Intravenous contrast bolus is injected at
and the interventricular septum is a typical a fast rate (5–6 mL/second), followed by
site a saline chaser to reduce SVC and right
• High levels of enhancement correlate with heart streak artefacts
a worse prognosis –– Data are continuously acquired over
Sarcoidosis one or more heart beats
• Cardiac involvement seen in 20–30% of –– Reconstructed using various post-
cases processing algorithms
• May initially cause a restrictive picture; a –– Phase of least cardiac motion (end-
dilated picture more typical of late-stage diastole) is selected at around 70% of
disease the R–R interval
• Mid-myocardial wall hyperenhancement –– Review is made using axial source
seen in affected areas, most often along images, maximum intensity projections
the interventricular septum and basal (MIPs) and multiplanar reformats
inferolateral wall (MPRs)
–– Isotropic voxel dimensions (equal
Myocarditis size in the x, y and z axes) allow
• Non-specific, patchy mid-myocardial wall reconstruction in any plane without
hyperenhancement loss of image quality
• Diffuse involvement, which decreases –– Volume-rendered images are not
during healing phase diagnostic but help in obtaining a bird’s
eye view of grafts and other anomalies
Amyloidosis
• Infiltration of fibrillar proteins causing a
restrictive cardiomyopathy

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88 Cardiothoracic and vascular system

Image resolution Cardiac CT dose reduction


• Cardiac CT requires high temporal and • Typical effective CT calcium scoring dose
spatial resolution to ‘freeze’ cardiac is 1–3 mSv
motion and resolve the coronary vessels • Typical effective CT angiography dose is
5–15 mSv (equivalent to 500 CXRs)
Temporal resolution • Effective dose using ECG-gated dose
• 64-slice CT: approximately 160 modulation is 3–10 mSv
milliseconds • This compares with diagnostic coronary
• Dual-source CT: approximately 80 catheter angiography at 3–6 mSv
milliseconds
• Electron beam CT: approximately 70 ECG-gated dose modulation
milliseconds • A method of reducing dose by 10–40%
• This compares with MRI at 30–40 • Uses ECG gating to reduce the tube current
milliseconds and fluoroscopy at 1–10 during parts of the cardiac cycle that are
milliseconds not used in image reconstruction, e.g.
systole
Factors to improve temporal
resolution Heart valve imaging
• Echocardiography is the first-line test
• Increased gantry rotation time • Cardiac MRI is also a reliable means
• Multiple segment reconstruction (in of assessment and provides accurate
which data are selected from several assessment of transvalvular gradients
cardiac cycles instead of from a single • Cardiac CT is the best means of assessing
cycle; this requires retrospective ECG valvular calcification. Although it cannot
gating) directly assess the gradient across a valve it
can be used to provide indirect assessment
of function via orifice area measurement
Spatial resolution
• 64-slice CT: approximately 0.4 mm, versus
MRI (1–2 mm)

Factors to improve spatial


resolution

• Thinner detector width


• Decreased reconstruction interval
(the degree of overlap between
reconstructed axial images; decreasing
the reconstruction interval increases
overlap and improves resolution)
• Small field of view
• Low pitch

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Chapter 2
Musculoskeletal system
and trauma

2.1 High-yield anatomy


2.2 Arthritides and connective tissue
diseases
2.3 Endocrine diseases
2.4 Infections and nutritional and
metabolic diseases
2.5 Trauma
2.6 Tumours
2.7 Miscellaneous conditions
2.8 Lists to remember

Ch-02.indd 89 8/12/2010 12:18:08 PM


2.1 High-yield anatomy

Ossification centres –– Anterior inferior iliac spine: rectus


femoris (straight head)
• Clavicle –– Groove above the anterior acetabular
–– Membranous ossification with the rim: rectus femoris (reflected head)
secondary ossification centre appearing –– Symphysis and inferior pubic ramus:
at the sternal end adductors, gracilis
• Scapula • Femur
–– Coracoid (17 years), acromion –– Greater trochanter: gluteus medius,
(15–18 years), scapula (20 years) gluteus minimus
• Humerus –– Lesser trochanter: iliopsoas
–– Greater tuberosity (1 year), lesser • Knee
tuberosity (2 years), humeral head –– Lateral tibial plateau: second fracture,
(5 years) bony avulsion injury of the iliotibial
• Elbow band and the lateral collateral ligament
–– CRITOL: capitellum (1 year), radial (anterior oblique band)
head (3 years), internal epicondyle –– Fibular head: lateral collateral ligament,
(5 years), trochlea (9–11 years), long head of biceps femoris (conjoint
olecranon (9–11 years), lateral tendon)
epicondyle (9–11 years) –– Anterior tibial intercondylar area:
–– Epiphyses fuse together and with the anterior cruciate ligament
shaft at 17 years –– Posterior tibial intercondylar area:
• Hand posterior cruciate ligament
–– Capitate and hamate (4 months), –– Inferior pole of patella: patellar tendon
triquetral (3 years), lunate (4 years), • Ankle and foot
trapezium, trapezoid and scaphoid –– Calcaneus, posterior surface: Achilles
(6 years), pisiform (11 years) tendon
–– Metacarpal heads fuse before the bases –– Base of fifth metatarsal: peroneus brevis
• Femur • Humerus
–– Proximal femur (6 months to 1 year), –– Greater tuberosity: supraspinatus,
greater trochanter (3–5 years), lesser infraspinatus and teres minor
trochanter (8–14 years), distal femur –– Lesser tuberosity: subscapularis
(at birth) • Elbow
• Tibia –– Medial epicondyle: common flexor
–– Proximal tibia (at birth) origin
• Feet –– Lateral epicondyle: common extensor
–– Calcaneus and talus (6 months), cuboid origin
(at birth), navicular (3 years)
Accessory bones
Important muscle
• Accessory navicular (os tibiale externum,
attachments os naviculare): seen in 5% of the
• These muscle attachments are best population, found on the medial aspect of
remembered by the patterns of avulsion the foot
injuries encountered in practice • Os trigonum: found just posterior to the
• Pelvis talus
–– Ischial tuberosity: hamstrings • Os vesalianum: found near the base of the
–– Anterior superior iliac spine: sartorius, fifth metatarsal
tensor fascia lata • Os acromiale: failure of fusion of the

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High-yield anatomy 91

acromion, seen in 10% of the population • Between distal end of the radius and the
over 25 years of age scaphoid, lunate and triquetral bones
• Contents of the carpal tunnel: nine
Joints of the upper limb tendons (four flexor digitorum superficialis
tendons, four flexor digitorum profundus
Shoulder joint tendons and one flexor pollicis longus
• A synovial ball-and-socket joint tendon) and the median nerve
• The ulnar nerve and artery course through
Rotator cuff Guyon’s canal, at the volar aspect of the
• Four muscles comprise the rotator cuff; it pisiform
is deficient in the inferior aspect
–– Supraspinatus: from the supraspinous Carpals
fossa of the scapula to the greater
tuberosity of the humerus • Proximal row: scaphoid, lunate, triquetral
–– Infraspinatus: from the infraspinous and pisiform
fossa of the scapula to the greater • The pisiform is a sesamoid bone in the
tuberosity of the humerus tendon of the flexor carpi ulnaris
–– Teres minor: from the lateral border of • Distal row: trapezium, trapezoid, capitate
the scapula to the greater tuberosity of and hamate
the humerus
–– Subscapularis: form the anterior Articulation of the carpals
surface of the scapula to the lesser
tuberosity of the humerus • Scaphoid articulates with the
trapezium and trapezoid distally, the
Bursas
capitate and lunate medially
• There are two bursas associated with the
• Capitate articulates with seven bones:
shoulder joint
the scaphoid and lunate proximally, the
–– The subacromial–subdeltoid bursa
second, third and fourth metacarpal
–– The subscapular bursa, which normally
distally, the trapezoid on the radial side,
communicates with the joint space
and the hamate on the ulnar side
(unlike the subacromial–subdeltoid
• Hamate articulates with the fourth and
bursa)
fifth metacarpals distally, the trapezium
Elbow joint medially, and the capitate laterally
• Synovial hinge joint
• The capitellum articulates with the radial
head and the trochlea articulates with the Scaphoid
semilunar notch (trochlear notch) of the • Boat-shaped, with a body, waist and
ulna tuberosity
• There are two fat pads, anterior and • Blood supply is from distal to proximal
posterior • Fractures through the waist of the
–– The anterior is seen in 15% of the scaphoid can disrupt the blood supply,
population resulting in avascular necrosis of the
–– Elevation of the posterior fat pad is proximal part
always abnormal
• The anterior humeral line intersects the Carpal tunnel
mid-third of capitellum; the longitudinal • Fibro-osseous tunnel bound by the carpals
axis of the radius should pass through the dorsally and the flexor retinaculum on the
centre of capitellum volar aspect
• Contents
Wrist joint –– Median nerve
• Synovial ellipsoid joint –– Tendons of the flexor digitorum

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92 Musculoskeletal system and trauma

superficialis and profundus (in a –– From the shaft (the nutrient artery)
common sheath) –– Through the ligament of the head of the
–– Flexor pollicis longus femur
–– Palmaris longus, flexor carpi radialis • The most important source is the capsular
and flexor carpi ulnaris are the three artery, which can get disrupted in fractures
tendons that are outside the carpal of the neck of the femur
tunnel
The linea aspera
• A ridge of roughened surface on the
Joints of the lower limb posterior aspect of the femur
Pelvis and hip • Attachments
–– Gluteus maximus
• Synovial ball-and–socket joint between the –– Short head of the biceps femoris
head of femur and the acetabulum –– Pectineus and iliacus
• The width of the symphysis pubis should –– Adductor magnus
not exceed 7 mm on anteroposterior views –– Vastus medialis and vastus lateralis
• A fibrocartilagenous rim (the labrum) –– Adductor brevis and adductor longus
deepens the acetabulum
• The symphysis pubis is a secondary Sacroiliac joint
cartilaginous joint • Synovial joint at its inferior two thirds
• Ligaments and a fibrocartilaginous articulation at its
–– Ischiofemoral ligament superior one third (similar to the pubic
–– Pubofemoral ligament, which is symphysis)
triangular in shape • Ligaments
–– Iliofemoral, which is an inverted ‘V’ –– Ventral and dorsal sacroiliac ligaments
shape –– Interosseous sacroiliac ligament
–– Ligament of the head of the femur (strongest ligament in the body)
• Important relations
–– Anterior: the femoral vessels and the Knee joint
femoral nerve • Modified synovial hinge joint
–– Posterior: the sciatic nerve • Consists of two joints between the femoral
–– Shenton’s line: seen on anteroposterior and tibial condyles and the patellofemoral
radiograph of pelvis, it joins the inferior joint
edge of inferior ischiopubic ramus
to the inferior edge of the shadow of
Bursas
neck of femur; discontinuity denotes a • Important bursas are the suprapatellar,
fracture of the neck of femur pre-patellar, infrapatellar and popliteal
bursas
Imaging of the hip joint Ligaments
• Important ligaments
• Von Rosen’s view: 45° abduction and –– Medial collateral ligament: between
internal rotation of the hip, used to the medial femoral epicondyle and the
assess instability of the hip joint in medial tibial condyle
children –– Lateral collateral ligament: between
the lateral femoral epicondyle and the
fibular head
• The blood supply of the femoral head
–– Anterior cruciate ligament: between the
comes from three sources
anterior tibial intercondylar area and
–– Along the capsule of hip joint, derived
the posterior part of the medial surface
from the trochanteric anastomosis
of the lateral femoral condyle

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High-yield anatomy 93

–– Posterior cruciate ligament: between quadriceps tendon and is attached to the


the posterior tibial intercondylar area tibial tuberosity
and the anterior part of the lateral
surface of the medial femoral condyle;
Popliteus muscle
prevents the femur from sliding • Origin is the lateral surface of the lateral
forwards on tibia femoral condyle
• Insertion is at the posterior tibia, deep to
Menisci the lateral collateral ligament
• The menisci are semicircular, • Action: ‘Key’ muscle that helps unlocking
fibrocartilaginous bands the knee at beginning of flexion by
• They appear triangular on coronal and facilitating the slight inward rotation of the
sagittal imaging as they taper from a height tibia
of 3–5 mm at the periphery to a sharp, thin, • Also attached to the lateral meniscus by
central free margin ligaments
• Medial meniscus is larger and more ‘C’-
shaped than its lateral counterpart Ankle joint and foot
• Its posterior horn is larger than its anterior
horn Articulation of the tarsals
• It is more closely attached to the joint
capsule, and being less mobile has a • Navicular articulates with the talus
greater risk of tearing proximally, the three cuneiform bones
• The body of medial and lateral menisci distally, and occasionally with the
show a ‘bowtie’ configuration on two cuboid laterally
contiguous sagittal 4 mm MRI scans • Cuboid articulates with the fourth
• Discoid meniscus (with predilection and fifth metatarsal bases distally the
for the lateral meniscus) represents calcaneus proximally (forming the
a thickened meniscus with a bowtie calcaneocuboid joint), and the lateral
configuration seen on three or more cuneiform bone and the navicular bone
sagittal MRI scans medially; the inferior surface has a
• Meniscofemoral ligaments course from the groove at its distal third for the tendon
posterior horn of the lateral meniscus to of the peroneus longus muscles
the medial femoral condyle
• If present anterior to the posterior cruciate
ligament, it is called the ligament of • The fifth metatarsal
Humphry; if posterior to the posterior –– Articulates with cuboid and the fourth
cruciate ligament, it is called the ligament metatarsal
of Wrisberg –– Peroneus tertius and peroneus brevis
insert on it
Patella –– Its normal apophysis is longitudinal to
• Sesamoid bone it, at the base of bone
• Appears at the age of 3 years –– Jones’s fracture involves the
• Can be bipartite in 10% of people metaphyseal–diaphyseal junction
• Prone to lateral dislocation and should not be confused with the
• Fabella is found in 25% of adults and lies in commoner distal, transversely oriented
the lateral head of the gastrocnemius fracture that occurs at the base of the
• The patellar tendon is a continuation of the fifth metatarsal

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Arthritides and
2.2 connective tissue diseases

Osteoarthritis Imaging features


• Polyarticular synovitis with osteoporosis
• Degenerative joint disease affecting males
• Uniform cartilage destruction
and females equally
• No productive changes, i.e. absent
• Due to intrinsic degeneration of articular
osteophytes/periostitis
cartilage or excessive wear and tear
• Typically bilaterally symmetric
• Affects the hips and knees more
involvement of the following joints
commonly than shoulders and elbows
–– Wrist: the radiocarpal joints and the
• Note there is a rare erosive variant of OA
distal radio-ulnar joints
that is seen in middle-aged women
–– Hand: the MCP joints and the PIP joints
• Small joint involvement
–– Foot: the metatarsal phalangeal (MTP)
–– Primary osteoarthritis frequently
joints and the retrocalcaneal bursa
involves the hand, wrist,
• Rotator cuff tears at the shoulder, valgus
acromioclavicular joint, hip, knee
deformity of knee and protrusio deformity
(medial compartment), foot and spine
of the hips are associated features
–– Involvement of hands is 10 times more
• Upper cervical spine may display facet
common in women than in men
erosions and atlantoaxial subluxation
–– In the hand, the distal interphalangeal
(DIP) joints are the most commonly Juvenile rheumatoid arthritis/
involved juvenile chronic arthritis
–– Loss of cartilage in the first
• Still’s disease is a type of juvenile
carpometacarpal (CMC) and
rheumatoid arthritis, presenting as an
metacarpophalangeal (MCP) joints is
acute illness with pyrexia, anaemia,
typical
leukocytosis, hepatosplenomegaly,
Imaging features lymphadenopathy and polyarthritis
• Cartilage destruction in the weight-bearing • Only 10% of cases are positive for
portions of the joint rheumatoid factor
• Absence of erosions • Radiographic features are generally mild
• Bone density preserved and only about 25% of patients develop
• Narrowing of joint space is an early feature chronic and destructive arthritis
• Osteophytosis • Commonly affected joints: knees, elbows
• Subchondral sclerosis and hips
• Subchondral cysts, due to microfractures • Metaphyseal and epiphyseal ballooning
in the subchondral bone (due to hyperaemia)
• The growth plate closes early as a result
of hyperemia and there is asymmetric
Rheumatoid arthritis maturation of ossification centres
• Erosive arthropathy affecting females more • Can cause ankylosis in the carpus, tarsus
than males and spine
• Rheumatoid factor may be negative early • Atlantoaxial subluxation is not a common
in the disease, but becomes positive in finding and occurs in seropositive cases
90–95% of cases subsequently
• Rheumatoid factor may be falsely positive Ankylosing spondylitis
in older people
• An autoimmune spondyloarthropathy

Ch-02.indd 94 8/12/2010 12:18:09 PM


Arthritides and connective tissue diseases 95

associated with ulcerative colitis, enteritis, Reiter’s syndrome


iritis and aortic valve disease
• Commoner in males • Clinical triad of arthritis, uveitis and
• Median age 15–35 years urethritis
• Males are more frequently affected than
Imaging features females
• Sacroiliitis is the hallmark feature. • May be sexually transmitted or occur
Involvement of the axial skeleton is following dysentery
more common than peripheral skeleton. • HLA-B27 positive in 80% of cases
Unilateral early on, then becomes bilateral
• Changes in the spine include: vertical Imaging features
syndesmophytes (bamboo spine), • Identical to psoriasis, but usually involves
squaring, marginal syndesmophytes lower extremity instead
• Spine fractures easily at cervicothoracic • Osteoporosis is a feature of acute disease,
and thoracolumbar junctions but not of recurrent or chronic disease
• Peripheral large joint erosive arthritis is • Calcaneal erosions with spur formation are
also a feature typical
• Bulky asymmetric paravertebral
ossification are seen in the thoracolumbar
Psoriatic arthropathy spine
• Seronegative spondyloarthropathy
involving synovial and cartilaginous joints Systemic lupus
• Occurs in 5% of patients with psoriasis and
may occur before the onset of skin changes erythematosus
• Five clinical and radiological types • Over 80% have symmetric, non-erosive,
–– Polyarthritis with predominant non-deforming polyarthritis affecting the
involvement of the DIP joints small joints of the hands, wrists, knees,
–– Seronegative rheumatoid-like shoulders
polyarthritis • Bilateral and symmetrical
–– Oligoarticular arthritis • Reducible deformities:
–– Spondyloarthritis mimicking –– ulnar subluxation of the MCP joints and
ankylosing spondylitis the first CMC joint
–– Arthritis mutilans –– flexion or extension deformities of the
Imaging features interphalangeal joints
• Associated with avascular necrosis in
• DIP joints more severely affected than PIP
unusual sites such as the talus
joints
• Soft tissue calcification (usually in the
• Bone density preserved
lower extremities) in 10% of cases
• Periostitis and ‘sausage digit’ (soft tissue
swelling of the entire digit) may help
Jaccoud’s arthropathy
distinguish psoriatic arthropathy from
rheumatoid arthritis
• ‘Pencil-in-cup’ deformity may be seen, • Irreversible deformities
secondary to erosions that are initially • Ulnar drift at the MCP joints as well as
periarticular and progress to involve the swan-neck and boutonière deformities
entire articular surface • Seen in rheumatic fever
• Tuft resorption or reactive sclerosis in
distal phalanx (‘ivory phalanx’)
• Asymmetric sacroiliitis and spondylitis
with paravertebral ossification
Scleroderma
• Bulky asymmetric osteophytoses • 50% of patients have articular involvement

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96 Musculoskeletal system and trauma

• The fingers, wrists and ankles are • No juxta-articular osteoporosis is seen


commonly affected unless the joint is infected
• Erosions may be seen at the DIP joints, the
first MCP joints, the CMC joints and the
MTP joints
Gout
• Acro-osteolysis with soft tissue atrophy is a • Sodium urate crystal arthropathy
feature • Middle-aged and elderly men are usually
• Soft tissue calcification may be associated affected
• Long latent period between onset of
symptoms and bony changes
Amyloidosis
• Primary or secondary
Imaging features
• Bulky nodular synovitis • Normal bone density, and cartilage is often
• Well-marginated erosions intact
• Wrists, elbows and shoulders can be • No joint space narrowing till late, since this
affected is primarily a non-inflammatory process
• The cervical spine is classically involved in with recurrent acute, intermittent episodes
dialysis-related spondyloarthropathy rather than chronic progression
• Olecranon bursitis is commonly associated
• Often asymmetric and monoarticular
Charcot’s joints • The first MTP joints, the DIP joints and
(neuropathic arthropathy) the patellofemoral joints are the most
frequently involved joints
• Severely destructive, rapidly progressive
• Erosions are sharply marginated and may
arthropathy
be intra-articular or para-articular
• Usually monostotic
• An overhanging edge of a para-articular
• Common causes include diabetes mellitus,
erosion is pathognomonic
syphilis, syringomyelia and spinal cord
• Gouty tophus may rarely show amorphous
injury
calcification
• Rarer causes are multiple sclerosis,
• Gouty tophus is seen on MRI as low on T1-
hereditary sensorimotor neuropathy
weighted images (T1WI) and variably high
(Charcot–Marie–Tooth disease),
or low on T2-weighted images (T2WI),
alcoholism, amyloidosis, intra-articular
depending on calcium content and status
corticosteroids, congenital insensitivity
of calcium hydration
to pain, scleroderma and dysautonomia
(Riley–Day syndrome)
• Three forms are described: hypertrophic Pyrophosphate arthropathy
(accounting for 20% of cases), atrophic • May be idiopathic or associated with
(40%) and mixed (40%) hyperparathyroidism or hemochromatosis
Imaging features • Chondrocalcinosis is usually present
• Classic description of the hypertrophic • Symmetric involvement of the knees,
form is of ‘five Ds’: normal Density, wrists and MCP joints
joint Distension, bony Debris, joint • Presents clinically with a sudden onset
Disorganisation, Dislocation of pain and fever, with a tender, swollen,
• The atrophic form shows severe bone red joint that has a reduced range of
resorption, with little or no debris movements
• All forms are invariably associated with
large effusions

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Arthritides and connective tissue diseases 97

Findings in calcium pyrophosphate Marfan’s syndrome


dihydrate deposition disease • Connective tissue disorder of unknown
cause
• Knee: patellofemoral disease • Autosomal dominant
predominates • One in 10,000
• Wrist: radiocarpal disease, progress to • Commonest locations are the lumbar
scapholunate advanced collapse (SLAC) spine and sacrum
• Hand: involves the second and third • Skeletal manifestations
MCP joints –– Kyphoscoliosis
• Large subchondral cysts may be seen –– Posterior scalloping, due to dural
ectasia
–– Increased interpedicular distance
–– Increased anterior translation and
Bone sarcoid subluxation at the C1–C2 level
–– Increased height of the odontoid
• Bony involvement is rare (occurs in up to process
15% of known cases) –– Basilar invagination
• The most frequently noted abnormality is –– Biconcave vertebrae
lytic lesions with lacy trabeculae, usually –– Early osteoarthritis
in the middle or distal phalanges –– Protrusio acetabuli
• Generalised osteopenia, sclerosis of –– Pectus excavatum
phalangeal tufts, and focal or generalised –– Pes planus, club foot, hallux valgus and
sclerosis are other known manifestations hammer toes

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2.3 Endocrine diseases

Primary • ‘Pepper-pot’ skull


• Diffuse osteosclerosis (rare)
hyperparathyroidism • Chondrocalcinosis in 10–20% of cases,
• Increased levels of parathyroid hormone characteristically in the knee, symphysis
• Increased serum calcium and decreased pubis and wrist
serum phosphate Bone scan
• Parathyroid adenomas account for over • Normal in 80% of cases
90% of cases • 24-hour study shows increased retention
• Middle-aged women most commonly of phosphate tracers, focal uptake in
affected affected areas, and brown tumours
• Common clinical manifestations include
nephrolithiasis, hypertension, peptic ulcer, Primary hyperparathyroidism
pancreatitis, bone pain and psychiatric versus secondary
disorders hyperparathyroidism
• Incidence of bone lesions is 25–40%
• Associations • Adenomas commonly in primary
–– Multiple endocrine neoplasia type 1 • Brown tumours are seen in primary
(Wermer’s syndrome), with pituitary more than in secondary
adenoma and pancreatic islet cell • Soft tissue calcification is seen in
tumour secondary more than in primary
–– Multiple endocrine neoplasia • Bone sclerosis is seen in secondary
type 2 (Sipple’s syndrome), with more than in primary
medullary thyroid carcinoma and • Periostitis is seen in secondary more
phaeochromocytoma than in primary
Imaging features
Plain X-ray
• Subperiosteal bone resorption (seen in
only 10% of cases), commonly along the Osteoporosis
radial aspect of the middle phalanges of • Characterised by diminished bone mass,
the second and third digits (other sites with a normal ratio of non-mineralised to
include the tufts, the medial metaphysis of mineralised bone
the proximal humerus, the femur and the • Multiple causes
tibia) –– Diminished oestrogen levels
• Resorption at other sites, which can be –– Family history
intracortical, endosteal, subchondral, –– Low levels of weight-bearing exercise
subligamentous (in the calcaneus or –– Smoking
clavicle) or trabecular –– Alcohol abuse
• Osteopenia in the majority of cases –– Drugs, e.g. corticosteroids, heparin,
• Cortical thinning phenytoin, phenobarbital
• Soft tissue calcifications –– Poor nutrition
• Rarely, brown tumours
• Erosions in the sacroiliac joints, symphysis Imaging features
pubis, ligamentous insertions Plain X-ray
• Resorption of the distal or medial end of • Insensitive for the detection of
the clavicle and the vertebral endplates osteoporosis: 50% bone loss is required
(aggressive Schmorl’s nodes) before the loss can be seen

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Endocrine diseases 99

• Cortical thinning • ‘Rugger jersey’ spine: bands of hazy


• Fractures with delayed healing and poor sclerosis in parallel with the end-plates
callus formation • Profuse soft tissue calcifications and
Dual energy X-ray absorptiometry (DEXA) tumoural calcinosis
scan
• The most precise way of estimating disease Acromegaly
burden
• Excess of growth hormone
• It works by comparing a patient’s bone
• Leads to gigantism in skeletally immature
density with normal ranges in age-
people
matched populations (giving the Z score)
• Leads to bone widening and acral growth
and young populations (giving the T score)
in adults
• The T score is more useful as they give an
indication of fracture risk Imaging features
• T scores 1–2 standard deviations below • Soft tissue thickening over the phalanges
the mean density are defined as being and heel pad
osteopenic • Enlarged sella turcica
• T scores < 2 standard deviations below the • Prominence of facial bones, increased
mean density are defined as indicating vertebral body and disc height with
osteoporosis posterior vertebral scalloping
• Exaggerated thoracic kyphosis is
Renal osteodystrophy associated
• Enlarged paranasal sinuses with increased
• Bone changes in chronic kidney disease pneumatisation
• Secondary hyperparathyroidism, • Spade-like phalanges
osteomalacia, bone sclerosis and
aluminium toxicity all contribute to the
findings in renal osteodystrophy Hypothyroidism
Imaging features • Mild osteoporosis
• Soft tissue swelling
• Osteopenia and/or osteosclerosis
• Delayed skeletal maturity
• Changes due to osteomalacia and rickets:
• Delayed dental development
coarsened, blurred trabeculae and
• Wormian bones
metaphyseal fraying or cupping
• Bullet-shaped vertebrae at the
• Secondary hyperparathyroidism, with
thoracolumbar junction
typical changes of bone resorption, brown
• Stippled epiphyses
tumours, periosteal new bone formation
and chondrocalcinosis

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Infections and nutritional
2.4 and metabolic conditions

Infections • This is followed by signs of intramedullary


destruction: a subtle permeative pattern
Osteomyelitis within the bone or indistinctiveness of the
cortex
Aetiology
• Cortical destruction follows, with
• Haematogenous spread is the commonest accompanying endosteal cortical
mode scalloping and periosteal reaction
–– Neonates: Staphylococcus aureus, group • A sequestrum and involucrum eventually
B streptococci, Escherichia coli develop if left untreated
–– Children: Staphylococcus aureus • Cloaca: a cortical and periosteal defect
–– Sickle cell anaemia: staphylococci through which pus drains from infected
still predominate, but with a higher medullary cavity
incidence of Salmonella
–– Adults: Staphylococcus aureus Imaging features of chronic
–– IVDU: Gram-negative bacteria osteomyelitis
(Pseudomonas, Klebsiella) • Brodie’s abscess
• Contiguous spread from soft tissue ulcers • Thickened cortex (host reaction) with
• Spread along fascial planes from soft tissue mixed bone density
infections of the hand
Imaging features of acute
Tuberculous osteomyelitis
• Slower course and less host reaction than
osteomyelitis in pyogenic osteomyelitis
• Obliteration of soft tissue fat planes is often • In children tuberculosis may first manifest
the first sign itself in the small bones of the hands
• Osseous changes may not be evident for and feet, where it causes dactylitis (spina
1–2 weeks ventosa)

MRI findings in osteomyelitis

Situation T1WI T2WI T1WI plus gadolinium


Marrow inflammation Low signal High signal High signal
Intraosseous abscess Low signal High signal High signal in rim
Sequestrum Low signal Low signal High signal around
sequestrum
Cortical breech Intermediate signal Intermediate or Intermediate signal
high signal
Cloaca Hard to see in High signal Not seen
low-signal periosteum
Sinus tract Not seen High signal High signal

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Infections and nutritional and metabolic diseases 101

Infection versus neuropathy in diabetes: diabetic dilemma

Osteomyelitis Neuroarthropathy
Location Adjacent to ulcers Joints
Metatarsal heads Tarsometatarsal (Lis franc)
Calcaneal tuberosity Talonavicular and
Distal phalanges Calcaneocuboid
Malleoli Ankle and subtalar joint
Other features Cortical destruction Bone fragmentation
Sequestrum Malalignment
Abscess
Non-discriminatory Effusion
Soft tissue oedema
Bone marrow oedema
Periosteal reaction

Causes of rickets

< 6 months of age > 6 months of age


• Hypophosphatasia • Dietary deficiency of vitamin D
• Prematurity • Liver dysfunction
• Primary hyperparathyroidism • Malabsorption
• Maternal deficiency of vitamin D • Chronic renal disease

Diabetic dilemma Nutritional, metabolic and


• The dilemma is: infection versus haematological diseases
neuropathy? (See table above)
Rickets
Septic arthritis • Osteomalacia in the skeletally immature
• Early joint effusion • Caused by vitamin D deficiency (e.g.
• Osteoporosis and cartilage destruction inadequate dietary intake or reduced
follow secondary to local hyperaemia exposure to sunlight); see table above
• Erosion and destruction make way to
osteomyelitis Imaging features
• Ankylosis may eventually occur • Calcification of cartilage and osteoid
• Tuberculous and fungal septic arthritides does not occur, leading to widening of the
are more chronic processes than bacterial zone of provisional calcification, which
arthritis; cartilage destruction is slow and is perceived as metaphyseal widening on
the joint space remains relatively well plain X-rays
preserved till late • Widened and irregularly shaped
metaphyses with flaring is caused by stress
at sites of ligamentous attachments

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102 Musculoskeletal system and trauma

• Microfractures of the primary spongiosa Scurvy


are caused by protrusion of physeal
• Caused by a long-term deficiency of
cartilage
vitamin C
• Physeal width is 2.5–3 mm in rickets
(normal range is 0.9–1.9 mm) Imaging features
• Cupping and fraying of the metaphyses is • Ground-glass osteoporosis
typical • Increased density and widening of the
• Metaphyseal spurs are also a feature zone of provisional calcification (white line
• Poorly mineralised epiphyseal centres with of Frankl)
delayed appearance • Metaphyseal spurs or marginal fractures
• Periosteal reaction may be present (Pelkan’s sign)
• Coarse trabeculation is noted (but ground- • Transverse radiolucent band at the
glass appearance is not a feature) metaphysis subjacent to the zone of
• Soft bones with deformities such as provisional calcification (scurvy line or
bowing Trummerfield’s zone, which is a site of
• Risk of Salter–Harris type I fractures in fractures and infarction)
weight-bearing areas • Parke corner sign: subepiphyseal
• Skull: frontal bossing, premature fusion of infarction leading to cupping of the
the sagittal suture, delayed dentition and epiphysis
hypoplasia of enamel • Ring of increased density surrounding the
• Ribs: cupping of the ends of the ribs, with epiphysis (Wimberger’s sign)
widening of rib epiphyseal cartilage, giving • Periosteal elevation
a ‘rachitic rosary’ appearance • Sites of abnormalities include the distal
• Spine: scoliosis and biconcave vertebral end of femur, the proximal and distal ends
bodies of the tibia and fibula, the distal end of the
• Pelvis: coxa vara, and triradiate pelvis radius and ulna, the proximal humerus,
caused by inwardly migrating sacrum and and the distal ends of the ribs
acetabula
• Knees: genu varum or valgum Haemophilia
• Bleeding disorder
Osteomalacia • X-linked-recessive inheritance, hence
• Vitamin D deficient state in adults found only in males
• Osteoid formation is normal, but
mineralisation does not occur Imaging features
Associations include neurofibromatosis type • Multiple episodes of haemarthroses,
1 and haemangiopericytoma commonest in the knee, elbow and ankle
joints (large joints)
Imaging features • Dense effusions
• Generalised bone demineralisation: lucent • Hypertrophied synovium with
bones with indistinct trabecular detail and haemosiderin deposits (low signal on
thinned cortex T1WI and T2WI)
• Soft bones with various deformities such • Resulting hyperaemia causes osteoporosis
as protrusio acetabuli of adjacent bones and epiphyseal
• A specific feature is the appearance of overgrowth (enlargement and ballooning)
looser zones, or pseudofractures; these with early closure
are characteristically located in the medial • Hence there is widening of the
aspects of the proximal femurs, the pubic intercondylar notch on the femur
bones, the dorsal aspect of the proximal • Cartilage destruction, erosions and
ulnas and the distal aspect of the scapula subarticular cysts are typical
bones and ribs • Secondary degenerative joint disease
invariably ensues

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Infections and nutritional and metabolic diseases 103

• A pseudotumour of haemophilia may by the spine, the pelvis and then long
be seen: a non-neoplastic mass lesion bones such as the femur
appearing secondary to a bleed; pressure • Normal calcium and phosphate levels;
erosion occurs at the cortical region, raised alkaline phosphatase levels
with extrinsic or intrinsic scalloping and • Three stages of disease: lytic, mixed and
extensive periosteal reaction. A soft tissue sclerotic
mass may be associated with this • Complications
–– Osteoarthritis (in 50–96% of cases)
Sickle cell anaemia and –– Basilar skull invagination (in 30% of
thalassaemia cases)
• Haemoglobinopathy (glutamic acid in –– Insufficiency fractures, protrusions and
position 6 is substituted by valine) proximal femoral varus deformity may
• Autosomal-recessive inheritance be seen
–– Deafness (both types), due to spinal
Imaging features stenosis
• Patchy bone density secondary to chronic –– Sarcomatous transformation (in < 1%
bone infarcts of cases)
• Avascular necrosis –– Osteomyelitis and metastases are more
• Chest radiograph should be evaluated for common in Paget’s bones, owing to
cardiomegaly and pulmonary infarcts hypervascularity
• Gallstones and autosplenectomy may be
seen Imaging features
• Three characteristic features • Pathognomic triad
–– Marrow hyperplasia; in thalassaemia, –– Bone expansion
marrow hyperplasia is florid with –– Cortical thickening
widening of the diploic space, with –– Trabecular bone thickening
a ‘hair-on-end’ appearance; the • Disease starts at one end of the bone and
paranasal sinuses are often obliterated. progresses along the shaft
AVN is uncommon in thalassaemia • Osteoporosis circumscripta of skull in
–– Marrow infarction (dactylitis is a the active stage, cotton-wool appearance
common manifestation in the young; it with mixed lytic and blastic pattern of the
is a periosteal reaction with soft tissue thickened calvarium in late stages
swelling and avascular necrosis of the • Paget’s disease of the spine
hip and humeral head) –– Upper cervical spine, low thoracic spine
–– Osteomyelitis; while Salmonella and the mid-lumbar spine are the sites
osteomyelitis is known to occur in this most commonly affected
patient group, staphylococci remain the –– Enlarged vertebral body, with a ‘bone-
commonest pathogens overall within-bone’ appearance
–– Ivory vertebrae
Paget’s disease –– Ossification of spinal ligaments,
• Chronic skeletal disease characterised by paravertebral soft tissue and disc spaces
abnormal osteoblastic and osteoclastic
activity resulting in abnormal bone Osteopetrosis
remodelling • Increased bone density due to impaired
• Rare before the age of 40 years; 3% occur osteoclastic function
in those > 40 years of age, 10% in those > 80 • Bone marrow failure ensues in severe
years of age forms, contributing to mortality
• Males are more commonly affected than • Pathological fractures (usually heal well)
females • Associated with rickets (secondary to
• Most commonly affects the skull, followed sequestration of calcium within bones)

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104 Musculoskeletal system and trauma

Imaging features Vertebral imaging


• ‘Bone-within-bone’ appearance
Skull imaging
• ‘Picture-frame’ vertebrae with sclerotic
• Narrowed foramina
borders
• Underdeveloped paranasal sinuses
• Tendency to infection
• Retention of deciduous teeth
• Hypertelorism

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2.5 Trauma

Cervical spine Thoracic and lumbar spine


• The normal basion–axial distance and the injuries
normal basion–dental distance is 12 mm • Injury patterns
Injury mechanisms –– Compression and wedge fractures
–– Burst fractures
Hyperflexion –– Chance fractures: ‘lap-belt’ injury
• Hyperflexion sprain caused by sudden, severe flexion that is
• Hyperflexion dislocation (without facet usually centred on the thoracolumbar
lock or with unilateral or bilateral facet region; injury can be purely bony,
lock) purely ligamentous or a combination of
• Comminuted (‘tear-drop’) the two
• Burst: two-column injury, with the fracture
extending to involve the posterior cortex of Spondylosis
the vertebral body • Defect in the pars interarticularis
• Hyperflexion fracture–dislocation • A form of chronic stress fracture, with non-
• Occipito-atlantal dislocation or union, typically seen in adolescents who
subluxation engage in sport
• Atlantoaxial dislocation • Commonly seen at the L4–L5 and L5–S1
• Anterior or lateral fracture–dislocation of levels
the dens
• Clay shoveler’s fracture: a fracture through Spondylolisthesis
the spinous process at the C6–T1 levels • Bilateral pars defects lead to ventral
subluxation of the vertebral body
Hyperextension • Aetiologies include dysplastic, isthmic
• Hangman’s fracture: hyperextension and (commonest type secondary to
traction injury of the second cervical spondylolysis), degenerative, traumatic
vertebra and pathological changes
• Hyperextension sprain • Four grades are described, based on the
• Posterior fracture: dislocation of the dens degree of anterior displacement
• Posterior atlantoaxial dislocation
Axial compression Upper limb trauma
• Jefferson’s fracture, from a compression
force to the first cervical vertebra; typical
Thumb fractures
in diving injuries • Bennett’s fracture
• Occipital condyle fracture type 3 –– Unstable fracture
–– Intra-articular fracture involving the
Unstable injury patterns base of the first metacarpal
• Bilateral locked or jumped facets –– The distal metacarpal fragment is
• Flexion tear-drop fractures pulled dorsally and laterally by the
• Hangman’s fracture abductor pollicis longus muscle
• Hyperextension dislocation–fracture • Rolando’s fracture
• Jefferson’s facture –– Unstable fracture
• Odontoid fracture (particularly type 2) –– Intra-articular, comminuted fracture of
the base of the first metacarpal

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106 Musculoskeletal system and trauma

• Extra-articular fracture
–– Stable injury Dorsal intercalated segment
–– The CMC joint is not involved instability (DISI) or dorsiflexion
–– Treated by closed reduction instability
• Gamekeeper’s or skier’s thumb
–– Injury to the ulnar (medial) collateral • The lunate is angulated dorsally
ligament at the MCP joint, occasionally • The scapholunate angle is increased
accompanied by an avulsion fracture of (30–60° is normal, 60–80° is
the proximal phalangeal base questionably abnormal, > 80° is
–– Steners lesion represents an abnormally abnormal)
interposed adductor aponeurosis • The capitolunate angle is increased
between the torn ulnar collateral (< 30° is normal)
ligament and its distal attachment to
the proximal phalangeal base

Carpal injuries Volar intercalated segmental


instability (VISI), volarflexion
• Fractures of the scaphoid account for
instability or palmar flexion instability
60–70% of all carpal injuries, fractures of
the triquetrum for 7–20%, while trapezoid
fractures are the rarest type • The lunate is tilted in a palmar direction
• Scaphoid fracture • The scapholunate angle is decreased
–– Peak age is 15–40 years (to < 30°)
–– The arterial supply enters the waist • VISI may be a normal variant, especially
of the scaphoid on the anterolateral if the wrist is very lax
surface
–– 70% in waist, 10% proximal pole, 20%
• Lunate dislocation
distal pole
–– Most severe carpal injury, and
–– Healing without periosteal callus
associated with a trans-scaphoid
–– Delayed or non-union is encountered
fracture
in practically every proximal pole
–– The lunate dislocates anteriorly (in a
fracture and 30% of waist fractures
volar direction); the radius and capitate
Imaging features remain in a straight line
• Views in a scaphoid series: –– Triangular appearance of lunate on
posteroanterior, lateral, 45° pronation frontal projection
oblique, and posteroanterior view with • Perilunate dislocation
ulnar deviation –– Results from fall on the outstretched,
hyperextended hand
MRI –– Less common than lunate dislocation
• Fracture line appears as low signal on –– Associated with scaphoid waist
T1WI, high signal on T2WI fractures
• Sagittal images show scaphoid flexion –– Whole of the carpus, except the lunate
(humpback deformity) is displaced posteriorly; the radius and
• Avascular necrosis is seen as loss of lunate remain in a straight line, and the
marrow signal, low signal on T1WI and capitate lies posteriorly and out of line
T2WI; the risk of avascular necrosis is –– Triangular appearance of lunate on
highest with proximal pole fractures, while frontal view
there is no such risk with fractures of the • Triangular fibrocartilage tears
distal pole –– The triangular fibrocartilage is
Bone scan composed of the dorsal and volar
• May be negative in the first 48 hours radioulnar ligaments, as well as the
(unlike MRI, which will be positive) articular disc

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Trauma 107

–– It is a biconcave disc of hypointense • Galeazzi fracture


signal on MRI –– Fracture of the shaft of the radius
–– Triangular fibrocartilage tears are a associated with dislocation at the distal
cause of ulnar-sided wrist pain and radio-ulnar joint
are associated with positive ulnar • Fractures of the olecranon
variance, while negative ulnar variance –– Account for 20% of all elbow injuries
is associated with Kienböck’s disease –– Apophysis fuses by the age of 14–16
(avascular necrosis of the lunate) years
–– On MRI, the presence of fluid extending –– Inability to extend the elbow is
across the articular disc or discontinuity characteristic of olecranon injuries and
in the contour of the triangular is caused by triceps tendon dysfunction
fibrocartilage is pathognomonic or injury
–– Chondromalacia of the lunate, –– Soft tissue swelling in the olecranon
triquetrum or ulna are related findings, bursa indicates injury
particularly when there is positive ulnar –– A posterior fat pad is a typical imaging
variance feature
–– Likely to need surgical intervention
in patients < 40 years of age, in whom Elbow dislocation
trauma is often the aetiology • Commonest site of dislocation in children
–– Degenerative tears are common in • Posterior or posterolateral (displacement
patients > 40 years of age, and treatment of radius and ulna in relation to the
is usually conservative humerus) in 80–85% of cases
• Other forms are lateral, anterior and
Fractures of the distal radius medial
• Colles’ fracture • Associated with fractures in 20–60% of
–– Dorsally angulated, impacted distal cases, most commonly of the medial
radial fracture with dorsal and radial condyle or epicondyle, followed by the
displacement of the distal fracture head or neck of the radius
segment • Myositis ossificans appears in 3% of all
–– More frequent in osteoporotic women elbow injuries, but in 89% of fractures and
• Smith’s fracture dislocations about the elbow in adults with
–– Reverse Colles’ fracture, with volar associated head injury
angulation and volar displacement of • Usually appears within 3–4 weeks of injury
distal fracture segment • Usually anterior to the joint
• Barton’s fracture • Incidence increased by too early return to
–– Unstable intra-articular fracture of the activity following an injury
distal radius
–– The fracture extends through the dorsal Supracondylar injuries
margin of the radius, with associated • These fractures account for > 60% of all
dorsal dislocation of the carpus elbow fractures in children
• Reverse Barton’s fracture • Hyperextension type of injury caused by a
–– The anterior (volar) cortex of the distal fall on the outstretched hand in > 95% of
radius is involved cases
–– A highly unstable injury • The elbow becomes locked in
hyperextension
Fractures of the radius and The olecranon is displaced superiorly into
ulna the olecranon fossa, causing the anterior
• Monteggia fracture humeral cortex to bend and eventually
–– Fracture of the shaft of the ulna break
associated with dislocation of the head • If the force continues, both the anterior
of the radius and posterior humeral cortices can
fracture

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108 Musculoskeletal system and trauma

• If there is only minimal or no glenohumeral ligament), leading to


displacement, these fractures can be joint laxity
occult on X-ray, and the only sign will be a –– Hill–Sachs lesions in 77% of cases
positive fat pad sign –– Rotator cuff tears
• Usually there is some displacement, and –– Anterior labroligamentous periosteal
the anterior humeral line will not usually sleeve avulsion (ALPSA), which
pass through the centre of the capitellum; represents a cartilaginous variant
rather it will traverse the anterior third of Bankart’s tear in which the torn
of the capitellum or even pass further labroligamentous complex is displaced
anterior to the capitellum medically and inferiorly and the
scapular periosteum is preserved at the
Shoulder dislocation site of the labral tear
• The most commonly dislocated joint –– Perthes’ lesion, another Bankart
• Anterior dislocation: arm held in variant, in which the scapular
abduction and external rotation; account periosteum remains intact but is
for 95–98% of emergency/casualty stripped medially, and the anterior
admissions for shoulder dislocation labrum is avulsed from the glenoid
• Posterior dislocation: common during but remains partially attached to the
seizure or electrocution, arm held in scapula by the intact periosteum
adduction and internal rotation –– Glenoid labral articular disruption
• Inferior dislocation: arm fully abducted (GLAD), a tear of the anteroinferior
with elbow commonly flexed; account for labrum with avulsion of the adjacent
0.5% of emergency/casualty admissions glenoid hyaline cartilage; the lesion is
for shoulder dislocation clinically stable because the labrum is
not detached; the mechanism of injury
Imaging features is glenohumeral impaction in the ABER
Plain X-ray (ABduction and External Rotation)
• Anteroposterior view position
–– Anterior dislocation characterised by • Injuries associated with posterior
subcoracoid position of the humeral dislocation
head –– Posterior labral tear or detachment
–– Posterior dislocation characterised by (reverse Bankart’s tear)
‘light-bulb’ appearance of the humeral –– Tear of the posterior band of the inferior
head, although this is not always seen; glenohumeral ligament
it can be diagnosed radiographically –– Reverse Hill–Sachs lesion (impacted
by identifying subtle asymmetry of the fracture of the anterior aspect of the
glenohumeral joint space in a post- humeral head), giving a ’trough’ sign
trauma setting –– Teres minor injury
• Injuries associated with anterior
dislocation Subacromial subdeltoid bursal
–– Labral lesions, e.g. anteroinferior impingement
Bankart’s tear, which is the most • Compression of the supraspinatus
common lesion in anterior muscle between humeral head and the
glenohumeral instability and is coracoacromial arch
associated with a torn scapular • Common in occupations and sports that
periosteum involve overhead activities
–– Bony glenoid lesions, e.g. osseous • Anatomical variants that predispose to the
anterior glenoid rim fractures in 44% of impingement
cases –– Curved acromial arch
–– Ligamentous lesions (particularly –– Anteroinferiorly hooked acromion
of the anterior band of the inferior

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Trauma 109

–– Enthesophyte underneath the acromion anterior arch and the posterior arch on the
–– Lateral acromial downsloping ipsilateral side
–– Os acromiale • Results in double vertical fractures, most
commonly through superior and inferior
Imaging features pubic rami and sacrum/ilium
Ultrasound • Usually results in superior displacement of
• Bunching of the subacromial subdeltoid the affected hemipelvis and hip
bursa during abduction
Straddle fracture
MRI • Injury resulting from landing on a hard
• Thickened heterogeneous or torn tendon, object in the straddle position, as seen in
with fluid in the subacromial bursa, bicycle accidents
• Increased signal in the distal supraspinatus • Bilateral fractures of pubic and ischial rami
tendon on T1WI and T2WI • Medial fracture fragments are usually
elevated
Lower limb trauma • Associated with urethral and bladder
injuries in 20% of cases
Pelvic injury
• Pelvic fractures can be stable or unstable
Pelvic dislocation
• Stable pelvic fractures • Sprung pelvis (‘open-book’ injury) is
–– Consist of single breaks in the pelvic usually associated with genitourinary
ring or fractures of the peripheral injury with disruption of the pubic
margins that do not disrupt the ring symphysis and bilateral sacroiliac joints
–– Account for two thirds of all pelvic • Occurs secondary to an anteroposterior
injuries compression force
–– Unilateral fracture of the pubic ramus • Normal sacroiliac joint space is 1–4 mm
(commonest pelvic fracture) • Normal width of the pubic symphysis is
–– Iliac wing fracture (Duverney’s 5 mm
fracture) involves the lateral margin Bucket-handle fracture
of the ala and is caused by direct • Fracture of the anterior arch and the
lateral compression; associated with contralateral posterior arch
paralytic ileus and abdominal rigidity • Rare
(due visceral injury or irritation of
the peritoneum by the underlying Incidences of types of pelvic injury
haematoma)
–– Sacral fractures are usually transverse
and in the S3–S4 region • Vertical shearing: 10%
• Unstable fractures are those that disrupt • Anterior compression: 15%
the pelvic ring • Lateral compression: 65%
–– Typical examples include Malgaigne’s • Mixed: 10%
fracture, straddle fracture, pelvic
dislocation and bucket-handle fracture
• Posterior arch fractures have a higher Acetabular fracture
morbidity than anterior arch fractures • 20% of all pelvic fractures involve the
• Overall mortality in pelvic fractures is acetabulum
between 9-19% • CT is the best modality of evaluating
Malgaigne’s fracture acetabular fractures
• Acetabular fractures are classified into four
• Commonest unstable pelvic fracture
types: posterior rim fractures, transverse
(accounting for 14% of all types)
acetabular fractures, anterior column
• Vertical shearing force involves both the
fractures and posterior column fractures

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110 Musculoskeletal system and trauma

• Posterior rim fracture –– Most commonly involves the liver,


–– Most common acetabular fracture, seen spleen and the left side of the
in 33% of cases diaphragm
–– Occurs with posterior hip dislocation, • Neural injury
the commonest type of hip dislocation –– The sciatic nerve is susceptible to
• Transverse acetabular fracture injury in posterior fracture–dislocation
–– Separates the bone into the halves injuries
–– Disrupts along the iliopubic and –– Reported incidence of neurological
ilioischial lines deficits in conjunction with pelvic
–– May be associated with central fractures is approximately 12%; they are
dislocation of femoral head more likely to occur in sacral fractures
• Anterior column fracture • Bowel injury
–– Fracture through the iliopubic line –– Rare but carries a high mortality
–– May be associated with central –– Small bowel is more susceptible than
dislocation of femoral head the large bowel
• Posterior column fracture –– Paralytic ileus is a common occurrence
–– Fracture through the ilioischial line in pelvic injuries
–– May be associated with central • Infections
dislocation of femoral head –– Retroperitoneal abscesses may form,
even 3 months after injury if there has
Avulsion fractures of the pelvis: been an open fracture
patterns and affected attachments
• Ischial tuberosity (hamstrings) Knee injuries
• Anterior inferior iliac spine (rectus Meniscal tears
femoris-straight head)
• Meniscal tears occur in two primary
• Anterior superior iliac spine (sartorius)
planes, vertical and horizontal
• Iliac crest (abdominal muscles)
• Three basic shapes of meniscal tears:
Complications and associated longitudinal, horizontal and radial;
injuries complex tears are a combination of these
• Haemorrhage basic shapes
–– Occurs 75% of cases of pelvic fracture • Two most important criteria for detecting
–– Usually arises from laceration of one meniscal tears
or more branches of the internal iliac –– Abnormal shape
artery, particularly the superior gluteal –– High signal intensity unequivocally
branch reaching the meniscal articular surface
• Urinary tract injury (superior or inferior, or both) on sagittal
–– All patients who have sustained a or coronal proton-density images
pelvic fracture must be assumed to Displaced tears
have a urinary tract injury until proven • Bucket-handle tear is a displaced
otherwise longitudinal tear, which is best recognised
–– Urethra is injured in 4–17% of cases, on coronal images
almost exclusively in males • Flap tear is a displaced horizontal tear
–– The urethra at or above the level of • Parrot-beak tear is displaced radial tear; it
the urogenital diaphragm (posterior is an unstable tear
or membranous urethra) is the most • Normally there are only two structures in
susceptible to injury the intercondylar fossa: the anterior and
–– The bladder is injured in 4% of cases, posterior cruciate ligament; any other
and in 60–80% of these cases the injury structure in the intercondylar fossa is
is extraperitoneal abnormal and should raise suspicion for a
• Visceral injury displaced meniscal fragment
–– Occurs in 20% of cases

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Trauma 111

Meniscal cyst • There is non-visualisation of 5–10% of


• Horizontal tears divide the meniscus into normal anterior cruciate ligaments on
top and bottom parts sagittal-plane views
• If a horizontal tear extends all the way • Injury mechanisms and patterns
from the apex to the outer margin of the –– Valgus and anterolateral rotatory
meniscus, it may result in the formation of subluxation of the knee typically causes
a meniscal cyst injury to the anterior cruciate ligament
• Synovial fluid runs through the horizontal –– Commoner in females and young,
tear and accumulates peripherally, active people
resulting in a meniscal cyst –– Location of rupture is mostly near
• A meniscal cyst can lose its femoral origin of the ligament
communication with the joint space and –– MRI does not accurately differentiate
hence may not fill with contrast on MR between partial and complete tears
arthrography –– On X-rays an important indirect sign
of an anterior cruciate ligament tear
The three criteria for the diagnosis is a Segond fracture, which is a bony
of a meniscal cyst avulsion injury of the iliotibial band or
the lateral collateral ligament (anterior
oblique band)
1 Horizontal tear
2 Focal area of fluid accumulation (bright
on T2WI)
Indirect signs of an anterior
3 Adjacent to the peripheral margin of
cruciate ligament tear
meniscus
High specificity
• Deep lateral femoral notch sign
• Abnormal orientation of the posterior
Discoid meniscus cruciate ligament
• Large dysplastic meniscus with loss of the Low sensitivity
normal semilunar shape • Bone contusions of the posterior aspect
• Commonly affects the lateral meniscus of the lateral tibial plateau and the mid-
and may be bilateral portion of the lateral femoral condyle
• Patients usually present with pain, clicking • Anterior tibial translocation
and snapping of the knee • Uncovering of the posterior horn of the
• Continuous appearance of the meniscus lateral meniscus
on three consecutive, 4–5 mm thick sagittal • Visualising the lateral collateral
slices is characteristic ligament in just one coronal image
(rather than two or three images)
Ligamentous injury • Fracture with high association of
Anterior cruciate ligament anterior cruciate ligament tears,
• Normally an intracapsular extrasynovial including a Segond fracture
structure
• Primary stabiliser against anterior tibial
subluxation Posterolateral corner injury
• Composed of between three and five layers • Infrequent injury
of fibres • Usually associated with anterior cruciate
• Between the fibres there can be fat or ligament and/or posterior cruciate
synovium or sometimes a small amount ligament injuries
of fluid. This explains the fact that the • Severe disability ensues from
anterior cruciate ligament is not entirely of unrecognised injury, owing to instability
low signal on proton-density images and articular degeneration
• Oriented at a 55° incline to the tibial
plateau

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112 Musculoskeletal system and trauma

• Missed injury can result in failure of Posterior cruciate ligament


reconstructed anterior cruciate ligament • Prevents posterior subluxation of the tibia
or posterior cruciate ligament • Isolated injuries to the posterior cruciate
• If a bone bruise is seen anteromedially, ligament are rare
ligamentous injury on the contralateral • A typical scenario would be a dashboard
side (the posterolateral corner) ought to be injury, which is a hyperflexion mechanism
suspected of injury

Posterolateral corner of the knee Medial cruciate ligament


• Extends from the medial epicondyle to
The posterolateral corner (or insert along the medial tibia, usually 7 cm
posterolateral complex) is a complex below the joint space
anatomical system that contains seven or • A long ligament, which firmly adheres to
eight structures. The orthopaedic surgeon the joint capsule and the medial meniscal
is interested particularly in three of these body
structures, which are easily visualised on Lateral collateral ligament (fibular
MRI:
collateral ligament)
1 Lateral collateral ligament (fibular
• Part of the posterolateral complex of the
collateral ligament)
knee
2 Biceps femoris muscle and tendon
• Originates in a sulcus along the lateral
3 Popliteus tendon
femoral condyle to insert as a conjoint
tendon with the biceps femoris tendon on
to the fibular head
Contusion patterns in knee trauma
• Pivot shift injury: contusion in the Sites of post-traumatic avascular
posterolateral tibial plateau and the mid- necrosis
portion of the lateral femoral condyle
• Dashboard injury: contusion in the • Hip involvement of one hip increases
anterior aspect of the proximal tibia the risk to the contralateral hip (in such
• Hyperextension injury: kissing cases, avascular necrusosi is seen in up
contusion between the anterior aspect to 70%)
of the proximal tibia and distal femur; an • Blount’s disease (also called tibia vara)
anteromedial bone bruise is seen is avascular necrosis of the medial tibial
• Clip injury: contusion in the lateral and condyle
medial femoral condyles • Kienböck’s disease is avascular necrosis
• Lateral patellar dislocation: oedema in of the lunate bone
the inferomedial patella and the anterior • Köhler’s disease is avascular necrosis of
aspect of the lateral femoral condyle the tarsal bone

The ‘unhappy’ triad (O’Donoghue’s


triad)
Perthes’ disease
Commonly occurs in contact sports such
as football when the knee is hit from the • Avascular necrosis of the femoral head in
outside, which causes an injury to three children
knee structures: • Peak age is 4–8 years
1 Anterior cruciate ligament • Bilateral in up to 10% of cases
2 Medial cruciate ligament • Males are five times more commonly
3 Medial meniscus affected than females

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Trauma 113

• Usually idiopathic, but may be secondary Imaging features


to trauma • Widened growth plate (prior to the slip)
Imaging features • Posteromedial displacement of head
during the slip
Plain X-ray • Decrease in the neck–shaft angle
• Smaller femoral epiphysis on the affected • If long-standing, then one sees sclerosis
side, typically with sclerosis and irregularity with a widened physis
• Widened joint space (secondary to
thickened intra-articular cartilage, joint
fluid or joint laxity) Stress fractures
• Later, a lucent subchondral fracture line • May be an insufficiency fracture or a
appears, followed by fragmentation of the fatigue fracture
femoral head • Insufficiency fracture
CT –– Normal stresses but abnormal bone
• Loss of asterisk in the centre of the femoral –– Causes include osteoporosis,
head (‘asterisk’ refers to the stellate pattern osteomalacia, steroids, Paget’s disease,
of crossing trabeculae in the centre of a hyperparathyroidism, rheumatoid
normal femoral head) arthritis and radiation
• Fatigue fracture
MR –– Abnormal stress on normal bone
• Marrow signal from femoral epiphysis is
–– Causes include new activity,
low on T1WI and high on T2WI
poor equipment and abnormal
• Double-line sign: sclerotic rim between
biomechanics
viable and non-viable bone is edged by a
high-signal rim of granulation tissue
Osteochondritis dissecans
Slipped femoral epiphysis • Subchondral fracture
• Often seen in athletes
• Atraumatic fracture through the physeal
• Typically affects the lateral aspect of the
plate
medial femoral condylar articular surface
• Usually one-sided
• Other sites include the weight-bearing
• Associations
surfaces of the lateral femoral condyle,
–– Trauma (Salter-Harris type I injury)
tibia, talus, patella and capitellum
–– Growth spurt (commonly seen in boys
aged 8–17 years) Imaging features
–– Renal osteodystrophy and rickets • Subchondral fracture line paralleling the
–– Childhood irradiation joint surface in a concave fashion
–– Growth hormone or steroid treatment
MRI
–– Developmental dysplasia of the hip
• The focus is seen as low signal on T1WI
–– Perthes’ disease
and T2WI, with variable amounts of
–– Endocrine problems, e.g.
oedema
hypothyroidism, hypoestrogenism,
• Loose osteochondral fragment are
acromegaly, cryptorchidism, pituitary
demonstrated on all MR sequences
adenoma, parathyroid adenoma
• An osteochondral fragment is unstable
• Complications
when a T2 high-signal line (representing
–– Avascular necrosis (seen in 10–15% of
fluid) separates an undisplaced
cases)
osteochondral fragment from native bone
–– Coxa vara
• Other signs of instability are a displaced
–– Osteoarthritis (in 90% of cases)
fragment and adjacent cyst formation
–– Discrepancy in limb length

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114 Musculoskeletal system and trauma

Tendon injuries in the lower • Occurs after trauma and burns and in the
neurological impairment
limb • The thigh and the elbow are common
Achilles tendon tear locations, but it can occur anywhere
• Related to the time since the trauma
• Typically seen in middle-aged men who –– < 2 weeks after the trauma: soft tissue
participate in athletic sports mass that is painful, warm and doughy
• Usually ruptures 2–6 cm superior to the os to the touch
calcis –– 3–4 weeks after the trauma: amorphous
• The tendon tears at its myotendinous density within the mass, with associated
junction in cases of direct trauma underlying bony periosteal reaction
Imaging features –– 6–8 weeks after the trauma: amorphous
bone matures into compact bone that
MRI surrounds a lacy pattern of less mature
• Normal tendon is uniformly hypointense bone
on all sequences –– Maturation proceeds centripetally, and
• Disruption in continuity with or without a it takes 5–6 months to form mature
wavy retracted tendon is diagnostic bone
• Haemorrhage, fluid and interposed fat can
appear hyperintense Imaging features
Tibialis posterior tendon tear MRI
• Early: isointense to muscle on T1WI,
• May be spontaneous or associated with high signal on T2WI, with prominent
synovitis, corticosteroid injections or surrounding oedema; periosteal reaction
trauma and bone marrow oedema may be present
• Chronic degeneration occurs in women in • After 8 weeks: inhomogeneous centre and
their fifth and sixth decades a rim that is low intensity on all sequences
• Clinically presents with medial foot pain • Parosteal osteosarcoma is the closest
and flat foot differential diagnosis to myositis
• Unilateral in 90% OF CASES ossificans; it shows as reverse-zoning
• Usually ruptures within 6 cm proximal to organised bone centrally and as less
the navicular insertion mature bone peripherally
Anterior talofibular ligament Progressive myositis ossificans
tear (fibrodysplasia ossificans
• The anterior talofibular ligament is the progressiva)
lateral ankle ligament that tears most
• Hereditary mesodermal disorder
commonly
• Autosomal-dominant inheritance
• Caused by inversion and internal rotation
• Progressive ossification of striated muscle,
combined with plantar flexion
tendons and ligaments
• Associated with anterior displacement of
• Muscle involvement secondary to pressure
the talus and contusion of the medial part
atrophy from interstitial tissues
of the talus
• Acute torticollis is the most frequent
presentation
Myositis ossificans • Bridging heterotopic bone seen in severe
• Formation of mature bone within soft cases
tissues • Death may result from respiratory failure
caused by chest wall restriction

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2.6 Tumours

Benign tumours of bone Multiple enchondromas (Ollier’s


disease)
• Mnemonic from Helms (2005):
‘FEGNOMASHIC’ (Fibrous dysplasia, • Not premalignant on its own
Enchondroma, Giant cell tumour, Non- • Not hereditary
ossifying fibroma, Osteoblastoma, • Called Maffucci’s syndrome when
Metastatic disease, Aneurysmal bone cyst, associated with soft tissue haemangiomas,
Simple bone cyst, Hyperparathyroidism, and predisposes to malignancy
Infection (osteomyelitis), Eosinophilic granuloma
Chondroblastoma).
• Peak age is 5–10 years
Fibrous dysplasia • Usually monostotic, but progresses to
• Monostotic (most commonly) or polyostotic disease within 6 months in
polyostotic (in 30% of cases) 10–20% of cases
• Predilection for the pelvis, femur, ribs and Imaging features
skull • Variable appearances: lytic or sclerotic,
• Polyostotic type occasionally occurs well defined or ill defined, periosteal
in association with McCune–Albright reaction present or absent
syndrome • Periosteal reaction, when present, is thick,
• When seen in the tibia and jaw, consider wavy and uniform
adamantinoma (a rare malignant tumour • May appear aggressive with a permeative
that radiologically and histologically pattern and a soft tissue component
resembles fibrous dysplasia • May occasionally have a bony sequestrum
Imaging features Giant cell tumour
• Expansile, with ground-glass matrix and a
Four important criteria, according to Helms:
sclerotic rind
–– Occurs only in patients with a closed
• Often purely lytic, but may progress to
epiphysis
matrix calcification and appear sclerotic
–– Lesion is epiphyseal and abuts the
(lesions in skull may be densely sclerotic)
articular surface
• No periosteal reaction unless fractured
–– Eccentric location
Enchondroma –– Sharp non-sclerotic zone of transition
• Metaphyseal cartilage tumour Non-ossifying fibroma and
• Can be central or eccentric, expansile or
non-expansile
fibrous cortical defect
• 50% of cases occur in tubular bones • Metaphyseal defect
(phalanges) of hands and feet, where the • Common in children (30% of cases occur
tumour appears lucent in children; rarely seen after the age of 30
• Calcified chondroid matrix is invariably years)
present except when located in the • Males are twice as commonly affected as
phalanges females
• Sclerotic margin is absent or thin • Involutes after 2–4 years
• No periosteal reaction • Known as a non-ossifying fibroma when
large (> 2 cm in length)
Imaging features
Imaging features
MRI
• Lobulated, bright signal on T2WI with Plain X-ray
low-signal calcification, arcs (chondroid • Most commonly around the knee, but can
matrix) and ring-enhancement pattern occur in any long bone

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116 Musculoskeletal system and trauma

• Sclerotic border with mild endosteal in particular), the skull, the ribs and the
scalloping proximal humerus
• Slightly expansile • Breast primary tumour
• No periosteal reaction –– Bone involvement in 24% of early cases
Bone scan and MRI and 84% of advanced cases
• Minimal or mild uptake, hot during –– Osteolytic (most commonly) or
‘healing’ phase osteoblastic (in 10% of cases) or mixed
• MRI shows that 80% of cases are (in 10% of cases)
hypointense on both T1WI and T2WI; –– Common sites are the vertebrae, pelvis
others are hypointense on T1WI and and ribs
hyperintense on T2WI • Prostate primary tumour
• Intense contrast enhancement in 80% of –– Predominantly blastic or mixed
cases –– Florid periosteal ‘sunburst’ appearance
• Hypophosphatemic rickets a known is known to occur
complication • Lung primary tumour
–– Usually primaries are small cell
Jaffe–Campanacci syndrome carcinoma and oat cell carcinoma:
• Non-ossifying fibroma with extraskeletal majority of bone secondaries are
manifestations in children osteolytic
• Associations include mental retardation, –– Adenocarcinoma and bronchial
hypogonadism, ocular defect, congenital carcinoid normally produce focal or
heart defect and café-au-lait spots diffuse densely blastic lesions
• Kidney or thyroid primary tumour
Osteoblastoma –– Almost all bone secondaries are lytic
• Most commonly occurs in the posterior • Gastrointestinal primary tumour
elements of the vertebral bodies –– Bone secondaries are usually lytic,
• Speckled calcification or ossification seen but sclerotic metastases are seen with
in half of all cases adenocarcinoma
• Expansile, lytic lesion • Bladder primary tumour
–– Bone secondaries usually lytic, but
Metastatic disease exuberant periosteal new bone may be
• Commonest malignant tumours affecting seen occasionally
the skeleton –– Predilection for the bones of the legs
• Good evidence suggests that distant
metastases spread from venous tumour
Imaging features
emboli, rather than via arterial or • Features favouring a diagnosis of
lymphatic routes metastases rather than primary
• Common organs for the primary malignancy include a diaphyseal location
malignancy are the breast, bronchus, and involvement of the vertebral body and
prostate, kidney and thyroid pedicles
• The site of the primary carcinoma may • Consider an alternate diagnosis in
remain unknown in as many as 17.6% of the absence of bone expansion, florid
patients even after full investigation periosteal reaction, new bone formation
• Skeletal biopsy is confirmatory and a large soft tissue mass
• Bones containing red marrow are the most • Kidney, lung, thyroid and breast primaries
commonly involved (85–90% of cases), commonly give lytic bony metastases
and the axial skeleton is more commonly • Prostate and breast primaries commonly
involved than the appendicular skeleton give sclerotic bony metastases; bladder
• Common sites are the vertebrae, pelvis, and carcinoid primaries uncommonly give
proximal femur (the lesser trochanter sclerotic bony metastases

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Tumours 117

Bone scan Imaging features


• Most cost-effective modality
MRI
• 5% of metastases have a normal scan
• Low tumour burden: normal scan
• Abnormal uptake relies on intact local
• Focal involvement: identical picture to that
blood flow and increased osteoblastic
seen with metastases
activity
• Pre-treatment:
• Superscan may be seen with diffuse
–– T1 signal equal to or lower than that of
osteoblastic metastases from the prostate
muscle
or breast: presence of generalised increase
–– Diffuse enhancement with contrast
in skeletal activity with reduced or absent
• Post-treatment
renal activity seen on scanning
–– Complete response: resolution with
• Baseline scan has a high sensitivity for
no enhancement or persistent lesions
breast, prostate and lung metastases, but a
with no (or rim) enhancement (plain
poor sensitivity for infiltrative lesions like
X-rays may show sclerotic rim around
multiple myeloma, neuroblastoma and
the lytic lesions)
histiocytosis
–– Incomplete response: persistent
• Follow-up scan: a stable scan suggests a
enhancement
relatively good prognosis
• However, increased activity in a follow-up Osteoid osteoma
scan may be seen • A hamartoma composed of osteoid and
–– Enlargement of lesions woven bone, < 1.5 cm in diameter
–– New lesions • Peak ages is the second and third
–– Healing flare with treatment (this decades
does not signify tumour is responding • Males are 2.5 times more commonly
favourably to treatment) affected than females
–– Avascular necrosis • Pain responsive to aspirin
–– Radiation-induced changes • Commoner in the appendicular skeleton
• Decreased activity implies osteolytic • Painful scoliosis: concave towards lesion
lesions, or it may occur following • No site in the body is exempt
radiotherapy • Can involve the medulla or cortex or be
MRI subperiosteal; can involve the metaphyses
• Most metastases are located in the or diaphyses
medulla and show reduced signal intensity
on T1WI and increased signal intensity on
Imaging features
T2WI and short inversion time inversion • Nidus may be lucent or sclerotic, is 10 mm
recovery (STIR) sequences or less in diameter and is surrounded by
• Osteoblastic metastases may appear reactive sclerosis
hypointense on all sequences Bone scan
• Identification of a halo of high signal • Double-density sign (focal increased
around a lesion is a suggestive feature of activity surrounded by area of slightly
metastasis on T2WI lesser intensity, caused by reactive
sclerosis
Multiple myeloma
CT
• Solitary or multiple punched-out, lytic
• Cortical tumors often show a lucent nidus
lesions
with surrounding reactive sclerosis and
• Diffuse osteopenia
periosteal reaction
• Plasmacytoma shows as a focal lytic
• Brodie’s abscess can have a similar
expansile lesion
appearance
• Bone scan and skeletal survey are
• CT guidance is necessary for
complementary
radiofrequency ablation, the current
• MRI may be used as a survey tool

Ch-02.indd 117 8/12/2010 12:18:13 PM


118 Musculoskeletal system and trauma

commonly practised method for treatment • Fluid–fluid levels (a non-specific feature


of osteoid osteomas since this can be seen in several other
MRI bone tumours)
• Complements CT and bone scan Solitary bone cyst
• Shows intense oedema on STIR sequences
• Most commonly occurs in the first and
around the nidus
second decades of life
• Commonest sites are the proximal
Intra-articular osteoid osteoma
humerus (in 50% of cases) and the
proximal femur (in 20% of cases)
• A rare form of the disease • Central, metaphyseal location
• Usually presents as a rounded soft- • Mildly expansile with cortical thinning,
tissue mass narrow zone of transition and a fine
• May result in joint effusions or synovial sclerotic rim
inflammation • ‘Fallen fragment’ sign: a fractured
• Difficult to visualise on CT or MRI fragment that settles in the dependent
portion of the fluid-filled cyst

Chondroblastoma
Aneurysmal bone cyst • Eccentrically located epiphyseal lesion
• Primary or secondary to benign or • Occurs in patients < 30 years of age
malignant conditions, e.g. trauma, giant • Commonest site is the proximal humerus
cell tumour, osteosarcoma • Calcification occurs in 40–60% of cases
• Occurs in patients < 30 years of age • Can show surrounding oedema on MRI
• Males and females are equally affected
• Presents with local pain and swelling, Chondromyxoid fibroma
and with neurological signs if the spine is • Rare, benign cartilaginous tumour
involved • Most frequently seen in the second and
• Can occur at any site, but predominantly third decades
in the vertebrae (usually in the appendage) • Usually located eccentrically within the
and long bones (metaphyseal or metaphysis, commonly in the proximal
diaphyseal) third of the tibia
Imaging features • Thick sclerotic margin with mild cortical
expansion is typical
Plain X-ray • Malignant transformation is rare
• Classically an expansile, eccentric lesion in • High recurrence rate following curettage
the metaphysis of a child
• Matrix entirely lytic Imaging features
• Thin ‘egg-shell’ cortex • Eccentric, lobulated, lucent lesion
• Rapid growth involving the cortex and medulla
• No periosteal reaction, unless a fracture is • Characteristic sclerotic endosteal border
seen with scalloped margins
• No periosteal reaction
CT
• Soft tissue extension os uncommon
• Complex cystic lesion within the periosteal
• Calcification of the matrix is very rarely
membrane
seen on imaging
• Absence of matrix calcification
• MRI is non-specific, with high signal on
MRI T2WI
• Heterogeneous lesion, with blood
products

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Tumours 119

Malignant tumours of bone Chondrosarcoma


• Cartilage-producing sarcoma, often
Osteosarcoma appearing radiologically non-aggressive
• Commonest malignant primary bone • A lytic, destructive lesion with amorphous
tumour ‘snow-flake’ calcification in a patient > 40
• Occurs in patients < 30 years of age, years should prompt the diagnosis
although some present in older patients • Endosteal cortical scalloping of > 70%
with malignant degeneration of Paget’s of the cortex in a minimally aggressive
disease lesion should also suggest the diagnosis,
• Originates in the metaphysis and extends as should increasing pain and size of an
across the physeal plate to the epiphysis exostosis in an adult
• Central and parosteal are the two common
forms Chondrosarcoma versus
• Telangiectatic osteosarcoma is a rare type, enchondroma
which appears similar to an aneurismal
bone cyst on imaging
Points that favour chondrosarcoma:
• Parosteal osteosarcoma • Lesional pain
–– Median age is older than for the • Larger size
commoner central type • Axial location
–– Posterior aspect of the distal femur is a • Epiphyseal (particularly clear cell type)
common location • Oedema in adjacent marrow or soft
–– Wraps around underlying bone as it tissues
grows, and therefore prognosis is better • Any destructive change
than for the central type because it can • Significant depth and length of cortical
be relatively easily resected scalloping (involving more than two
Ewing’s sarcoma thirds of the cortex)
• Commonest primary malignant bone
tumour in the first decade of life
• 95% occur in patients aged 4–25 years Malignant fibrous histiocytoma
• Bone and lung metastases are common at
the time of presentation (and give a 5-year
(fibrosarcoma)
survival rate of 50%) • Peak age range is 30–60 years
• Most commonly arises as a primary lesion,
Imaging features but may be secondary to another lesion,
Plain X-ray e.g. Paget’s disease, radiation-induced
• Typically, permeative lesion in the changes
diaphysis of long bones; however, lesions • High-grade lesions carry a 5-year survival
may be metaphyseal or diametaphyseal rate of 25%
with a wide zone of transition Imaging features
• 75% of cases involve the pelvis or long
tubular bones; other sites include the MRI
shoulder girdle, rib and vertebral body • Usually detected on MRI
• ‘Onion-skin’ periosteal reaction is typical, • Isointense to muscle on T1WI, high signal
but ‘sunburst’ and amorphous forms can on T2WI
be present atypically • Occasionally shows dystrophic
calcification
MRI
• Low signal on T1WI, high signal on Bone lymphoma
T2WI, with a particularly large soft tissue • Primary lymphoma of the bone is almost
component, often with central necrosis exclusively non-Hodgkin’s lymphoma

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120 Musculoskeletal system and trauma

• Accounts for 1% of all cases of non-


Hodgkin’s lymphoma Hodgkin’s disease
• Average age at presentation is 24 years
• Males are more commonly affected than • Soft tissue disease may involve
females adjacent bones
• Affects the appendicular skeleton (most • Classic finding is the sclerotic ‘ivory’
commonly the femur, followed by the tibia vertebra
and then the humerus) • Direct invasion of the bone by local
• Bone involvement in non-Hodgkin’s lymph node disease is denoted by the
lymphoma is usually permeative osteolytic suffix ‘E’, added to the appropriate stage
(in 77% of cases); it is sclerotic in 4% of • Involvement of the bone marrow
cases indicates stage 4 disease: worse
prognosis than involvement of other
Diagnostic criteria for primary viscera and osseous bone
lymphoma of the bone

• Only a single bone involved


• Unequivocal histological evidence of Osteochondroma
lymphoma
• Other disease is limited to regional • Cartilage-capped exostosis
areas at the time of presentation • Generally accepted to be a developmental
• Primary tumour precedes metastasis by anomaly
at least 6 months • Childhood radiotherapy is an association
• Presents from 2–60 years (median age is 20
years)
• Males are 1.4 times more commonly
Secondary lymphoma affected than females
• More frequently seen in children • Occurs in long bones
• The axial skeleton is involved more • Metaphyseal in origin but migrates to the
commonly than the appendicular skeleton diaphysis as growth occurs (away from
• Secondary bony involvement is seen in joint, causing a ‘coat-hanger appearance’)
20% of patients with Hodgkin’s disease; • Typically occurs around the knee
primary Hodgkin’s disease of the bone is • Pedunculated and sessile forms are known
extremely rare • Diaphyseal aclasis: multiple cartilaginous
• Bone involvement in Hodgkin’s disease is exostoses constituting an autosomal-
sclerotic or mixed; Hodgkin’s disease can dominant disorder
cause ivory vertebral body • Malignant change in occurs in < 1% of
cases of osteochondroma
Imaging features • Malignant change in diaphyseal aclasis
MRI occurs in up to 5% of cases
• Modality of choice for staging and follow- Imaging features
up; MRI can upstage by nearly 30%
• Tumour infiltration of low signal on T1WI; MRI
high signal on STIR sequences • Cortical and medullary continuity between
the osteochondroma and parent bone
• Evaluates the hyaline cap (normally < 1 cm
in thickness)

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Tumours 121

• Variably sized erosions with sclerotic


Malignant transformation in margins may be seen
osteochondroma
CT
• Irregular and thickened synovium on
• Cartilage cap thickness is > 2 cm
arthrography
• Dispersed calcification in the cap
• Absence of calcification is typical
• Increased uptake on bone scan
• Clinical criteria: growth after MRI
maturation, pain, enlargement • Diffusely thickened synovium with low-
signal intensity on all sequences, owing
to the hemosiderin deposits, resulting
in a characteristic blooming artefact on
gradient-echo MRI
Synovial lesions • Focal mass typically adjacent to the patella
Bursitis in Hoffa’s fat pad (focal nodular synovitis
or focal pigmented villonodular synovitis)
• Benign uni- or multilocular soft tissue
masses Synovial chondromatosis
• Fluid-filled and lined by synovium • Synovial metaplasia of unknown aetiology
• Commonest sites are the subacromial with development of cartilaginous and
or subdeltoid, olecranon, iliopsoas, osteocartilaginous nodules within the
prepatellar and semimembranosus– metaplastic synovium
gastrocnemius bursas • Males are more commonly affected than
• Baker’s cyst involves the females
semimembranosus bursa • Typically occurs in the third, fourth of fifth,
• Note that ganglia, unlike bursas, are not but it is seen in all age groups
lined by synovium • Most commonly occurs in the knee,
Imaging features followed by the hip, shoulder and elbow
Ultrasound Imaging features
• Anechoic or hypoechoic Plain X-ray
CT • Multiple round bodies of similar size and
• Hypodense relative to muscle variable mineralisation within an effusion
• Mechanical erosions may be formed by
MRI
the bodies, and changes of secondary
• Hyperintense on T2WI and STIR
osteoarthritis may be seen in the long term
sequences (rim can enhance minimally
post-contrast); variable signal intensity on MRI
T1WI depending on protein content • Round bodies that conform to the signal
characteristics of bone or cartilage,
Pigmented villonodular depending on their constitution
synovitis
• Monoarticular synovial proliferative
Synovial sarcoma
disorder • Synovial sarcoma is a misnomer that arose
• Presents as a painless soft tissue mass because the tumour appears histologically
• Males and females are equally affected similar to synovium; however, this tumour
• Diffuse and focal nodular forms are known does not arise from the joint synovium
• Indolent expansile malignancy
Imaging features • Exact origin of the tumour is unknown but
Plain X-ray it arises from soft tissues in proximity to a
• Effusion is typical joint

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122 Musculoskeletal system and trauma

• Despite the misnomer, only 10% are intra- • Involves the adjacent bone in around 20%
articular of cases, with periosteal reaction
• Peak age range is 30–50 years
• Females are more commonly affected than
Imaging features
males MRI
• Commonly seen in the lower extremity • Poorly defined lesion
(especially the knee and foot) • Low on T1WI, but high if haemorrhage is
• Usually occurs within 5 cm of a joint present; heterogeneous on T2WI (cystic,
• Calcification is seen in 30% of cases, in solid or fibrous)
more peripheral part of lesion • Fluid–fluid levels can be seen

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2.7 Miscellaneous conditions

The prosthetic joint Achondroplasia


• The initial films serve as baseline study • Autosomal-dominant, rhizomelic, short-
and are used as reference films for follow- limb dwarfism
up imaging • Normal intelligence and life span

Dislocation Imaging features


• Most commonly occurs in the immediate Plain X-ray
postoperative period (occurs in 3% of • Lumbar kyphosis in infancy progressing to
patients) exaggerated lordosis in adulthood
• Can also occur as a late complication in • Bullet-shaped vertebral bodies in infancy,
prostheses that are not well positioned vertebra plana in adulthood
• Posterior vertebral scalloping
Loosening • Interpedicular distance progressively
• Radiographic manifestations decreases caudally
–– A lucent zone > 2 mm at the interface • Spinal and cranial stenoses are the major
(indicative of loosening) causes of morbidity
–– Component migration (diagnostic of • Spinal stenosis occurs secondary to
loosening): seen for example in the degenerative disease superimposed on
hip joint as interval tilting or cranial congenitally short pedicles
migration of the acetabular cup or as • Squared iliac wings in pelvis
subsidence (> 10 mm) and varus tilting • ‘Champagne glass’ inlet in the pelvis
of the femoral stem • Long bones are short and wide
• Enlarged skull with narrowed foramina
Particle disease (cement • Bone mineral density preserved
disease) Osteogenesis imperfecta
• Mostly seen in non-cemented hips as a
• Collagen disorder of the bone
reaction to small polyethylene particles in
• Blue sclerae (in 90% of cases),
the prosthesis
osteoporosis, scoliosis, grey teeth, hearing
• Granulomatous lesions seen as focal
loss (due to otic bone fractures)
radiolucencies of varying size around the
• Wormian bones are frequently associated
prosthesis
• Severe osteopenia and multiple fractures
• Tends to occur 1–5 years after surgery
with excessive callus
and is associated with smooth endosteal
• Non-accidental trauma is the main
cortical scalloping
differential diagnosis
• The key feature is that it produces
no secondary bone response (unlike
Types of osteogenesis imperfecta
infection)

Infection Type I: commonest form


• Ill-defined bone resorption Type II: most severe form, lethal in the
• Absence of a sclerotic margin neonatal period
• Ill-defined endosteal surface Type III: severely deforming, but
compatible with life
Fracture Type IV: mild form, no blue sclera
Frequency of fractures postoperatively
–– Cemented total hip arthroplasty: 0.4%
–– Revision hip arthroplasty: 7.2%

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124 Musculoskeletal system and trauma

Transient osteoporosis of the –– Caisson’s disease


–– Radiation
hip –– Systemic lupus erythematosus (which
• A form of regional osteoporosis can cause avascular necrosis in unusual
• Commonly seen in middle-aged men sites, such as the talus or humerus)
(bilaterally) and pregnant women (usually
in the left hip) Imaging features
• Presents clinically with a sudden onset of Plain X-ray
severe hip pain • Sclerosis, followed by subchondral fracture
• Self-limiting, and warrants no treatment; and flattening (‘snow cap’ sign)
however, it however recur in other joints • Cartilage remains intact until the onset of
• Pathological fracture may occur through secondary degenerative arthritis
the affected bone
• In comparison, regional migratory Radionuclide study
osteoporosis (another form of regional • Initially shows a photopenic region,
osteoporosis) affects multiple consecutive followed by increased activity with
joints revascularisation and repair
MRI
Imaging features • Initial oedema
Plain X-ray • Subsequently the ‘double-rim’ sign
• Severe osteopenia of the acetabulum and develops: distinct abnormality of the
femoral head with loss of subchondral marrow signal in the subchondral region,
cortex. The joint space is preserved with peripheral low-intensity rim on
MRI both T1WI and T2WI sequences and an
• Striking oedema in the proximal femur, adjacent inner rim of increased intensity
usually associated with a joint effusion on T2WI
• Low signal on T1WI, high signal on
T2WI and STIR sequences with contrast Avascular necrosis of the spine
enhancement throughout the femoral
head and neck • Air within the vertebral body is
• No subchondral sclerosis characteristic (MR signal void)
• MRI abnormalities subside in 6–10 months • ‘H’-shaped vertebra, due to collapse
of superior and inferior endplates
Musculoskeletal effects of (typically seen in sickle cell disease)
phenytoin
• Gum hypertrophy
• Osteomalacia
• Thickening of the skull vault Baker’s cyst
Avascular necrosis of the • Synovium-lined bursa that lies between
femoral head the medial head of the gastrocnemius
muscle and semimembranosus muscle
• Causes
• A contralateral, subclinical Baker’s cysts is
–– Trauma
a common finding
–– Steroids
• Affects 15% of the population
–– Alcoholism
• Commoner in males
–– Sickle cell disease
• Associated with osteoarthritis of the knee
–– Gaucher’s syndrome
joint

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Miscellaneous conditions 125

Imaging features bodies can lie within the cyst as a result of


underlying knee joint pathology
MRI
• Low signal on T1WI unless haemorrhagic, Ultrasound
high signal on T2WI • Anechoic with posterior enhancement
• May be septated, with complex features • Extension through the knee capsule is not
• Intra-articular bony or cartilaginous seen with an intact cyst

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2.8 Lists to remember

Widening of the femoral –– Idiopathic precocity


–– Adrenal and gonadal tumours
intercondylar notch –– Acrodysostosis
• Mnemonic: ‘BAIT’
–– Blood disorder, e.g. hemophilia Juxta-articular osteoporosis
–– Arthritis, e.g. gout, rheumatoid arthritis
and juvenile rheumatoid arthritis, • Causes
psoriasis –– Rheumatoid arthritis, juvenile
–– Infection, e.g. tuberculosis rheumatoid arthritis
–– Tumour, e.g. pigmented villonodular –– Tuberculosis or pyogenic infection
synovitis –– Reiter’s syndrome
–– Scleroderma
–– Haemophilia
Raised alkaline phosphatase
• Causes Fusion of the cervical spine
–– Paget’s disease
–– Osteomalacia and rickets • Mnemonic: ‘PIKRAD’
–– Renal osteodystrophy –– Post-trauma
–– Metastases –– Infection, e.g. tuberculosis
–– Primary bone tumour, e.g. sarcoma –– Klippel–Feil syndrome
–– Recent fracture –– Reiter’s syndrome, rheumatoid arthritis,
–– Puberty psoriasis
–– Ankylosing spondylitis
–– Diffuse idiopathic skeletal hyperostosis
Acro-osteolysis of (DISH)
the terminal phalanx
• Causes Protrusio acetabuli
–– Hyperparathyroidism • Abnormal medial position of the femoral
–– Psoriasis, rheumatoid arthritis, Reiter’s head compared with the pelvis
syndrome • Primary (otto pelvis) form is bilateral,
–– Raynaud’s disease, scleroderma familial and more common in women
–– Dermatomyositis • Causes
–– Diabetes mellitus –– Rheumatoid arthritis, osteoarthritis
–– Sarcoidosis –– Turner’s syndrome
–– Pyknodysostosis –– Pelvic trauma
–– Lesch–Nyhan syndrome –– Bone-softening conditions, e.g.
–– Leprosy Paget’s disease, fibrous dysplasia,
–– Polyvinyl chloride workers osteomalacia, renal osteodystrophy
–– Marfan’s syndrome
Accelerated bone
maturation Sclerotic pedicle
• Causes • Contralateral pedicle congenitally absent
–– Hyperthyroidism • Osteoid osteoma
–– Pseudohypoparathyroidism • Unilateral spondylolysis
–– Hypothalamic hamartoma • Sclerotic metastases
–– McCune–Albright syndrome

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Lists to remember 127

Vertebra plana –– Late rheumatoid arthritis


–– Gout, pseudogout
• Flattening of the vertebral body –– Acromegaly, ochronosis
• Causes –– Osteitis condensans ilii (seen in young,
–– Trauma multiparous women)
–– Tumour (myeloma, metastases, • Bilaterally asymmetric sacroiliitis
leukaemia) –– Early ankylosing spondylitis
–– Infection –– Early psoriatic arthropathy (50%)
–– Steroids –– Reiter’s syndrome
–– Haemangioma –– Juvenile rheumatoid arthritis
–– Eosinophilic granuloma • Unilateral sacroiliitis
–– Infections
Periarticular calcification –– Osteoarthritis

• Hypercalcaemic states
–– Sarcoidosis Erlenmeyer’s flask
–– Hypervitaminosis D deformity
–– Renal failure with secondary
• Condition where the distal end of long
hyperparathyroidism
bones is expanded
–– Tumoral calcinosis
• Most common bone to get affected is the
• Degenerative changes
femur
–– Calcium pyrophosphate dihydrate
• Results from a wide metaphysis secondary
deposition disease (CPPD)
to failure of osteoclastic function
–– Calcium hydroxyapatite deposition
• Causes
disease
–– Osteopetrosis
• Inflammatory conditions
–– Haemolytic anaemias, e.g.
–– Dermatomyositis
thalassaemia, sickle cell anaemia
–– Gout
–– Storage disorders, e.g. Gaucher’s
–– Bursitis
disease, Niemann-Pick disease
–– Scleroderma
–– Pyle’s disease (metaphyseal dysplasia)
• Neoplasms
–– Lead poisoning
–– Synovial chondromatosis
–– Rickets, hypophosphatasia
–– Synovial sarcoma
–– Fibrous dysplasia
–– Down’s syndrome
Sacroiliitis –– Achondroplasia
• Changes of sacroiliitis typically begin –– Leukaemia
in the lower and middle thirds of the
sacroiliac joint Acromioclavicular joint
• The iliac side of the joint is more severely
affected than the sacral side
injury grades
• Erosive changes initially lead to pseudo- • Grade I: acromioclavicular ligament sprain
widening of the joint space • Grade II: acromioclavicular ligament
• Bony ankylosis ensues from progressive rupture with coracoclavicular ligament
subchondral sclerosis intact; acromioclavicular joint space
• Eventually, the bones return to normal > 5mm, coracoclavicular < 11–13 mm
density • Grade III: acromioclavicular and
• Bilaterally symmetrical sacroiliitis coracoclavicular rupture
–– Ankylosing spondylitis • Grade IV: clavicle dislocated posteriorly:
–– Inflammatory bowel disease anteroposterior views may be normal; MRI
–– Late psoriatic arthropathy (in 50% of can assess for soft tissue pathology
cases)

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128 Musculoskeletal system and trauma

Diffusely increased bone –– Fibrous dysplasia


–– Neurofibromatosis
density –– Osteogenesis imperfecta
• Causes –– Congenital
–– Myelofibrosis
–– Mastocytosis Excessive callus
–– Metastatic disease
–– Sickle cell disease • Causes
–– Paget’s disease –– Steroids
–– Pyknodysostosis –– Neuropathic
–– Renal osteodystrophy –– Congenital insensitivity to pain
–– Osteopetrosis –– Paralysis
–– Fluorosis –– Osteogenesis imperfecta
–– Renal osteodystrophy
–– Burns
Dense metaphyseal bands –– Scurvy
• Causes
–– Normal finding Common sites of
–– Lead poisoning
–– Vitamin D excess
mandibular fractures
–– Stress lines
–– Post-treatment rickets Fracture type Prevalence
–– Scurvy Body 30–40%
Angle 25–31%
Bowed long bones in
Condyle 15–17%
children
Symphysis 7–15%
• Causes
–– Rickets Ramus 3–9%
–– Osteogenesis imperfecta
Alveolar 2–4%
–– Trauma
–– Madelung’s deformity Coronoid process 1–2%
–– Neurofibromatosis

Neurofibroma of bone
Superscan
• Scoliosis: short segment
• Prone to rapid progression • Metabolic disorders
• Enlarged neural foramina –– Renal osteodystrophy
• Posterior vertebral body scalloping –– Osteomalacia
• Dural ectasia –– Hyperparathyroidism
• Hypoplastic cranial bones –– Hyperthyroidism
• Anterior distal tibial bowing • Lesional causes
• Pseudoarthroses –– Diffuse metastases
• Multiple non-ossifying fibromas –– Myelofibrosis
• Ribbon ribs –– Aplastic anaemia
–– Leukaemia
–– Waldenström’s macroglobulinaemia
Pseudoarthrosis –– Systemic mastocytosis
• Causes –– Widespread Paget’s disease
–– Non-union of a fracture

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Lists to remember 129

No uptake of technetium- –– Sickle cell and thalassemia


–– Congenital syphilis
99m-methylene –– Osteopetrosis
diphosphonate –– Radiation
–– Acromegaly
• Causes
–– Paget’s disease
• Bone island
–– Gaucher’s disease
• Osteopoikilosis
• Non-ossifying fibroma, fibrous cortical
defect Wormian bones
• Osteopathia striata • Sutural bones
• Calcified gall stones • Causes
–– Cleidocranial dysostosis
Associations of slipped –– Pyknodysostosis
femoral epiphysis –– Osteogenesis imperfecta
–– Hypothyroidism
• Growth spur –– Down syndrome
• Perthes’ disease
• Renal osteodystrophy
• Rickets
Permeative pattern in bone
• Childhood irradiation • Causes
• Growth hormone therapy –– Lymphoma, leukaemia
• Trauma –– Multiple myeloma
• Malnutrition –– Ewing’s sarcoma
• Developmental dysplasia of the hip –– Neuroblastoma
• Endocrine disorders, e.g. hypothyroidism, –– Osteomyelitis
hypo-oestrogenic conditions, acromegaly,
gigantism, cryptorchidism, pituitary
adenoma, parathyroid adenoma
Lytic bone lesions in
children
Stippled epiphysis • Causes
–– Infection
• Causes –– Eosinophilic granuloma
–– Normal finding –– Leukaemia
–– Avascular necrosis –– Neuroblastoma metastases
–– Hypothyroidism –– Ewing’s sarcoma
–– Chondrodysplasia punctata
–– Multiple epiphysial dysplasia
–– Down’s syndrome Ivory vertebra
–– Hypoparathyroidism • Causes
–– Homocystinuria –– Paget’s disease
–– Zellweger’s syndrome –– Lymphoma
–– Haemangioma
‘Bone-within-bone’ –– Metastases
appearance
• Causes
MRI findings
–– Endosteal new bone formation Entities that shorten T1 (high
–– Normal finding in the thoracic and signal intensity on T1WI)
lumbar vertebra in infants
• Lipoma
–– Growth lines
• Haematoma
–– Caffey’s disease

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130 Musculoskeletal system and trauma

• Intralesional haemorrhage Low signal on T2WI


• Gadolinium
• Scar
• Fatty stroma of haemangioma, lipoma
• Dense mineralisation
• Protein
• Melanin
• Melanin
• Subacute hematoma
• Calcium in some cases
• Gas

MRI findings

Structure T1 signal T2 signal


Haematoma (acute) Intermediate to high High
Haematoma (subacute) Intermediate to high High
Haematoma (chronic) Low Low
Haemangioma Intermediate (higher than muscle) High
Lipoma High Intermediate
Protein High High
Fluid Low High
Air Low Low
Red marrow Low Intermediate
Hyaline cartilage Intermediate Intermediate
Cortical bone Low Low
Tendons Low Low
Ligaments Low Low
Fibrocartilage Low Low
Scar Low Low
Fat, fatty marrow High Intermediate
Muscle, nerves Low to intermediate Intermediate

Haematoma signal on MRI

Hyperacute Oxyhaemoglobin T1: iso T2: iso/bright


Acute Deoxyhaemoglobin T1: intermediate/low T2: dark
Subacute, early Intracell methaemoglobin T1: bright T2: dark
Subacute, late Intracell methaemoglobin T1: bright T2: bright
Chronic Haemosiderin T1: dark T2: dark

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Lists to remember 131

• Vascular flow void –– Haemopoitic marrow


• Foreign body –– Mastocytosis
• Hemosiderin –– Haemosiderosis
• Pigmented villonodular synovitis –– Myelofibrosis
• Giant cell tumor of the tendon sheath (a –– Osteopetrosis
form of pigmented villonodular synovitis) • Focal low signal
• Synovial pannus tissue –– Bone island
• Gouty tophus –– Sclerotic metastasis
• Amyloid –– Vacuum phenomenon

Signal and resolution on MRI Haematoma signal on MRI


• Good signal but poorer resolution • Remember that imaging features of
–– • Increased slice thickness soft tissue haematomas can be quite
–– • Increased field of view variable (unlike those of intracranial
–– • Decreased imaging matrix haemorrhages)
• Better resolution but worse signal • See table
–– • Decreased slice thickness
–– • Decreased field of view MRI appearances of
–– • Increased imaging matrix denervation
• Normal nerves appear slightly
Gadolinium hyperintense to muscle on T2WI
• Paramagnetic at room temperature • Acute (< 2 weeks since denervation):
• Atomic number 64 normal signal
• Electrons per shell: 2, 8, 18, 25, 9, 2; • 1–12 months since denervation: high on
however, paramagnetic gadolinium-3+ has T2WI, owing to extracellular intramuscular
seven unpaired electrons in its outer shell oedema
• Shows increased signal on T1WI • > 12 months since denervation: high on
• Adverse effects include nephrogenic T1WI, owing to fatty infiltration
systemic fibrosis and nephrogenic
dermopathy Osseous lesions containing
high signal on T1WI
STIR sequence
• Intraosseous lipoma
• Repetition time (TR) > 2000 milliseconds; • Bone infarct
echo time (TE) > 30 milliseconds • Hemangioma
• Fat-suppression technique • Paget’s disease
• Should not be used with gadolinium
• Good for imaging bone, cartilage and Osseous lesion containing low
muscle signal on T2WI
• Useful when there is significant artefact
• Sclerosis, calcification or matrix
from metal prosthesis
• Fibrous lesions
• Tissues with short T1 are bright
• Primary bone lymphoma
• Tissues with long T2 are bright

Low marrow signal


• Diffusely low signal
–– Bone marrow infiltration,
e.g. leukaemia, myeloma

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Chapter 3
Gastrointestinal
system

3.1 Hepatobiliary system


3.2 Gallbladder and biliary tree
3.3 Pancreas
3.4 Oesophagus
3.5 Stomach
3.6 Large and small bowel

Ch-03.indd 133 8/12/2010 12:18:26 PM


3.1 Hepatobiliary system

Liver anatomy • Middle hepatic vein divides the liver into


the right and left lobes
• The liver is a large gland situated in the • The portal vein divides the liver into an
right upper quadrant of the abdomen upper and lower portion
• The gland anatomy can be studied under • The segments are then numbered around
the three parts the inferior vena cava (IVC) from 1 to 8
–– Bile duct anatomy • The caudate lobe is segment 1
–– Functional segmental anatomy • Segments 4a and 4b are found between the
–– Vascular anatomy middle hepatic vein and the left hepatic
vein, above and below the portal vein
Bile duct anatomy
• The intrahepatic ducts follow the portal Vascular anatomy
venous and the hepatic arterial branches • The liver gets its blood supply from the
• The intrahepatic ducts join to form the hepatic artery and the portal vein
right hepatic duct and the left hepatic duct, • 80% of its supply is from the portal vein,
which then form the main intrahepatic with 20% from the hepatic artery
duct or common hepatic duct (CHD)
• The main intrahepatic duct is joined by the Hepatic artery
cystic duct to form the common bile duct • In 50–60% of people, the hepatic artery
(CBD) originates from the common hepatic
• The CBD then joins the pancreatic duct at artery, which is a branch of the celiac axis
the ampulla of Vater (the major duodenal • The common hepatic artery further divides
papilla) and drains into the duodenum into the gastroduodenal artery and the
• Normal diameter of the bile ducts hepatic artery proper
–– CBD: 6–7 mm; > 8 mm is dilated • Commonest variants
–– CHD at the porta hepatis: 5 mm –– The common hepatic artery divides into
the gastroduodenal artery and the right
Bile duct variants hepatic artery (in 10% of people)
• Variants found in 1–3% of all autopsies –– The common hepatic artery divides into
• Variants of important for postoperative the gastroduodenal artery and the left
bile leaks and obstruction hepatic artery (in 10% of people)
–– Aberrant intrahepatic dusts • The hepatic artery waveform is pulsatile
–– Cystic duct entering hepatic duct and low-resistance and shows a rapid
–– Duplication of cystic ducts systolic upstroke with a continuous
–– Ducts of Luschka (numerous ducts diastolic flow
draining directly into the cystic duct) • The normal velocity measures
–– Congenital tracheobiliary fistula 30–60 cm/second with a resistive
index of 0.6 ± 0.06
Cystic duct variants
• Account for 15–25% of bile duct variants Portal vein
• Commonest is the cystic duct inserting • Formed by the confluence of the splenic
into the middle third of the extrahepatic vein and the superior mesenteric vein
duct (75% of cases) • Cross-sectional diameter is approximately
• < 1% of cases show insertion into the right 12–14 mm
hepatic duct • Flow is non-pulsatile, in a hepatopetal
direction with mild respiratory variation
Functional segmental anatomy and a flow velocity of 20–30 cm/second
• Based on hepatic and portal venous
anatomy

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Hepatobiliary system 135

Hepatic veins Cirrhosis


• Waveform is triphasic, with both
• Chronic diffuse liver disease
respiratory and cardiac variation
• Aetiology is vast but most cases (75%) are
• Hepatic vein sampling should occur at its
due to alcoholic liver disease
confluence
• Causes
–– Toxins: alcohol, drugs such as
Hepatitis methotrexate, nitrofurantoin, isoniazid
• Inflammation of the liver resulting in –– Inflammatory: from hepatitis of various
deranged liver functions followed by causes, most commonly hepatitis B or C
imageable changes –– Biliary disease: inflammatory bowel
• Two main types: acute and chronic disease, cystic fibrosis
–– Nutritional: severe steatosis,
Acute hepatitis malnutrition
• Markedly raised AST and ALT with –– Hereditary disorders:
increased serum conjugated bilirubin haemochromatosis, Wilson’s disease,
• Clinically causes abdominal pain and alpha-1 antitrypsin deficiency
jaundice with either a normal or palpable –– Auto-immune disorders: primary
liver biliary cirrhosis, primary sclerosing
• Common causes cholangitis
–– Viral (hepatitis A) –– Idiopathic
–– Drug-induced • Three morphological types
–– Alcoholic use –– Micronodular cirrhosis
–– Alcoholic liver disease –– Macronodular cirrhosis
–– Fatty liver disease –– Mixed cirrhosis
–– Alcoholic hepatitis • Nodular lesions are of four main types
–– Alcoholic cirrhosis –– Regenerative nodules, consisting of
hepatocytes and stroma
Imaging features –– Cirrhotic nodules, which are
Ultrasound regenerative nodules with surrounding
• Appearance depends on the severity and fibrosis
stage of disease –– Dysplastic nodules, which are hepatic
• Typically shows as a diffuse decrease in adenomas with areas of dysplasia
liver echogenicity within then (a tissue diagnosis and
not seen on imaging); they are seen
Chronic hepatitis in cirrhosis from hepatitis B and
• Defined as hepatitis lasting at least 6 hepatitis C
months –– Hepatocellular carcinoma
• Common causes • Clinical presentation is usually with
–– Primary biliary cirrhosis anorexia, nausea and vomiting;
–– Hepatitis B, C and D with symptoms and signs of portal
–– Primary sclerosing cholangitis hypertension such as variceal bleeding,
–– Wilson’s disease ascites; or with symptoms and signs of
–– Alpha-1 antitrypsin deficiency hepatic encephalopathy such as confusion

Imaging features Imaging features


Ultrasound Ultrasound
• Increased liver echogenicity • Usually non-specific
• Coarse echotexture • Hepatomegaly and an irregular outline to
• Loss of definition of portal vessels the liver edge seen in early disease

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136 Gastrointestinal system

• A shrunken liver is seen in late stage characteristically there is enhancement in


• Diffuse increase in echogenicity with a the arterial phase with wash-out during
coarse echotexture the portal venous phase. A ‘nodule-in-
• Spares the caudate lobe, which may nodule’ appearance is characteristic on
become hypertrophic T2WI, with a neoplastic nodule that shows
• Regenerative nodules are not seen unless an increased T2 signal being seen within a
they are large dysplastic nodule that shows a decreased
• Decrease in definition of the portal vein T2 signal
wall
• Ancillary signs of liver failure include
ascites and portal hypertension with
Haemochromatosis
splenomegaly and varices • Abnormal deposition of iron in the liver
• Causes
CT
–– Primary, due to an autosomal-recessive
• Similar findings to ultrasound
disorder that causes an increased
• Characteristically there is a decrease
uptake of transferrin
in size of the right lobe and the
–– Secondary to iron deposition, which
medial segment of the left lobe, with
may be erythrogenic, due to Bantu
compensatory hypertrophy of the caudate
siderosis or transfusional (after > 30
lobe
units of blood)
• The regenerative nodules are seen if they
• Clinical findings
are siderotic, in which case they show
–– Hyperpigmentation
as high-attenuation nodules without
–– Arthralgias
enhancement during the arterial phase
–– Diabetes mellitus in 30–40% of cases
but as similar to surrounding liver in portal
–– Liver failure in long-standing cases
venous phase
• Main use is to identify complications Imaging features
including bacterial peritonitis
CT
• May also help in characterising focal
• Generalised increase in liver attenuation
lesions
• Portal vein branches seen as prominent,
• Nodular outline with hypertrophy of the
low-attenuation structures
caudate lobe and relative atrophy of the
quadrate lobe MRI
• Low-attenuation focal nodules are more • Reference tissue is skeletal muscle
suspicious of malignancy • Liver is low signal on both T1WI and T2WI,
and is best seen on gradient-echo T2WI,
MRI
which shows magnetic susceptibility
• Problem-solving tool to characterise focal
artefacts
lesions
• Pancreas also shows low signal late in the
• Benign regenerative nodules show an
disease
increased signal on T1-weighted images
• Spleen is normal in primary disease but
(T1WI) and a decreased signal on T2-
low signal in secondary disease
weighted images (T2WI) as a result of
siderosis
• Dysplastic nodules show a homogeneously Liver abscess
increased signal on T1WI and a very • Localised collections of pus within the
much decreased signal on T2WI, with liver
enhancement with gadolinium (similar to • Most commonly bacterial in aetiology (in
findings with hepatocellular carcinoma) 80–90% of cases)
• Neoplastic nodules in cirrhosis • Fungal abscesses account for < 10% of
show variable appearances, but cases, amoebic abscesses for 5% of cases

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Hepatobiliary system 137

Pyogenic liver abscess • Peripheral wall enhancement on contrast


• Commonly polymicrobial, although studies
Escherichia coli and streptococci account • Simple or multiloculated with nodularity
for the majority of organisms grown of the wall
• Aetiology varies: biliary spread is the • Pyogenic abscesses contain gas whereas
commonest (20–30%); others include amoebic abscesses do not
contiguous spread, trauma, portal venous • Two signs specific to pyogenic abscesses
• Most commonly seen in those aged 60–70 –– Double target sign: wall enhances, with
years a surrounding hypodense zone caused
• Right lobe is the commonest location (in by oedematous liver tissue
> 50% of cases) –– Cluster sign: several abscesses cluster
• Multiple abscesses are more common that together, looking as if they might
a single abscess coalesce
• Central lesions are common MRI
• Low signal on T1WI, high signal on T2WI
Amoebic liver abscess
• Enhancement of wall on gadolinium
• Entamoeba histolytica is the causative
• Target sign on T2WI: cavity shows a high
organism
T2 signal with the wall showing a low T2
• Most commonly seen in those aged 30–50
wall and peri-lesional oedema producing a
years
high T2 signal
• Usually located in the right lobe of the liver
and in the periphery Nuclear medicine imaging
• Typically a single lesion • Uptake of the sulphur colloid of
• Size varies; often the abscess is > 5 cm in technetium-99m and of labelled white
diameter blood cells
• The sulphur colloid is taken up by
Imaging features abscesses, giving rise to a focal area on
Plain X-ray imaging
• Raised right dome of the diaphragm • Gallium is taken up by pyogenic abscesses
• Right basal atelectasis but not by amoebic abscesses
• Right pleural effusions
Ultrasound Hepatic haemangioma
• In early stages the abscess appears as a • Commonest benign liver lesions,
hypo-echoic, ill-defined area accounting for 70–80% of liver lesions
• Later it becomes better defined, with • Usually single (in 90% of cases) and
posterior enhancement and a thick peripheral
reflective wall • Associated with focal nodular hyperplasia
• Simple or multi-loculated and Osler–Weber–Rendu syndrome
• Single or multiple • May present with spontaneous
• Central (pyogenic) or peripheral haemorrhage or with Kasabach–
(amoebic) Merritt syndrome (haemangioma plus
• Depending on the type, the abscess thrombocytopenia)
contents may be homogeneous (in • Usually < 4 cm in diameter; produces a
amoebic and pyogenic abscesses) or giant cavernous haemangioma when > 4
more heterogeneous, with low level foci cm
of echogenicity (in pyogenic or fungal • Blood supply comes from the hepatic
abscesses) artery
CT • May rupture spontaneously
• Low-attenuation lesion causing mass
effect

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138 Gastrointestinal system

Imaging features • Hypoechoic central scar seen in 15–20% of


cases
Plain X-ray
• Lesion displaces vessels around it
• Calcifications within the liver
• Colour Doppler imaging shows enlarged
Ultrasound vessels within the lesion, with a central
• Variable features artery and large peripheral draining veins
• Majority are hyperechoic although can be
CT
hypoechoic
• Ill-defined, low-attenuation mass
• May show acoustic enhancement
• Transient hyperintensity in the portal
• No flow on colour Doppler
venous phase of contrast injection that
CT then leaches away to isointensity to the
• Well-circumscribed, predominantly low- surrounding tissue in the equilibrium
attenuation lesion phase
• On contrast enhancement there is a • ‘Spoke-wheel’ pattern with a central scar is
peripheral nodular enhancement that fills seen in 20–30% of cases
in over time as seen on delayed images • Central scar is seen in delayed images
• Small lesions, though they enhance
MRI
immediately
• On T1WI the lesion is isointense, with a
MRI well-defined hypointense central scar
• Sharply defined lesion • On T2WI the lesion is usually isointense,
• Low signal on T1WI and markedly with a hyperintense central scar in 75% of
hyperintense on T2WI cases
• Central fibrous scar present • On gadolinium-enhanced dynamic
• Similar enhancement pattern to that imaging, there is intense enhancement
seen on CT, with peripheral nodular in the arterial phase that progressively
enhancement that progresses centripetally becomes less intense
with central uniform enhancement taking • The central scar enhances early and is a
approximately 15 minutes characteristic finding
• Kupffer cell-specific agents, such as
Focal nodular hyperplasia superoxides, cause enhancement of the
lesion
• Second commonest benign liver lesion
• Peak age is 30–50 years, with a female Differential diagnoses
predominance
• Associated with hepatic haemangiomas
• Fibrolamellar hepatocellular carcinoma
• More commonly located in the right lobe
(HCC)
• Solitary lesion < 5 cm in diameter
• Hepatic adenoma
• Tumour-like lesion that is seen on the
• Well-differentiated HCC
surface of the liver
• Giant cavernous haemangioma
• Non-encapsulated, sometimes
• Hypervascular metastasis
pedunculated
• Intrahepatic cholangiocarcinoma
• Central scar that contains an arteriovenous
malformation
• Very vascular and contains Kupffer cells
• No calcification within the lesion
Hepatocellular adenoma
Imaging features
• Seen in women on oral contraceptives
Ultrasound • Peak age is 20–40 years
• Hyperechoic lesion showing as a • Men on anabolic steroids may develop it
homogeneous mass

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Hepatobiliary system 139

• Most commonly seen in the right lobe in a • Causes


subcapsular location –– Cirrhosis from hepatitis B or C (40–50%
• Typically 5–10 cm in diameter of cases)
• Adenomas consist of hepatocytes and –– Alcohol cirrhosis (< 10% of cases)
Kupffer cells with fat but no portal –– Vascular neoplasm
tracts within it. The lesion has areas of • Solitary in 30% of cases, multifocal in
haemorrhage and necrosis within it 60–70% and diffuse in 5%
• Invades the portal vein in 50–60% of cases;
Imaging features invades the hepatic vein or the IVC in
Ultrasound < 20%
• Heterogeneous solid lesion • Also invades the bile ducts
• Variable echotexture depending on • Metastasis are seen in 70% of cases
constituent elements • Commonest lymphatic site for metastases
• Hypoechoic rim due to compression of is the hepatoduodenal nodes
liver tissue around the tumour • Commonest sites for haematogenous
CT spread are the lungs, adrenal glands and
• Well-defined rounded lesion bone
• May have hyperdense areas if there has • Pathogenesis follows a stepwise process:
been haemorrhage within the tumour regenerative nodules progress to an
adenomatous nodule, which becomes a
MRI dysplastic nodule, then a small
• The appearances cannot be reliably HCC (< 2 cm in diameter) and then
distinguished from those of HCC a large HCC
• Areas of haemorrhage of differing ages
results in a lesion that is heterogenous in Imaging features
appearance on various sequences Ultrasound
• Predominantly hyperintense on T1WI • Variable echogenicity
• Isointense on T2WI • Background of cirrhosis
Nuclear medicine imaging • Invasion to hepatic or portal veins seen by
• Photopenic lesion on sulphur colloid colour flow
scintigraphy, surrounded by a rim of CT
increased uptake • Best seen with dynamic CT
• No uptake on gallium scan • Heterogenous enhancing mass with focal
necrosis
Hepatocellular carcinoma • Calcification is seen in 10% of cases
• Invasion is best demonstrated with CT
(HCC) • Features of portal venous invasion are
• Also known as a hepatoma arterial–portal fistulae, periportal streaks
• Commonest visceral malignancy of high attenuation, and dilatation of
• Peak age is 60–70 years; when seen in portal vein and branches
children it is usually in those > 10 years old • No enhancement of the segment of the
liver that is blocked
Aetiology of HCC • Intrinsic portal vein obstruction is specific
to HCC
W Wilson’s disease • Portal vein thrombosis is differentiated
H Haemochromatosis from HCC by the lack of enhancement of
A Alpha 1 antitrypsin deficiency the thrombus
T Tyrosinosis MRI
H Hepatitis • Focal liver lesions with high signal on
C Cirrhosis T2WI and variable intensity on T1WI
C Carcinogens –– Low T1 signal in 30–40% of cases

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140 Gastrointestinal system

–– High T1 signal in 40–50% of cases CT


–– Isointense T1 signal in 10–20% of cases • Lobulated mass
• High-grade dysplasia and small HCCs • Enhancing mass with central scar that
show a “nodule-in-nodule appearance does not enhance
especially within siderotic nodules • Pseudocapsule
• At dynamic gadolinium imaging intense
MRI
enhancement is seen
• Hypointense on T1WI
• Characteristic features of large HCC
• Hyperintense on T2WI
–– Mosaic pattern: confluent small
• Scar is typically hypointense on both T1WI
nodules separated by thin septae,
and T2WI
best seen on T2WI and giving
• Heterogenous gadolinium enhancement
a heterogenous appearance on
of the lesion but no enhancement of the
gadolinium-enhanced images
scar
–– Tumour capsule is present in 60–80% of
• Not typically known to cause vascular
cases and is hypointense on both T1WI
invasion
and T2WI
–– Extracapsular extension with satellite
nodules is seen in 50–70% of cases Fatty liver disease
–– Vascular invasion (as seen on CT), • Complication of many toxic, ischaemic
seen on MRI as the presence of high and metabolic insults to the liver
signal on T1WI and T1 gradient-echo • May be diffuse or focal
images within the portal vein, and • May be lobar, segmental or nodular
enhancement of the tumour thrombus
in the arterial phase and a filling Imaging features
defect in later phases on gadolinium- Ultrasound
enhanced imaging • Hyperechoic
Nuclear medicine imaging • Impaired visualisation of hepatic vessels
• Sulphur colloid scan shows a cold spot • If focal then the margins are poorly
• Avid uptake on gallium scan visualised
CT
Fibrolamellar HCC • Areas of low attenuation with average of 10
Hounsfield units
• Special type of HCC • Liver spleen density is reversed
• Peak age is 30–40 years • Hyperdense intrahepatic vessels
• No underlying cirrhosis or other risk • Focal fatty infiltration has no mass effect
factors for HCC and the vessels course normally through
• Solitary lobulated lesion with a central scar the area of infiltration
• Encapsulated with a pseudocapsule of
compressed hepatic tissue MRI
• Central amorphous calcification seen in • High signal on T1WI
30–40% of cases • Low signal on T2WI
• Regional lymphadenopathy seen in 50– • Conventional imaging insensitive for fatty
60%; commonest site is the porta hepatis infiltration
• Distant metastases are uncommon • Opposed-phase imaging turns the fat-
• Good prognosis infiltrated areas dark on out-of-phase
imaging
Imaging features
Ultrasound Metastases to the liver
• Heterogenous, predominantly hyperechoic
central scar • One of the commonest organs for
metastases

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Hepatobiliary system 141

• Commonest malignant lesion of the liver, • Causes of haemorrhagic metastases


usually arising from primary in the colon, –– Colon carcinoma
breast, lung or pancreas –– Thyroid carcinoma
• Both lobes are involved in 75% of cases –– Breast carcinoma
• Lesions are solitary in 10% of cases –– Choriocarcinoma
–– Melanoma
Imaging features
Ultrasound Budd–Chiari syndrome
• Focal metastases are space-occupying
lesions that distort the liver anatomy • Occlusion of the venous outflow of the
• Variable echogenicity liver: occlusion of the hepatic vein or the
• Echogenic liver lesions are usually from IVC
the gastrointestinal tract or the urogenital • Presents clinically with hepatomegaly and
tract ascites
• No Doppler signal is generated, the • Aetiology is diverse, but most often
exception being carcinoid tumours idiopathic (in 60–70% of cases)
CT Thrombotic obstruction
• Attenuation is variable but most are (Virchow’s triad)
predominantly low-attenuation lesions
• The three features of Virchow’s triad
• Peripheral ring enhancement occurs
–– Blood flow problems
frequently
–– Problems of the constituents
• Cystic metastases can be differentiated
–– Problems with the walls
from benign cysts by mural nodule, fluid–
• Causes are the ‘five Ps’
fluid levels or septations
–– Pregnancy
• Most sensitive imaging modality is CT
–– Pill
angiography, which is used to assess
–– Polycythemia rubra vera
potentially resectable metastases
–– Platelets – abnormal/excess
–– Paroxysmal nocturnal haemoglobinuria
Exclusion criteria for
metastatectomy Non-thrombotic obstruction
• Causes
• Advanced stage of primary tumour –– Right heart failure
• More than four metastases –– Constrictive pericarditis
• Extrahepatic metastases –– Right atrial tumour
• < 30% of liver tissue left after resection –– IVC membranes
Imaging features
• Causes of calcified liver metastases Ultrasound
–– Gastric carcinoma • Hepatomegaly
–– Malignant melanoma • Caudate lobe hypertrophy
–– Renal cell carcinoma • Gallbladder wall thickening
–– Colon carcinoma • Obliteration of the hepatic veins and/or
–– Ovarian carcinoma the IVC
• Causes of hypervascular metastases • Portal vein enlargement and change in
–– Thyroid carcinoma flow dynamics
–– Renal cell carcinoma • Hepatopetal or hepatofugal flow may be
–– Carcinoid seen in the portal vein
–– Melanoma • Portosystemic anastomosis and the
–– Pancreatic islet cell tumours azygos and hemiazygos systems become
prominent

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142 Gastrointestinal system

• Doppler studies show absence of flow or • Occurs in 30% of cases of HCC and in
reversal of flow within the hepatic veins, 5–10% of cases of cirrhosis
with dampening of the IVC waveform • Can be acute or chronic
• Hepatic artery resistance index > 0.7 • Clinical manifestations include
CT haematemesis, hepatic encephalopathy
• Hepatomegaly and ascites and abdominal pain
• Non-homogeneous liver enhancement
with a predominantly central area of Causes of portal vein thrombosis
enhancement and delayed enhancement
of the periphery • Idiopathic in neonates
• The periphery may show low attenuation • Malignancy (HCC, cholangiocarcinoma,
• The caudate lobe is enlarged and enhances pancreatic carcinoma, liver metastases)
floridly • Hypercoagulable states
• Poor identification of the hepatic veins • Trauma, especially iatrogenic by
• Identification of the cause of obstruction introduction of umbilical vein catheter
may be possible • Intraperitoneal inflammation
• Thrombosis in the IVC and hepatic veins • Umbilical sepsis
may be difficult to see • Pancreatitis
• Peritonitis
MRI
• Ascending cholangitis
• Peripheral liver gives low signal on T1WI
and high signal on T2WI
• Liver parenchyma is not homogeneous
• Gadolinium enhancement is variable Imaging features
• Comma-shaped intrahepatic varices, Ultrasound
caused by intrahepatic collateral vessels, • Echogenic material within the portal vein
are characteristically seen on coronal in the acute phase that becomes less well
images visualised with age
• Reduction in calibre or absence of the • Increased portal vein diameter
hepatic veins and IVC • Thickened lesser omentum in the acute
phase
Major differential diagnoses
• Cirrhosis CT
• Hepatic veno-occlusive disease • There is low attenuation thrombus within
• Right heart failure the portal vein on portal venous phase
• Decrease in attenuation of the liver
Hepatic veno-occlusive disease • In chronic stages multiple peripheral
• Commonly caused by radiation or collaterals are seen
chemotherapy in transplant patients MRI
• Demonstrated on MRI by occlusion of the • Gradient-echo imaging shows flow voids
small centrilobular venules without major in the portal vein
hepatic vein thrombosis • Depending on the age of the thrombus,
• Ultrasound shows bidirectional flow in the signal changes differ, with high T1 and T2
portal veins without evidence of hepatic signal within the first 5 weeks and high T2
vein or IVC obstruction, with gallbladder signal after that
wall thickening
Peliosis hepatis
Portal vein thrombosis
• Rare benign disorder characterised by
• Thrombosis of the portal vein is a a dilatation of sinusoidal blood-filled spaces
causative factor for the development of and of blood-filled spaces within the
portal hypertension in children organs of the reticuloendothelial system

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Hepatobiliary system 143

• Affects the liver, spleen, bone marrow, Imaging features


lymph nodes and lungs
Ultrasound
• Causes
• Multiple echogenic or hypoechoic areas
–– AIDS
–– Chronic infections, e.g. tuberculosis CT
–– Anabolic steroids • Enhancing, round lesions within the
–– Chronic renal failure organs
–– Tamoxifen • The lesions are initially hypoattenuating;
they become isoattenuating with time

Ch-03.indd 143 8/12/2010 12:18:27 PM


Gallbladder and
3.2 biliary tree

Anatomy Nuclear medicine imaging


• Delayed filling of the choledochal cyst on
• The biliary tree transports bile from the hepatobiliary imino-diacetic acid (HIDA)
liver to the second part of the duodenum scans
• Bile duct variants are common, the most • Differential diagnoses include hepatic
important being the aberrant cystic duct cysts, pancreatic pseudocysts and enteric
• Variations of the cystic duct occur in < 25% duplication cysts
of cases
• 75% of cystic ducts insert into the middle
third of the extrahepatic CHD Cholelithiasis and
• 10% insert into the distal CHD cholangiolithiasis
• < 1% have a gallbladder draining directly
• Prevalence increases with age
into the CBD
• Causes
–– Haemolytic disease
Choledochal cysts –– Cholestasis
–– Metabolic disorders
• One of a spectrum of cystic dilatations of
–– Malabsorption
the biliary system, characterised by cystic
–– Increased incidence in diabetics
dilatation of the extrahepatic bile ducts
• Cholangiolithiasis is the commonest cause
• Classification is based on the extent and
of bile duct obstruction
morphology of the choledochal cysts
• Usually presents before the age of 10 years, Main types
with 30–40% presenting before the age of 1
• Cholesterol stones
year
–– Account for 70–80% of stones
• Associated with anomalies of the biliary
–– Radiolucent
and pancreatic ductal systems and with
–– 70% are mixed and 20% are radiopaque
liver cysts
–– Strong association with cirrhosis
• Clinical manifestations (triad seen in 25%
• Pigment stones
of cases)
–– Account for 30% of stones
–– Intermittent obstructive jaundice
–– 2–5 mm in diameter and faceted
–– Recurrent right upper quadrant pain
–– Predominantly contain calcium
–– Right upper quadrant mass
bilirubinate
Imaging features –– 50% are radiopaque
Ultrasound Imaging features
• Cyst adjacent to gallbladder
Ultrasound
• Intrahepatic bile duct dilatation, caused by
• Specific and sensitive for gallstones
pressure on the CHD
• Echogenic
• May be seen in antenatal scans
• Mobility needs to be assessed on
MRI ultrasound
• MR cholangiopancreatography (MRCP) • Wall–echo shadow complex is seen when
confirms the presence of the cyst and the gallbladder is contracted over the
identifies the anomalous anatomy gallstones

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Gallbladder and biliary tree 145

CT • Gallbladder is contracted and may be


• Hyperdense stones can be seen on CT calcified (‘porcelain’ gallbladder)
• Inverse relationship between the • Hepatobiliary imino-diacetic acid (HIDA)
cholesterol in the stones and degree of scanning is useful for looking at biliary
attenuation obstruction
MRI Acalculous cholecystitis
• Less sensitive for visualising stones than
• Caused by gallbladder dysmotility and
CT
super-added bacterial infection
• However, MRCP is often done to provide a
• Seen in burns, serious injury and extrinsic
further assessment of the anatomy and to
inflammation
visualise the stones better
• Similar ultrasound appearances to those
• Stones are seen as signal voids or filling
of calculous cholecystitis, but without the
defects within the biliary tree
gallstones
• Reduced motility with cholecystokinin
Acute calculous cholecystitis • Complications include gangrene of the
• Inflammation of the gallbladder gallbladder and perforation
• Peak age is 50–70 years Emphysematous cholecystitis
• Complications
• Ischaemia of gallbladder wall secondary to
–– Gangrene
inflammation and vasculitis
–– Abscess
• Typically seen in diabetes
–– Emphysematous cholecystitis
• Plain radiographs show gas around the
–– Bouveret syndrome (erosion of
gallbladder
gallstones into the duodenum)
• Pneumobilia
–– Empyema
–– Gallstone ileus Imaging features
Imaging features Plain X-ray
• Gas around the gallbladder
Ultrasound
• Pneumobilia
• Echogenic
• May be mobile Ultrasound
• Gallstones may be impacted in the neck • Gas obscures the wall of the gallbladder
• Sludge within the gallbladder • Pericholecystic fluid
• Gallbladder wall thickening > 3 mm • Pneumobilia
• Pericholecystic fluid
• Positive sonographic Murphy’s sign Xanthogranulomatous
CT
cholecystitis
• Distended gallbladder • Inflammation of the gallbladder with
• Multiple gallstones intramural nodules
• Pericholecystic fat stranding • Peak age is 70–80 years
• Increased attenuation of bile • Thick gallbladder wall and intramural
hypoechoic nodules
MRI • Focal fatty inflammation
• MRCP shows gallstones within the biliary • Biliary obstruction
tree
• Dilatation of the CBD
• Intrahepatic duct dilatation if there is Cholangiocarcinoma
proximal stone or if Mirizzi’s syndrome is • Associated with primary sclerosing
present cholangitis
• Most commonly located in the hilum or in
Chronic cholecystitis the distal CBD (in 30–40% of cases)
• Commonest gallbladder disease

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146 Gastrointestinal system

• Predisposing factors Obstructive cholangitis


–– Inflammatory bowel disease
• Caused by benign obstruction by surgery
–– Primary sclerosing cholangitis
or calculi in 60% of cases
–– Cholelithiasis
• Malignant obstruction may be due to an
–– Choledochal cysts
ampullary tumour
–– Caroli’s disease
• Acutely non-suppurative in 10–20% of
–– Biliary atresia
cases; suppurative in 80–90% cases
–– Adult polycystic kidney disease
• Escherichia coli is the commonest
–– Congenital hepatic fibrosis
causative organism
–– Papillomatosis of the gallbladder
Imaging features
Intrahepatic
cholangiocarcinoma Ultrasound
• Shows the cause of the obstruction
• Peak age is 50-60 years • May show pneumobilia
• Mass with satellite nodules
• Dilated biliary tree with a predominantly CT
hyperechoic mass (in 75% of cases) • Peri-biliary enhancement on arterial
• Shows homogeneous delayed phase imaging
enhancement on CT, with dilated
intrahepatic ducts
Primary sclerosing cholangitis
• A Klatskin’s tumour is located at the • Chronic obliterative fibrotic inflammation
confluence of the right and left hepatic of the biliary tree
ducts; indirect signs are segmental • Intrahepatic bile ducts are more often
dilatation of the main ducts, portal vein involved than extrahepatic bile ducts
obstruction and lobar atrophy • Associations
–– IBD
Extrahepatic –– Pancreatitis
cholangiocarcinoma –– Sjögren’s
–– Retroperitoneal fibrosis
• Peak age is 60–70 years
–– Peyronie’s disease
• 10% present with cholangitis
• CBD is always involved
• Pure intrahepatic duct dilatation
• Mass seen in the extrahepatic biliary tree Imaging features
• Usually an infiltrating lesion of low
Ultrasound
attenuation that shows delayed
• Echogenic portal triads
enhancement
CT
• Pruning of the biliary tree and beading of
Mirizzi’s syndrome the ducts, with wall nodularity
• Impaction of the gallstone in the neck of Cholangiogram
the gallbladder • Characteristic beading of the ducts with
• Obstructs the cystic duct pruned appearance
• Gives rise to cholecystitis
MRI
• MRCP shows pruning of the biliary tree
Cholangitis and similar pruning appearance
• Inflammation of the bile ducts
• Most commonly due to obstruction

Ch-03.indd 146 8/12/2010 12:18:28 PM


3.3 Pancreas

Anatomy the body and tail of the pancreas through


the minor papilla
• Retroperitoneal structure within the
• The ventral duct (Wirsung’s duct) drains
anterior pararenal space at the T12–L1
the uncinate process through the major
level
papilla
• Four parts are described: the head, the
• The dorsal and ventral ducts fuse so that
body, the tail and the uncinate process
the main duct of Wirsung consists of the
• Central pancreatic duct feeds into the
ventral duct and proximal segment of the
ampulla of Vater, together with the CBD
dorsal duct; it drains into the major papilla
• They enter via the major duodenal papilla
• The distal dorsal duct regresses and drains
into the second part of the duodenum
as Santorini’s duct through the minor
• Accessory pancreatic duct entering into
papilla
the minor duodenal papilla
• Relations
–– Retroperitoneal, lying in the pararenal Pancreatic anomalies
space along with the retroperitoneal
colon and duodenum Frequency of anomalies
–– Lies anterior to the IVC, the aorta and
the first part of the duodenum at the • Dual drainage (accessory papillae) (30%
level of the superior mesenteric artery of cases)
–– Lies anterior to the confluence of the • Pancreas divisum (< 10% of cases)
superior mesenteric vein and the • Ectopic pancreatic tissue (< 10% of
splenic vein cases)
–– Lies posterior to the lesser sac and the • Annular pancreas (one in 7000 cases)
mesentery of the transverse colon
–– To the left the tail is related to the hilum
of the spleen
–– To the right the tail is related to the Pancreas divisum
second part of the duodenum • Formed by failure of the dorsal and ventral
–– Blood supply is via the superior buds to fuse
pancreaticoduodenal artery (a branch • The dorsal and ventral ducts drain
of the gastroepiploic artery) and the separately
inferior pancreaticoduodenal artery • The dorsal duct drains the body, tail and
(a branch of the superior mesenteric superior part of the head of the pancreas
artery) through the minor duodenal papilla
• The ventral duct drains the uncinate
Embryology process and the inferior part of the head
through the major papilla
• Formed by the fusion of the dorsal and • Presents with non-alcoholic recurrent
ventral buds of the foregut in the fourth pancreatitis in the dorsal segment, caused
week of gestation by stenosis of the minor papilla
• The dorsal duct (Santorini’s duct) drains

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148 Gastrointestinal system

Imaging features –– Hyperlipidaemia types I and V


–– Kwashiorkor
ERCP
–– Infection or infestation
• Difficult cannulation of the minor papilla
–– Trauma (iatrogenic in a Billroth
• Obvious absence of connection between
II gastrectomy, a splenectomy or
the dorsal and ventral ducts
choledochal surgery)
CT –– Structural abnormalities
• May show increase in the craniocaudal –– Diuretics (thiazides and frusemide)
length and a fat plane between the dorsal –– Anticancer drugs (azidothymidine,
and ventral elements asparaginase)
MRCP –– Antibiotics (sulphonamides,
• Similar findings to those of ERCP tetracycline)
–– Antidiabetic drugs (phenformin)
Annular pancreas –– Malignancy (metastasis and
• A rotational anomaly lymphoma)
• Located in the second part of the
duodenum Acute pancreatitis
• May cause obstruction in neonates or be
asymptomatic • May be diffuse (50% of cases) or focal (50%
of cases)
Imaging features • Commonly caused by gallstones
Plain X-ray • Two main pathological forms: oedematous
• ‘Double bubble’ sign in neonates, caused and necrotising
by duodenal obstruction • Haemorrhagic pancreatitis is characterised
• Upper gastrointestinal (GI) series shows by fat necrosis and haemorrhage
eccentric or concentric narrowing of the • Suppurative pancreatitis is caused by
duodenum with delayed gastric emptying; super-added bacterial infection
the duodenal mucosa is flattened, with no • Presents with abdominal pain radiating
ulceration to the back and raised levels of serum
amylase, lipase and calcium
CT • Signs of haemorrhagic pancreatitis
• Mass around the duodenum –– Cullen’s sign: periumbilical ecchymoses
Endoscopic retrograde –– Grey–Turner sign: ecchymoses on the
cholangiopancreatogram (ERCP) flanks
• Ventral duct wrapped around the second –– Fox sign: infrainguinal ecchymoses
part of the duodenum
Imaging features
AXR
Pancreatitis • Generalised or focal ileus (sentinel loop)
• Commonest pathology in the pancreas • Renal halo, caused by perirenal oedema
• Causes • Gas in the pancreatic region
–– Idiopathic • Ascites
–– Alcohol (15% of cases) CXR
–– Cholelithiasis (75% of cases), mainly • Basal subsegmental atelectasis
acute • Pleural effusions
–– Metabolic disorders • Elevation of the left hemidiaphragm
–– Hypercalcaemia in • Adult respiratory distress syndrome
hyperparathyroidism • Pulmonary oedema
–– Hereditary pancreatitis • Pulmonary infarction or infiltrates

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Pancreas 149

• Necrotising pancreatitis
Abdominal causes of pleural –– Focal or diffuse areas of necrosis of
effusions pancreatic parenchyma
–– Associated peripancreatic fat necrosis
Transudative pleural effusions –– Contrast-enhanced CT shows patchy
• Cirrhosis with ascites (usually right- enhancement of the pancreas
sided effusion) –– Morbidity and mortality is high, and
• Peritoneal dialysis it carries a poor prognosis and is an
indication for surgery
Exudative pleural effusions –– Complications include pancreatic
• Pancreatitis, pancreatic psuedocyst, abscess, pseudocyst, fistulae, pseudo-
pancreatic fistula (usually left-sided aneurysms and exocrine or endocrine
effusion) deficiency
• Boerhaave’s syndrome (usually left- –– Pancreatic abscess needs to be
sided effusion) differentiated from necrosis: a well-
• Subphrenic abscess (usually right-sided marginated fluid collection is seen in an
effusion) abscess but not in necrosis
• Abdominal malignancy (bilateral –– Rate of infection with necrosis is high,
effusions) and infection cannot be detected
• Meigs’s syndrome (bilateral effusions) accurately on CT
• Endometriosis • Acute fluid collections
• Biliary fistulae –– Caused by the mesentery originating
from the pancreas, with spread of fluid
and subsequent ileus in the hepatic
Imaging features flexure, transverse colon and splenic
flexure
Upper GI contrast study –– Fluid is seen to accumulate in the lesser
• Non-specific findings sac first followed by anterior perirenal
• Widening of duodenal sweep space
• Thickened duodenal folds –– The fluid then may spread cephalad to
• ‘Reverse three’ sign (tethering of folds) the bare area or caudad to the pelvis via
• Enlarged major papilla the psoas muscle
• Anterior displacement of the stomach –– ‘Halo’ sign around the kidneys as a
• Jejunal or ileal fold thickening result of fluid in the anterior perirenal
Ultrasound space
• Enlarged pancreas
• Diffuse or focal hypoechoic areas Pancreatic pseudocyst
• Frequently difficult because overlying • Cyst formation within the pancreas,
bowel obscures the area usually secondary to inflammation (mostly
chronic pancreatitis); acute collections
CT
in the pancreas or lesser sac are not
• Modality of choice
pseudocysts
• Interstitial oedematous pancreatitis
• Requires > 4 weeks to form and matures in
–– Pancreatic and peripancreatic oedema
6–8 weeks
–– Contour changes
• Two-thirds are seen within the pancreatic
–– Duct dilatation
tissue, with the omental bursa the second
–– Altered pancreatic attenuation
commonest site for a pseudocyst
–– Differentiated from acute necrotising
• Complications
pancreatitis by the presence of contrast
–– Rupture into peritoneum (generalised
enhancement of the whole pancreas,
peritonitis) or into a loop of bowel
whereas in necrotising pancreatitis
(decompression/fistulation)
there is patchy enhancement

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150 Gastrointestinal system

–– Haemorrhage from vessels in wall or pancreaticoduodenal artery branches, and


pseudoaneurysm it can be confused with a pseudocyst
–– Air in pseudocyst must raise the • Haemorrhage occurs in up to 5% of cases
possibility of a fistula. The site can of pancreatitis as well as in relation to
usually be determined by a contrast pseudocysts and pseudoaneuryms
study
Emphysematous pancreatitis
Imaging features • Pancreatitis with super-added infection
Upper GI contrast study with gas forming bacteria
• Depending on where the pseudocyst • Commonest causative organism is
lies and on its size, it can cause smooth Escherichia coli
extrinsic indentations of either the • High mortality and morbidity
stomach and duodenal sweep or the • Majority of cases resolve with conservative
splenic flexure treatment
• Gastric outlet obstruction is a known • Differential diagnoses include duodenal
complication diverticulum, penetrating ulcer of the
Ultrasound stomach or duodenum, GI fistulae from
• Usually a single, unilocular cyst surrounding structures and iatrogenic
• May show debris within it aetiologies, e.g. following an ERCP or roux-
• May increase in size with time en-Y surgery
• Rarely obstructs the CBD or the pancreatic
duct, leading to a clinical presentation of Chronic pancreatitis
jaundice
• Recurrent or continued inflammation
CT of the pancreas leading to irreversible
• Starts as a subtle area of low density and changes to the anatomy and function of
then walls off the pancreas
• Contrast enhancement of the walls • Clinical features include recurrent
• Fluid in pseudocyst 0–30 Hounsfield units, abdominal pain and steatorrhea
but depends on the protein concentration • Often results in diabetes mellitus
of the fluid
Causes of chronic pancreatitis
Pancreatitis abscess
• Collection of pus close to the pancreas
Calcifying type
• Usually occurs 2–4 weeks after severe
• Juvenile tropical pancreatitis
acute pancreatitis
• Hereditary pancreatitis
• Most commonly caused by Escherichia coli
• Hyperlipidaemia
or polymicrobial
• Hypercalcaemia
• Candida abscesses may occur in
immunocompromised patients
Obstructive type
• May gas with some Gram-positive
• Trauma
infections
• Renal failure
Vascular complications • Cystic fibrosis
• Can cause vascular occlusion, • Sclerosing cholangitis
haemorrhage or pseudo-aneurysm • Ampullary tumour
• Occlusion of the splenic artery and vein is • Ampullary stenosis
common
• Pseudoaneurysm formation occurs in
10% of causes, and 30–40% of these have Imaging features
associated haemorrhage
Abdominal radiographs
• Usually affects the splenic artery
• Irregular calcification in the region of the
followed by the gastroduodenal and
pancreas

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Pancreas 151

CT and ultrasound • Mass or filling defect in the ampullary


• Gland size depends on fibrosis and region of duodenum
inflammation, which may be diffuse or CT
focal • Adenocarcinoma can be seen on CT in
• Hence there may be no change in size of 95% of cases
the pancreas, or atrophy or enlargement • Tumours of the head and body of the
• Associated loss of fat plane can be seen, pancreas produce a diffuse enlargement of
appearing like a tumour encasement the gland
• Calcification is irregular and most • Usually heterogeneous with central low
commonly first occurs in the head attenuation
• Duct involvement is important because • Biliary tree and pancreatic dilatation may
duct obstruction may cause symptoms and be seen
can be relieved surgically • Biliary tree dilatation occurs with tumours
• Decalcification of the gland is a known of the head of pancreas tumours; this is
entity abrupt compared with pancreatitis, in
• Beading (‘chain of pearls’ appearance) is which the dilatation is concentric and
seen in the pancreatic duct in 68% of cases gradual
MRI • Invasion of contiguous organs, including
• Loss of signal on fat-saturation T1WI the duodenum, stomach and mesenteric
• Poor contrast enhancement of the root
pancreas • Adenocarcinomas rarely calcify
• MRCP shows duct dilatation and beading (commonly non-functioning islet cell
tumours)
• Local extension to vessels is suggested
Pancreatic duct when there is a loss of the fat plane
adenocarcinoma between the mass and vessel
• Accounts for 80% of all pancreatic • Local extension may also cause dilatation
malignancies of the pancreaticoduodenal vein
• Peak age is 50–80 years • Lymph node spread is to the
• Causes peripancreatic and porta hepatis nodes,
–– Smoking which can be seen in the hepatoduodenal
–– Diabetes mellitus area and the root of the mesentery
–– Alcohol • Liver metastases occur commonly
–– Hereditary pancreatitis
• Commonest site is the head of the Intraductal papillary
pancreas (in 60–70% of cases)
• Presents clinically with weight loss,
mucinous tumour of
abdominal pain and obstructive jaundice the pancreas
Imaging features • Low-grade pancreatic malignancy arising
from the epithelial cells of the ducts
Ultrasound • Contains mucin
• Heterogeneous hypoechoic mass in the • Presents with abdominal pain and
pancreas pancreatitis
• Contour deformity • Two main types
• Pancreatic duct dilatation if the lesion is an –– Main duct intraductal papillary
obstructing one mucinous tumour of the pancreas
Barium meal –– Branch duct intraductal papillary
• ‘Inverted three’ sign mucinous tumour of the pancreas
• Antral padding • Branch type has good prognosis with
partial pancreatectomy

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152 Gastrointestinal system

Imaging features Imaging features


CT Ultrasound
• Dilatation of the main pancreatic duct • Cystic lesions with low-level internal
• Papillary projections into the main duct echoes
seen on thin-section CT CT
• Multilobulated cystic lesions in the • Single cyst or multiple cysts
uncinate process • Cysts are large and septate, with fluid
• Main duct dilatation of > 10 mm has a attenuation within them; attenuation
higher risk of being malignant value varies depending on the presence of
• Main duct dilatation may be segmental or protein or haemorrhage
focal, and the appearance may be of that of • Papillary or nodular excrescence is known
a pseudocyst
• The branch duct type is seen usually in the MRI
uncinate process • Similar findings to those of CT
• Two patterns are seen, microcystic and • Hyperintense or hypointense lesions
macrocystic within cysts on T1WI, depending upon
protein content
• On T2WI the cysts are hyperintense with
Cystic carcinomas of hypointense septae
the pancreas • Intracystic excrescences are seen
• Enhancement of the septae and solid
• Group of cystic tumours within the
components of the cyst
pancreas
• May affect the main duct or branch ducts Serous (microcystic) type
• Usually presents clinically with abdominal
• Usually benign
pain or weight loss
• Accounts for 40–50% of all cystic
• May be completely asymptomatic and
pancreatic neoplasms
discovered incidentally
• Peak age is 60–70 years
• Two main types
• Female predominance
–– Mucinous cystadenoma or
• Predominantly located in the head of
cystadenocarcinoma (macrocystic
the pancreas, although any part can be
type), which is thought to be malignant
affected
–– Serous cystadenoma or
• Associated with von Hippel–Lindau
cystadenocarcinoma (microcystic
disease
type), which is thought to be benign
• Multiple cysts (usually more than six),
Mucinous (macrocystic) type measuring < 2 cm in diameter, although a
few large cysts may be seen
• Includes both cystadenoma and
• Calcification is present and is non-specific
cystadenocarcinoma
• Good prognosis; can be surgically resected
• Accounts for 5–10% of all cystic pancreatic
neoplasms Imaging features
• Peak age is 40–50 years
Ultrasound
• Female predominance (19 times
• Multiple echogenic, predominantly solid
commoner in females)
areas
• Commonly seen in the body and tail of the
pancreas CT
• Usually fewer than six cysts of varying • Low-attenuation cystic areas
diameters but > 2 cm • Good enhancement on contrast, with
• Calcification is present and non-specific numerous small and medium-sized cysts
• There may be solid papillary projections that may be difficult to separate
into the cyst • A stellate late-enhancing scar is seen in
• Invasion of other organs is known 30% of cases

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Pancreas 153

MRI • Benign in 90% of cases, malignant in 10%


• High-signal cystic lesions on T2WI, with • Single in 90% of cases, multiple in 10%
well-defined septae • Sporadic in 90% of cases
• Hypointense on T1WI • < 5 cm in 90% of cases
• Central scar not well seen • Intrapancreatic in 90% of cases
• Delayed contrast enhancement
Gastrinoma
Cystic lesions of the pancreas • Second commonest islet cell tumour
• Peak age < 20 years
• Pancreatic collections or pseudocysts • Sporadic in 70% of patients
• Serous cystadenoma (second • Mostly benign and multiple
commonest cause) • Associated with multiple endocrine
• Pancreatic abscess neoplasia type 1, in which case they are
• Benign pancreatic cysts malignant
• Parasitic cysts • Usually in the head of the pancreas
• Pancreatic dermoid cysts • Gastrinoma triangle: porta hepatis at the
• Pancreatic haematoma and traumatic apex with the second and third parts of the
pancreatitis duodenum as the base
• Up to 30% are ectopic
• Presentation is with Zollinger–Ellison
syndrome
• Increased risk peptic ulcer disease
Pancreatic islet cell
tumours Non-functioning islet cell
tumours
• Derivatives of the amine precursor uptake
• Third commonest islet cell tumour
decarboxylase (APUD) cells or the islet
• May be benign or malignant
cells of Langerhans
• The benign type are usually asymptomatic
• Associated with multiple endocrine
and do not grow large enough to be
neoplasia type 1
diagnosed
• Functional in 80–90% of cases
• Malignant tumours needs to be
Imaging features differentiated from each other because the
treatment differs
Ultrasound
• 80–100% of all benign non-functioning
• Mass in the head of the pancreas
islet cell tumours eventually turn
• Hypoechoic
malignant
CT • May cause a mass effect and compression
• Mass in the head of the pancreas, with or or invasion of structures
without necrosis • Associated with multiple endocrine
• Coarse calcification neoplasia type 1
• Enhances with contrast • Peak age is from the third decade onwards
• Nodular calcification seen in 20–30% of
Insulinoma cases
• Commonest islet cell tumour • Usual location is the pancreatic head
• Peak age is 40–60 years
• Usually < 2 cm in diameter Glucagonoma
• May be multiple • Uncommon
• No specific predilection for any location • 75% are malignant
• Presents with Whipple’s triad • Almost all are symptomatic
–– Spontaneous hypoglycaemia • Associated with multiple endocrine
–– Blood glucose below 50 mg/ml neoplasia type 1
–– Relief by administration of glucose

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154 Gastrointestinal system

• Peak age is 50–79 years • Presents clinically with diabetes mellitus


• Female predominance or steatorrhea
• Commonest location is in the body or tail • Usually < 3 cm in diameter
of the pancreas, although ectopic sites • Malignant tumour that metastasises to the
seen liver
• Clinical presentation
–– Necrolytic erythema migrans Metastases to the pancreas
–– Diarrhoea
–– Diabetes mellitus • Renal cell carcinoma
–– Glossitis • Bronchogenic carcinoma
–– Weight loss • Breast carcinoma
–– Unexplained thromboembolic • GI stromal tumours
complications (deep venous • Melanoma
thrombosis, pulmonary embolism) in • Carcinoid
25% of cases • Adrenal carcinoma
• Large masses seen in the pancreas on • Thyroid carcinoma
imaging • Angiosarcoma
• Liver metastases occur in 50% of cases • Can affect any part of the pancreas,
although the head is the commonest
VIPoma area
• Secretes vasoactive intestinal peptide • Usually associated with metastases to
(VIP) other areas
• Two thirds occur in women • Differential diagnosis includes ductal
• Location carcinoma
–– Pancreas: in the body and tail
–– Extra-pancreatic: in the
retroperitoneum and mediastinum in
children, usually from a neurogenic
tumour, e.g. ganglioblastoma,
Multiple endocrine
neuroblastoma neoplasia
• Clinical presentation • Group of syndromes associated with
–– Watery diarrhoea, hypokalaemia, endocrine hyperplasia
hypochlorhydria (Verner–Morrison’s • Autosomal-dominant condition
syndrome) • Hyperplasia of glands
–– Gallbladder dilatation, caused by • Type 1 is also known as Wermer’s
paralysis of smooth muscle secondary syndrome
to the VIP secretion • Type 2 is also known as Sipple’s syndrome
–– Commonly malignant, with metastasis • Type 3 is also known as mucosal neuroma
to the liver in approximately 59% of syndrome
cases at presentation
Type 1
Somatostatinoma
• The glands involved have multiple
• Predominantly located in head of the tumours
pancreas • May be malignant
• Peak age is 50 years

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Pancreas 155

• Commonest sites are parathyroid glands, Type 2B


the pancreas (mostly islet cell tumours)
• Accounts for 5% of all cases of multiple
and the anterior pituitary gland
endocrine neoplasia type 2
• Peak age is 30–50 years
• Marfanoid features with mucosal
• An all-or-none phenomenon, i.e. all the
neuromas
above sites are affected in this syndrome
• Aggressive form that frequently manifests
• Multiple lipomas and angiofibromas may
itself in childhood
lead to the diagnosis before the endocrine
• Commonly causes medullary thyroid
manifestations are found
carcinoma, phaeochromocytomas, and
Type 2A oral, intestinal mucosal neuromas
• May cause intussusception as a result of
• Accounts for most cases of multiple
neuromas in the bowel
endocrine neoplasia
• Usually RET oncogene-positive Carney’s complex
• Commonest sites are the thyroid
• Rare type of multiple endocrine neoplasia
(medullary C cell carcinoma), the adrenal
• Causes primary pigmented adrenocortical
glands (phaeochromocytomas, which
disease, pituitary adenomas, Sertoli cell
are bilateral in 70% of cases) and the
tumours, thyroid nodules and non-
parathyroid gland (hyperplasia)
endocrine features including cardiac
• Associated with carcinoid and Cushing’s
myxomas and schwannomas
syndrome

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3.4 Oesophagus

Congenital oesophageal –– Type E: TOF without oesophageal


atresia or so-called H-type fistula (4%
atresia and tracheo- of cases)
oesophageal fistula (TOF) –– Type F: congenital oesophageal
stenosis (< 1% of cases)
• Oesophageal atresia refers to a
congenitally interrupted oesophagus Imaging features
• One or more fistulae may be present Antenatal ultrasound
between the malformed oesophagus and • Polyhydramnios, which occurs in 33% of
the trachea or the TOF fetuses with oesophageal atresia and distal
• Presents clinically with an inability to TOF and in almost 100% of fetuses with
swallow or with aspiration oesophageal atresia without fistula
• Variants
–– Type A: oesophageal atresia without CXR
fistula or so-called pure oesophageal • May show tracheal compression and
atresia (10% of cases) deviation
–– Type B: oesophageal atresia with • Absence of gastric air bubble indicates
proximal TOF (<1% of cases) type A or type B oesophageal atresia
–– Type C: oesophageal atresia with distal • Aspiration pneumonia into the posterior
TOF (85% of cases) segment of the upper lobes
–– Type D: oesophageal atresia with • Nasogastric tube ‘coils’ following insertion
proximal and distal TOF (< 1% of cases) • Contrast swallow may be necessary to
identify the position of the fistula but

Associated anomalies (VACTERL)

• Vertebral defects: multiple or single • Tracheo-oesophageal defects:


hemivertebrae, scoliosis, rib deformities oesophageal atresia

• Anorectal malformations: imperforate • Renal anomalies: renal agenesis


anus of all varieties, cloacal deformities (including Potter’s syndrome, bilateral
renal agenesis, bilateral reanal dysplasia),
• Cardiovascular defects: ventricular horseshoe kidney, polycystic kidneys,
septal defect (the commonest cardiac urethral atresia, ureteral malformations
defect), tetralogy of Fallot, patent
ductus arteriosus, atrial septal defects, • Limb deformities: radial dysplasia, absent
atrioventricular canal defects, aortic radius, radial ray deformities, syndactyly,
coarctation, right-sided aortic arch, single polydactyly, lower-limb tibial deformities
umbilical artery, others

VACTERL syndrome occurs when three or more of these associated anomalies are present

Ch-03.indd 156 8/12/2010 12:18:29 PM


Oesophagus 157

the examination carries a high risk of • Dysphagia for solids, exacerbated by stress
aspiration and cold liquids
• There is an association of right sided aortic • May be idiopathic or caused by Chagas’
arch, seen in 5% of patients with TOF, and disease (infiltration of Auerbach’s plexus
this is important for surgical planning by Trypanosoma cruzi)
• Complications include oesophageal
Acquired TOF carcinoma (in 2–7% of cases) and
aspiration pneumonia caused by
Iatrogenic acquired TOF regurgitation of pooled secretions and
• Traumatic TOF occurs secondary to either food particles
blunt trauma or open avulsion to the neck • Treatment is with balloon dilatation or
or thorax surgical myotomy (Ramsted’s operation)
• Usually located at the level of the carina • Main differential diagnosis is malignant
and appears several days after initial injury pseudoachalasia secondary to gastric
• An endotracheal tube cuff can increase and oesophageal carcinoma; the main
the pressure posteriorly when there is a differentiator is timing of symptom onset
nasogastric tube in the oesophagus, giving (pseudoachalasia characteristically has a
rise to a TOF more rapid onset)
• The risk is increased in patients with poor Imaging features
nutrition, as occurs in long-term intensive
care unit patients CXR
• Rarely occurs in relation to tracheostomy • Double right mediastinal stripe
tubes • Convex opacity behind right heart border
• Air–fluid level in thoracic oesophagus
Malignant acquired TOF (usually retrocardiac)
• Malignant TOF is most commonly • Small gastric air bubble
associated with oesophageal • Aspiration pneumonia
malignancies, although associations Barium swallow
with lung carcinoma and with metastatic • Pooling of contrast with distal oesophageal
squamous cell laryngeal carcinoma are dilatation (in 90% of cases)
also recognized • ‘Birds beak’ at the tip of the distal
• Most often occurs in the trachea (50% of oesophagus (in 90% of cases)
cases) and bronchi (40% of cases) • Failure of peristalsis to clear contrast
• Prognosis is poor because there is a high • Antegrade and retrograde motion of
risk of aspiration pneumonitis barium
• Imaging to demonstrate aspiration is by • Associated epiphrenic diverticulae
contrast swallows or CT
• Chest X-rays may imply the presence
of aspiration by evidence of recurrent
Oesophageal cancer
aspiration pneumonia • Accounts for 4–10% of all gastrointestinal
cancers
• Males four times more commonly affected
Achalasia than females
• Failure of peristalsis and relaxation of • 20% occur in the upper third of the
lower oesophageal sphincter oesophagus, 30–40% in the middle third
and 30–40% in the lower third

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158 Gastrointestinal system

• Risk factors Barium swallow


–– Barrett’s oesophagus • Most accurate when done with a double-
–– Alcohol abuse contrast technique
–– Lye’s stricture • Filling defect in the oesophagus or ‘bird
–– Coeliac disease beaking’ of the distal oesophagus
–– Head and neck cancer • Strictures are irregular and
–– Smoking circumferential, with loss of superficial
–– Plummer–Vinson syndrome, achalasia, mucosa
asbestosis or tylosis • Ulceration may be seen
• Prognosis is poor with a 5–20% 5-year
CT
survival rate
• Useful in staging and surgical planning, as
• Nodal involvement is a poor prognostic
well as for reviewing postoperative features
factor
and recurrence
• Staging
• Insensitive for identifying nodal disease
–– T1: invasion of lamina propria or
because the major criteria currently used
submucosa
is node size
–– T2: invasion of the muscularis propria
–– T3: invasion of the adventitia Endoscopic ultrasound
–– T4: invasion of adjacent structures • More accurate than endoscopy in
• Histology diagnosing recurrent disease and more
–– Squamous cell carcinoma accounts for accurate than CT in local disease staging
50–70% of cases • Especially useful for disease that is
–– Adenocarcinoma accounts for 30–50% confined to the mucosa and submucosa
of cases
–– Other types include spindle cell, Boerhaave’s syndrome
mucoepidermoid, adenoid cystic,
leiomyosarcoma, rhabdomyosarcoma, • Oesophageal rupture caused by a sudden
fibrosarcoma and metastases rise in intraluminal oesophageal pressure
• Criteria for unresectability produced during vomiting
–– Pericardial effusion • Neuromuscular inco-ordination causes
–– Intraluminal mass in airway failure of the cricopharyngeus muscle to
–– Tumour extension between the trachea relax
and the aortic arch or between the left • Commonest anatomical location of the
main bronchus and the descending tear is at the left posterolateral wall of the
aorta lower third of the oesophagus, 2–3 cm
–– >90° contact with the aortic proximal to the gastro-oesophageal
circumference junction
–– Pleural thickening or pleural effusion • Second commonest site of rupture is in the
adjacent to tumour subdiaphragmatic or upper thoracic area
–– Mediastinal or coeliac axis nodes • 80% of all patients are middle-aged men
• Overall mortality rate is 30%
Imaging features • Mortality is usually due to subsequent
CXR infection, e.g. mediastinitis, pneumonitis,
• Widened azygo-oesophageal recess pericarditis, empyema
• Thick posterior or right tracheal stripe • Surgical intervention is required in most
• Wide mediastinum cases
• Tracheal deviation • Patients who undergo surgical repair
• Retrocardiac mass within 24 hours of injury have a 70–75%
• Posterior tracheal indentation chance of survival
• Aspiration pneumonia • This falls to 35–50% if surgery is delayed
longer than 24 hours and to approximately
10% if delayed longer than 48 hours

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Oesophagus 159

Imaging features Upper and mid-oesophageal


CXR perforation
• Useful in the initial diagnosis because in • Commonest site is the cricopharyngeus
90% of cases there is an abnormal finding • Iatrogenic condition
after perforation
• Commonest finding is a unilateral Imaging features
effusion, usually on the left (as most CXR
perforations are left-sided) • Widening of the prevertebral fascia with air
• The V sign of Naclerio (seen in 20% of and fluid and a right-sided pleural effusion
cases) is a specific but not a sensitive sign:
air in the space between the heart and Lower oesophageal perforation
vertebral column, seen as a left paraspinal Imaging features
hyperlucency
• Continuous diaphragm sign CXR
• Other findings may include • Pneumomediastinum
pneumothorax, hydropneumothorax, • Subcutaneous emphysema
pneumomediastinum, subcutaneous • Widening of the mediastinum (secondary
emphysema or mediastinal widening to mediastinitis)
• 10% of CXRs are normal • Hydrothorax (usually left-sided)
• Findings may take an hour or more to • Hydropneumothorax
develop after perforation Water-soluble swallow
Contrast swallow • Single contrast swallow using a non-ionic
• Non-ionic contrast is used contrast
• Confirmatory test with up to 90% • Entry of contrast into the mediastinum
sensitivity or pleural cavity proves the presence of
• Contrast is seen to enter the pleural cavity rupture
• false-negative rate is approximately 10%
CT
• Extraluminal air within the mediastinum CT
in conjunction with the clinical picture • Similar findings to those seen in
gives up to a 90% correlation Boerhaave’s syndrome
• The air may be seen to track alongside the • Focal extraluminal collections of air
aorta adjacent to the site of rupture
• Oesophageal wall haematoma • Obliteration of fat planes in the
• Contrast extravasation mediastinum
• Peri-oesophageal or mediastinal fluid • Peri-oesophageal fluid collections
• If diagnosis has been delayed, there may • Contrast extravasation into the
be a mediastinal abscess or empyema mediastinum
• Delayed imaging may show the presence
of mediastinal abscess formation, seen as
Oesophageal perforation an enhancing cavitating lesion with an air–
• Associated with a 20–60% mortality fluid level
• Causes
–– Iatrogenic (in 55% of cases) Mallory–Weiss tear
–– Spontaneous (Boerhaave’s syndrome)
–– Trauma • Upper gastrointestinal bleeding secondary
–– Oesophageal cancer to longitudinal mucosal lacerations at the
–– Retained foreign body gastro-oesophageal junction or gastric
–– Barrett’s ulcer cardia

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160 Gastrointestinal system

• Bleeding stops spontaneously in 80–90% of A ring


patients • Seen  1.5 cm proximal to the
• With conservative therapy, most tears heal squamocolumnar junction
uneventfully within 48 hours • A muscular ring that may be seen
• Tears can easily be missed if endoscopy is intermittently on barium swallow studies
delayed
• Clinical presentation is with haematemesis B ring (Schatzki’s ring)
following a bout of retching or vomiting • Technically a web, since it consists of
• Associated with a hiatus hernia in 35–100% mucosa and submucosa
of cases • Typically found at the squamocolumnar
• Iatrogenic tears are uncommon junction and forms the upper limit of a
• Barium swallow and gastrografin studies hiatus hernia
should not be performed, owing to their • A common complication is impaction of
low diagnostic sensitivity and interference meat at this site
with endoscopic assessment and therapy
• Endoscopy is the procedure of choice for
C ring
both diagnosis and therapy • A rare anatomical variant, which seen as
• Split is 2–3 cm in length and a few an indentation of the lower oesophagus
millimetres in width caused by the diaphragmatic crura
• > 80% of cases present with a single tear Oesophageal web
• The usual location of the tear is just below
the gastro-oesophageal junction on the • A thin (2–3 mm), eccentric, smooth
lesser curve of stomach extension of normal oesophageal tissue
• Most patients have stopped bleeding at the consisting of mucosa and submucosa
time of endoscopy • Can occur anywhere along the length
of the oesophagus, but typically in the
anterior postcricoid area of the proximal
Oesophageal rings and oesophagus
webs • Plummer–Vinson syndrome is
characterised by a post-cricoid or upper
• Commonest structural abnormalities of oesophageal eccentric web and iron-
the oesophagus deficiency anaemia
• Most patients with oesophageal rings and • A web can also be congenital or
webs are asymptomatic developmental in origin, or inflammatory
Oesophageal ring or autoimmune in nature
• Barium swallow is the diagnostic test of
• Concentric, smooth, thin (3–5 mm) choice; endoscopy is less sensitive
extension of the normal oesophageal
tissue consisting of three anatomic layers
of mucosa, submucosa and muscle Oesophageal strictures
• Most oesophageal rings are found • Causes
incidentally, are asymptomatic, and do not –– Acid peptic disease
require treatment –– Autoimmune disease
• Those that are symptomatic may be treated –– Infection
with endoscopic dilatation or disruption –– Caustic injury
and, rarely, with surgery –– Congenital anomaly
• Three main types of oesophageal rings, –– Iatrogenic
classified as A, B (Schatzki’s ring) and C –– Medication-induced
(indentation caused by the diaphragmatic –– Radiation-induced
crura); other rings may be seen that are –– Malignancy
either congenital or inflammatory in origin –– Idiopathic

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Oesophagus 161

• Peptic strictures account for 70–80% increases capillary pressure in organs


of cases, usually originate from the drained by the obstructed veins
squamocolumnar junction and are • Gastroesophageal varices have two main
typically 1–4 cm in length in-flows: the left gastric or coronary vein
• 10 times commoner in whites and the splenic hilum via the short gastric
• Symptoms include heartburn, dysphagia, veins
odynophagia, food impaction, weight loss • Patients who have bled once from
and chest pain oesophageal varices have a 70% chance of
• Males are affected 2.5 times more rebleeding
commonly than females • Approximately one third of further
• Medical management is with proton pump bleeding episodes are fatal
inhibitors • Endoscopy is required at an early stage to
• Surgical management is by endoscopic formulate the management plan
dilatation, stricturoplasty, stenting or • Obstruction and increased resistance can
limited oesophagectomy occur at three levels in relation to hepatic
sinusoids
Classification –– Pre-sinusoidal venous obstruction
• Smooth oesophageal stricture may be due to portal vein thrombosis,
–– Inflammatory: acid peptic disease, schistosomiasis or primary biliary
scleroderma, corrosive injury, cirrhosis
iatrogenic –– Elevated portal venous pressure (but
–– Neoplastic: carcinoma, mediastinal with a normal wedged hepatic venous
tumour, leiomyoma pressure) can occur with any cause of
–– Achalasia liver cirrhosis
–– Skin disorders, e.g. epidermolysis –– Post-sinusoidal obstruction can occur
bullosa, pemphigus in Budd–Chiari syndrome or veno-
• Irregular oesophageal stricture occlusive disease in which the central
–– Neoplastic: carcinoma, hepatic venules are the primary site
leiomyosarcoma, carcinosarcoma, of injury; the wedged hepatic venous
lymphoma pressure is characteristically elevated
–– Inflammatory: acid reflux, Crohn’s
disease Imaging features
–– Iatrogenic: radiotherapy, fundoplication Contrast studies
Diagnosis • Tortuous structures presenting as
longitudinal filling defects projecting into
• Barium swallow provides an objective
the lumen of the oesophagus
baseline record of the oesophagus
• May also show as thickened oesophageal
prior to medical therapy or endoscopic
folds
intervention
• Scalloping of the borders on a single
• Other tests include CT, endoscopic
contrast examination
ultrasound, pH monitoring and
manometry CT
• Imaging findings on non-contrast scans
can appear similar to lymph nodes
Oesophageal varices • Post-contrast images show serpigenous
• Obstruction of the portal venous system tubular structures within the wall of the
leads to increased portal pressure oesophagus that project into the lumen of
• Normal pressure in the portal vein is the oesophagus
5–10 mmHg; elevated portal venous • Para-oesophageal varices are outside the
pressure (> 10 mmHg) distends the veins oesophageal wall
proximal to the site of the blockage and

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162 Gastrointestinal system

Medical management • Complications


• Oesophageal varices with no history of –– Strictures occur as a result of
bleeding are treated with non-selective circumferential fibrosis secondary to
beta-adrenergic blockers chronic deep injury
• Bleeding varices –– Barrett’s oesophagus
–– Endoscopic sclerotherapy is successful Barium swallow (to assess anatomy
in controlling acute oesophageal
and complications)
variceal bleeding in up to 90% of cases
–– Endoscopic variceal ligation (banding) • Oesophageal peristalsis: 50% of patients
can also be used have ineffective swallow associated with
–– Rebleeding occurs less frequently with GORD; this needs to be differentiated from
ligation than with sclerotherapy (26% achalasia and scleroderma, since they are
versus 45%) the differential diagnoses
–– Local complications are also less • Oesophagitis, stricturing and Barrett’s
common with ligation than with oesophagus can also be looked for
sclerotherapy • Hiatus hernias can be assessed and typed
–– Identification of the gastro-oesophageal
Surgical management junction and the diaphragmatic hiatus
• 5–10% of patients with oesophageal with more than 1.5 cm difference
variceal haemorrhage have conditions that between them indicates a sliding hernia
cannot be controlled by endoscopic or –– Para-oesophageal hernias are those in
pharmacologic treatment which the gastro-oesophageal junction
• Surgical options remains below the diaphragm and in
–– Portosystemic shunt which the fundus herniates
–– Devascularisation of the lower 5 cm –– Type 3 hernias consist of hernias with
of the oesophagus and the upper two both the above features
thirds of the stomach –– Type 4 hernias are herniation of all or
–– Orthotopic liver transplantation part of the stomach with some element
of organoaxial rotation
Radiological management • Shortened oesophagus
• Percutaneous transhepatic embolisation • Reflux demonstrated on the swallow
• Transjugular intrahepatic portosystemic
shunts (TIPS); complications include
encephalopathy (in 20% of cases) and Barrett’s oesophagus
shunt occlusion (50% at 1 year) • Finding of specialized intestinal
(columnar) metaplasia anywhere within
Gastro-oesophageal reflux the tubular oesophagus
• Commonest site is above the
disease (GORD) squamocolumnar junction
• Gastro-oesophageal disease is the • Barrett’s ulcers are deep penetrating ulcers
commonest cause of oesophagitis and are usually associated with strictures;
• Symptoms include dysphagia, at times only a slowly tapering stricture
odynophagia and heart burn without ulceration may be seen
• Classification • Risk factors
–– Grade I: erythema –– GORD
–– Grade II: linear, non-confluent erosions –– Hiatus hernia (in 75–90% of cases of
–– Grade III: circular, confluent erosions Barrett’s oesophagus)
–– Grade IV: stricture or Barrett’s –– Reduced lower oesophageal sphincter
oesophagus pressures

Ch-03.indd 162 8/12/2010 12:18:30 PM


Oesophagus 163

–– Delayed oesophageal acid clearance –– Grade III: circular, confluent erosions


time –– Grade IV: stricture or Barrett’s
–– Duodenogastric reflux oesophagus
• Risk of progression to adenocarcinoma
of the oesophagus is estimated at Candida oesophagitis
approximately 0.5% per year in patients • Predominantly occurs in
without dysplasia on initial surveillance immunosuppressed patients
biopsies • Oral thrush is a frequent finding and
• Management is often an indicator of oesophageal
–– Antireflux surgeries, such as a Nissen involvement
fundoplication, have not been shown • However, oral thrush can be absent in 25%
to reverse the outcome. Surgery seems of cases of Candida oesophagitis
to play no role in preventing the • Candida infection is frequently
progression to cancer asymptomatic
–– Oesophagectomy when high-grade • Predilection for the upper half of the
dysplasia is discovered and confirmed oesophagus, with longitudinal orientation
by a second pathologist
Imaging features
Imaging features Barium swallow
Barium swallow • Identified by double-contrast barium
• Reticular pattern (non-specific sign that is swallow in 90% of cases
also seen in other forms of oesophagitis) • Initially starts as a plaque lesions
• There may be stricturing or ulceration • Granular pattern of mucosal oedema,
• Stricturing occurs at the squamocolumnar caused by confluence of the plaques
junction, is short and tight and typically • In advanced cases, such as occur in
eccentric in nature, whereas peptic AIDS patients, there may a shaggy
strictures are more concentric and smooth appearance to the oesophagus, caused by
• An ascending stricture may be seen on pseudomembranes and confluent plaques
serial barium swallows; this is caused • Owing to the friable nature of the lesions,
by the proximal migration of the the barium sometimes dissects into the
squamocolumnar junction and is specific submucosa and gives a ‘double-barrel’
to Barrett’s oesophagus oesophagus
• Sliding hiatus hernia is also associated
with Barrett’s oesophagus Grading scale
• Needs confirmation with • Grade 1: A few raised, white plaques up to
oesophagogastroduodenoscopy 2 mm in size; no ulceration
• Grade 2: multiple raised, white plaques
> 2 mm in size; no ulceration
Oesophagitis • Grade 3: confluent, linear, nodular,
• Causes include GORD (the commonest elevated plaques with ulceration
cause), infections, medications, radiation • Grade 4: grade 3 with narrowed lumen
therapy, systemic disease and trauma (stricturing)
• Clinical features include dysphagia,
pain, odynophagia and (in severe cases) Tuberculous oesophagitis
malnutrition • Occurs with advanced mediastinal or
• Diagnosis is by barium swallow or pulmonary tuberculosis
endoscopy • Mediastinal nodes are enlarged and erode
• Classification into the oesophagus
–– Grade I: erythema • Oesophagogastroduodenoscopy reveals
–– Grade II: linear, non-confluent erosions shallow ulcers, heaped-up lesions

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164 Gastrointestinal system

that mimic neoplasia, and extrinsic HIV oesophagitis


compression of the oesophagus
• Usually presents with acute-onset
• Primary tuberculous infection of the
odynophagia plus a maculopapular rash
oesophagus is extremely rare but has been
• Causes giant flat ulcers > 1 cm in
reported; it appears as mucosal plaques or
diameter with satellite ulcers and a rim of
serpiginous ulcers, strictures or fistulas
oedematous tissue, similar in appearance
Cytomegalovirus (CMV) to CMV oesophagitis, although no
oesophagitis infectious aetiology is found
• Multiple, small, aphthoid lesions
• Almost always occurs in the • Later, giant deep ulcers extend up several
immunocompromised patients and most centimetres
commonly in patients with AIDS • Fistula formation, perforation,
• Presents with severe odynophagia and haemorrhage, or superinfection may
dysphagia, with evidence of dissemination complicate large ulcers
• One or more giant superficial erosions
in the middle to distal oesophagus are Behçet’s disease
observed • Tunneling ulcers, fistulas and perforations
• The erosions are diamond-shaped, ovoid seen
or serpiginous, with satellite ulcers and a
halo of oedematous tissue, as in herpes Scleroderma
simplex virus (HSV) oesophagitis • A more plastic deformity of the
• With progression of the infection, shallow oesophagus with poor clearing and
ulcerations may deepen and expand to up residual epithelial damage
to 5–10 cm in diameter
Inflammatory bowel disease
HSV oesophagitis • Oesophagitis is most commonly seen in
• Second commonest type of oesophagitis Crohn’s disease
• Diagnosis is made at endoscopy • Ulcers are aphthoid
• Earliest oesophageal lesions are rounded • Associated sinus, fistulous connections
vesicles 1–3 mm in diameter, located in the and polyps are seen
mid- to distal oesophagus • Management is directed at the underlying
• The centres of the lesions slough to form cause
discrete circumscribed ulcers with raised
edges
• A halo of oedema is seen at the margins Duplication cyst
• Advanced HSV oesophagitis may • Completely surrounded by muscularis
be indistinguishable from candidal propria
oesophagitis: plaques, cobble stoning or • Intramural masses
a shaggy ulcerative appearance may be • Fluid density (high protein may cause high
observed signal on T2WI)
Varicella–zoster virus
oesophagitis Leiomyoma
• The key to diagnosis is finding concurrent • Commonest benign tumour of the
dermatological varicella–zoster virus oesophagus
lesions • More common in the distal oesophagus
• The appearance on endoscopy ranges from (where there is smooth muscle)
occasional vesicles to discrete ulcerative • Arises from all layers of the wall of the
lesions to a confluence of ulcerations with oesophagus
necrosis

Ch-03.indd 164 8/12/2010 12:18:30 PM


Oesophagus 165

• Multiple leiomyomas are associated with • Appears through the thyrohyoid


neural deafness and renal impairment membrane
(Alport’s syndrome) • Usually asymptomatic
• Rarely associated with hypertrophic • Commoner in patients with chronically
osteoarthropathy elevated intrapharyngeal pressure, e.g.
• Predominantly an intramural lesion with glass-blowers, trumpeters
potential exophytic components
• They may metastasise if they are malignant Lateral cervical oesophageal
pouch and diverticulum
Imaging features
• Appears through the Killian–Jamieson
CT space, below the cricopharyngeus muscle
• Homogeneously enhancement or rim- • Usually asymptomatic
like enhancement with intratumour
calcification
• Well-defined mass with a smooth surface
Tertiary contractions in
the oesophagus
Pharyngeal and • Unco-ordinated, non-propulsive
oesophageal pouches and contractions
• Occur in patients with impaired motor
diverticula function due to muscle atrophy
Zenker’s diverticulum • Commonest in elderly patients (seen in
25% of those > 60 years)
• Located in the posterior part of the • Causes
oesophagus, usually to the left –– Reflux oesophagitis
• Forms between fibres of the inferior –– Obstruction at the gastric cardia
constrictor and cricopharyngeus muscles –– Presbyoesophagus
• Symptoms include dysphagia, –– Neuropathy
regurgitation, aspiration and hoarseness –– Diabetes mellitus
Lateral pharyngeal pouch and –– Alcohol excess
–– Malignant infiltration of the oesophagus
diverticulum –– Early achalasia
• Located in the anterolateral wall of upper
hypopharynx

Ch-03.indd 165 8/12/2010 12:18:30 PM


3.5 Stomach

Gastritis • Symptoms: Gnawing or burning epigastric


distress, occasionally accompanied by
• Inflammation of the gastric mucosa nausea and/or vomiting. The pain may
• The common mechanism of injury is an improve or worsen with eating
imbalance between the aggressive and • Complications
the defensive factors that maintain the –– Bleeding from an erosion or ulcer
integrity of the gastric lining (the mucosa) –– Gastric outlet obstruction due to
• Causes oedema limiting the adequate transfer
–– Drugs, including non-steroidal anti- of food from the stomach to the small
inflammatory drugs, which cause acute intestine
gastritis distally on or near the greater –– Dehydration from vomiting
curvature of the stomach –– Renal insufficiency as a result of
–– Alcohol dehydration
–– Bile
–– Ischaemia Imaging features
–– Bacterial (Helicobacter pylori), viral and Barium studies
fungal infection • Thick folds, inflammatory nodules, coarse
–– Acute stress (shock) area gastrica, and erosions
–– Radiation
Endoscopy
–– Allergy and food poisoning
• May reveal a thickened, oedematous, non-
–– Direct trauma
pliable wall with erosions and reddened
gastric folds
Helicobacter pylori gastritis
• Ulcers and frank bleeding are sometimes
present
• Helicobacter pylori gastritis starts as
acute gastritis in the antrum, causing
intense inflammation, and over time, it Phlegmonous gastritis
may extend to involve the entire gastric • Uncommon form of gastritis caused by
mucosa resulting in chronic gastritis numerous bacterial agents, including
• The acute gastritis encountered streptococci, staphylococci, Proteus
with Helicobacter pylori is usually species, Clostridium species and
asymptomatic Escherichia coli
• The bacteria embed themselves in the • Usually occurs in debilitated patients
mucous layer, a protective layer that • Associated with a recent large intake of
coats the gastric mucosa alcohol, a concomitant upper respiratory
• The bacteria protect themselves from tract infection and AIDS
the acidity of the stomach through the
production of large amounts of urease
• Infection produces inflammation via
Emphysematous gastritis
the production of a number of toxins • Widespread phlegmonous gastritis caused
and enzymes by mucosal disruption
• The intense inflammation can result in • Characterised by gas in the wall of the
the loss of gastric glands responsible for stomach
the production of acid (atrophic gastritis) • Clinical features include bloody, foul-
• Helicobacter pylori is associated with smelling emesis
60% of gastric ulcers and 80% of • There may be vomiting of a necrotic cast of
duodenal ulcers stomach, which is pathognomonic
• It is also associated with gastric cancer

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Stomach 167

Corrosive gastritis Mucosa-associated


• In acid-induced gastritis, the oesophagus lymphoid tissue (MALT)
is usually unharmed and gastric damage is lymphoma
severe
• In alkali-induced gastritis, the pylorus and • Normal stomach does not contain
antrum are frequently involved lymphoid follicles but they can develop
following infection with Helicobacter pylori
• Persistent antigenic stimulation by
Gastric cancer Helicobacter pylori is thought to lead to
• Third commonest GI malignancy neoplastic transformation
• Symptoms include GI bleeding, abdominal • MALT lymphoma is usually locally
pain and weight loss contained at the time of diagnosis and has
• Risk factors a better prognosis than non-Hodgkin’s
–– Smoking lymphoma
–– Nitrites and nitrates • Commonest site is the antrum
–– Pickled vegetables • Perforation is rare
–– Helicobacter pylori gastritis Imaging features
–– Chronic atrophic gastritis
–– Adenomatous and villous polyps Barium study
–– Gastrojejunostomy and partial • Infiltrative focal lesion or a diffuse lesion
gastrectomy • Ulceration is rare
–– Pernicious anaemia CT
–– Ménétrier’s disease • Thickening of the gastric wall with or
• Adenocarcinoma accounts for 95% of cases without ulceration and enhancement
• Overall 5-year survival rate is 5–18% • Dissemination is commonly seen
• Most are located in the distal third of
stomach or in the cardia; 60% are on lesser
curve, 10% on the greater curve and 30% at
Peptic ulcer disease
the gastro-oesophageal junction • Inflammatory injury in the gastric or
• Staging duodenal mucosa, with extension beyond
–– T1: limited to mucosa or submucosa the submucosa into the muscularis
–– T2: involves muscle or serosa mucosa
–– T3: penetrates through serosa • Risk factors
–– T4a: invasion of adjacent contiguous –– Helicobacter pylori infection
tissues –– Non-steroidal anti-inflammatory drugs
–– T4b: invasion of adjacent organs, –– Cigarette smoking
diaphragm or abdominal wall –– Zollinger–Ellison syndrome
• Metastases (gastrinoma)
–– Along peritoneal ligaments • Gastric and duodenal ulcers usually
–– To local lymph nodes cannot be differentiated on the basis of
–– Haematogenous spread, including to clinical history alone
the liver (25% of cases at presentation), • Complications
the adrenal glands and bone –– Haemorrhage
–– Peritoneal seeding, e.g. to the rectal wall –– Perforation
(Blumer’s shelf ), ovaries (Krukenberg’s –– Gastric outlet obstruction
tumour), the left supraclavicular node –– Gastric malignancy (the risk
(Virchow’s node) of malignant transformation is
approximately 2% in the initial 3 years)

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168 Gastrointestinal system

• Prognosis is excellent for benign ulcers, • Surgery has a role in life-threatening


especially if Helicobacter pylori is haemorrhage, ulcer perforation, gastric
completely eradicated and non-steroidal outlet obstruction, giant gastric ulcer, and
anti-inflammatory drugs are avoided a transfusion requirement of more than 6
units in 24 hours
Gastric ulcer
• Classic gastric ulcer pain is described as
occurring shortly after meals, for which
Gastric volvulus
antacids provide minimal relief • Abnormal degree of rotation of one part of
• Benign gastric ulcers are normally found stomach around another part
on the lesser curvature • > 180° rotation is required for complete
• These ulcers tend to project beyond the obstruction)
contour of the stomach, with radiating • Associated with araoesophageal hiatus
folds extending to the ulcer margin hernia and eventration of the diaphragm
• Malignant ulcers usually have irregular, • Two main types
heaped-up margins that protrude into the –– Subdiaphragmatic gastric volvulus,
lumen of the stomach which accounts for 30% of cases
–– Supradiaphragmatic gastric volvulus,
Duodenal ulcer which is seen in 60% of cases and is
• Duodenal ulcer pain often occurs associated with para-oesophageal
hours after meals and at night. Pain is rolling hiatus hernias
characteristically relieved with food or • Axis of rotation can be organoaxial (in
antacids which the rotation is along a line from the
• More than 95% found in the first part of the cardia to the pylorus in 60% of cases) or
duodenum mesenteroaxial (in which the rotation is
• Most < 1 cm in diameter around an axis extending from the lesser
curve to the greater curve)
Diagnosis • Symptoms include violent retching (but no
• Double-contrast barium study performed vomiting) and severe epigastric pain
by an expert GI radiologist has excellent • It may be impossible to pass a nasogastric
accuracy in diagnosing a typical gastric tube
ulcer • In cases of recurrent volvulus, a hiatus
• Endoscopy is the modality of choice hernia, eventration, diaphragmatic
• Provides the opportunity to perform trauma and abdominal adhesions should
multiple mucosal biopsies to check be considered as possible predisposing
for Helicobacter pylori and to rule out factors
malignancy
• A repeat endoscopy after 6 weeks of Imaging features
therapy is recommended to confirm AXR
healing of a gastric ulcer and to help to • Poor identifier but may show distended
rule out gastric malignancy definitively stomach and air–fluid levels with beaking
at the gastro-oesophageal junction
Treatment • If a nasogastric tube has been passed it lies
• Medical therapy: mucosal protectants, inferior to the gastro-oesophageal junction
histamine-2 blockers, proton pump
CT
inhibitors and Helicobacter pylori
• Identifies the anatomy better than AXR,
eradication (triple therapy has consistently
especially with the use of multiplanar
been shown to eradicate the organism in
reformats
> 90% of cases)
• Can also evaluate for ischaemic
• Endoscopic therapy: injection therapy,
complications
coagulation therapy, haemostatic clips and
argon plasma coagulation

Ch-03.indd 168 8/12/2010 12:18:31 PM


Stomach 169

Hypertrophic pyloric • Associated with neurofibromatosis type


1 and Carney’s triad (epithelioid gastric
stenosis stromal tumours, pulmonary chondromas
• Hypertrophy and hyperplasia of the and extra-adrenal paragangliomas)
circular muscle fibres of the pylorus, with • Medical treatment is with imatinib
proximal extension into gastric antrum mesylate (a selective tyrosine kinase
• Inherited as dominant polygenic trait inhibitor)
• Higher incidence in first-born boys • Surgical treatment is resection, which is
• Biochemistry shows hypokalaemic the definitive therapy
hypochloraemic metabolic alkalosis Imaging features
• Adult type
–– Secondary to mild infantile form Barium study
–– Acute obstructive symptoms • Double-contrast barium studies can
uncommon usually detect tumours that have grown to
–– Presents with nausea, vomiting and a size sufficient to produce symptoms
heartburn • Submucosal lesions with a smooth
• Infantile type overlying mucosa, thus preserving the
–– Presents at age 2–8 weeks areae gastrica
–– Clinical features include non-bilious • Larger lesions cause mucosal ulceration
projectile vomiting, a palpable olive- CT
shaped mass and hypochloraemic • Provides comprehensive information
metabolic acidosis about the size and location of the tumour
and its relationship to adjacent structures
Imaging features
• Lesions show rim enhancement or
Ultrasound homogeneous enhancement
• Target sign (hypoechoic ring of • Calcification may be present
hypertrophied pyloric muscle around • Larger masses undergo necrosis
echogenic mucosa centrally)
PET-CT
• Elongated pylorus (17 mm or more) • Useful for detecting metastases
• Pyloric muscle wall thickness (3 mm or
more)
• Pyloric transverse diameter 13 mm or
Causes of thickened gastric
more with channel closed mucosal folds (thickness
> 1 cm)
Gastric gastrointestinal • Inflammatory causes
stromal tumour –– Gastritis
–– Zollinger–Ellison syndrome
• Commonest mesenchymal neoplasm of
–– Acute pancreatitis
the GI tract
–– Crohn’s disease
• Third commonest tumour of the GI tract
• Infiltrative and neoplastic causes
(after adenocarcinoma and lymphoma)
–– Lymphoma
• 50–70% originate in the stomach; the small
–– Carcinoma
intestine is the second-commonest site
–– Pseudolymphoma
• Can also originate in the mesentery and
–– Eosinophilic gastroenteritis
omentum
• Other causes
• Submucosal lesions, which most
–– Ménétrier’s disease
frequently grow endophytically in parallel
–– Varices
with the lumen of the affected structure
• Clinical features include vague abdominal
pain, obstruction and bleeding

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170 Gastrointestinal system

Causes of linitis plastica Causes of target lesions in


• Neoplasia the stomach
–– Gastric carcinoma • Submucosal metastases (from melanoma,
–– Lymphoma lymphoma, carcinoma or carcinoid)
–– Metastases • Leiomyoma
–– Local invasion from pancreatic • Ectopic pancreatic tissue (pancreatic rest)
carcinoma • Neurofibroma
• Inflammatory causes
–– Corrosives
–– Radiotherapy
–– Granulomas
–– Eosinophilic enteritis

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3.6 Large and small bowel

GI tract trauma • Small bowel dilatation (> 2.5 cm) or a


fluid-filled small bowel
Haemoperitoneum • Presence of focal thickening and
• Found in paracolic gutters and the pelvis enhancement of the bowel wall with free
• CT attenuation values during intravenous fluid is suspicious of bowel perforation
contrast administration
–– Serum: 0–20 Hounsfield units
Blunt trauma to the bowel
–– Fresh unclotted blood: 30–45 • Causes include road traffic accident,
Hounsfield units bicycle handlebar injury and child abuse
–– Clotted blood: 50–100 Hounsfield units • The jejunum distal to the ligament of Treitz
–– Active bleed: > 180 Hounsfield units is the commonest location, followed by
• ‘Sentinel clot’ sign indicates the highest the duodenum, the ascending colon at the
attenuation value of blood clot and marks ileocaecal valve, and the descending colon
the site of injury Imaging features
Hypovolaemia CT
• Bowel discontinuity is primary finding but
Imaging features is rarely seen
CT • Extraluminal oral contrast is 100% specific
• Collapsed IVC (anteroposterior diameter but its sensitivity is low
< 9 mm) at three levels • Extraluminal air or pneumoperitoneum
–– Intrahepatic IVC with a halo of fluid has a sensitivity of 40–50% and is more
(non-specific) often seen in the setting of duodenal
–– At the renal artery level perforation or injury
–– 2 cm below renal artery level • Extraluminal air and intramural gas
• Heterogenous hepatic enhancement, indicates a full-thickness injury to bowel
typically 25 Hounsfield Units less than • Bowel wall thickening is seen in 75% of
the spleen, with intensely enhancing transmural injuries to the bowel and is
intrahepatic vessels and periportal very sensitive
oedema; differential diagnosis is fatty liver • Isolated mesenteric lacerations may also
• Small, hypodense spleen (non-specific) give this sign
• Small aorta (< 13 mm at levels 2 cm above • A combination of focal or disproportionate
and 2 cm below the renal arteries) and circumferential thickening of the
• Pancreatic enhancement (20 Hounsfield bowel wall with adjacent normal bowel is
Units greater than the liver) usually an indicator of injury
• Peripancreatic oedema in addition to • Bowel wall enhancement more than that
mesenteric and retroperitoneal fluid of the psoas muscle together with the signs
collections above is more specific for injury
• Intense and prolonged nephrogram (non- • Mesenteric stranding, predominantly on
specific) the mesenteric border, is an ancillary sign
• Intense adrenal enhancement greater than • Haemoperitoneum is a common finding
those of the IVC with intraperitoneal bowel or mesenteric
• Mucosal enhancement of the gallbladder lacerations
• Manifestations of shock bowel • Haematoma in the mesentery at the site
• Increased enhancement of small bowel of bowel wall thickening, as in the case of
mucosa (greater than the psoas muscle) a periduodenal haematoma, is also fairly
• Mural thickening of small bowel (> 3 mm) specific for bowel injury

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172 Gastrointestinal system

• Free fluid on three contiguous sections • Diaphragmatic hernias


suggests significant bowel or mesenteric –– Bochdalek’s hernia
injury –– Morgagni’s hernia
• Interloop fluid in the mesentery is • Lumbar hernias
associated with bowel wall injury –– Grynfelt’s hernia
• 5% of hepatic lacerations and splenic –– Petit’s lumbar triangle
lacerations are associated with major • Pelvic floor hernias
bowel injury –– Obturator (between the pectineus and
• Other findings are focal discontinuity of external obturator muscles)
the bowel wall, focal thickening of the –– Sciatic notch
bowel thickening, intramural haematoma, –– Perineal
mesenteric haematoma, extravasation of • Groin hernias
oral contrast and free fluid –– Inguinal (a direct inguinal hernia is
caused by a defect in Hesselbach’s
Malrotation triangle, medial to the inferior
epigastric vessels; an indirect hernia
• Abnormal position of the gut secondary to passes through the inguinal canal
narrow mesenteric attachment lateral to the inferior epigastric vessels)
• Caused by arrest in embryological –– Femoral
development of gut rotation and fixation –– Richter’s (entrapment of the
• Complications include midgut volvulus antimesenteric border of the bowel in
and duodenal obstruction the hernia orifice most commonly seen
in older women with a femoral hernia)
Imaging features
Barium meal Internal hernia
• Evaluates the position of peritoneal • Herniation of bowel through a
fixation developmental or surgically created defect
• In normal situation, barium flows in the peritoneum, omentum or mesentery
through the ‘C’-shaped configuration of or herniation through an adhesive band
duodenum; in addition, barium must pass • Paraduodenal hernias are associated with
to left of the spine a congenital defect in the descending
mesocolon
CT • Lesser sac hernias are through the foramen
• Superior mesenteric vein is seen to the left
of Winslow in a retrogastric location
the superior mesenteric artery (in 80% of
• Hiatus hernia: 99% are sliding hernias, in
cases)
which the gastro-oesophageal junction
remains in chest; in 1% of cases the
Hernia paraoesophageal portion of the stomach is
superiorly displaced into the thorax with
External hernia gastro-oesophageal junction remaining
• Bowel extends outside the abdominal subdiaphragmatic
cavity
• Ventral hernias
–– Postoperative Duodenum
–– At a trocar site (the commonest type) Duodenal diverticulum
–– Umbilical
• Primary: mucosal prolapse through the
–– Epigastric
muscularis propria
–– Spigelian (ventrolateral hernia through
• Secondary: involves all layers of the
a defect in the aponeurosis of the
duodenal wall; a complication of duodenal
transverse and rectus muscles)
inflammation

Ch-03.indd 172 8/12/2010 12:18:31 PM


Large and small bowel 173

• Usually occur in the first part of the –– Lymphatic obstruction


duodenum –– Angioneurotic oedema
• Complications include perforation, bowel • Infestations
obstruction, biliary obstruction, bleeding –– Worms
and diverticulitis –– Giardiasis

Causes of ‘cobblestone Causes of a dilated duodenum


duodenal cap’ • Mechanical obstruction
• Big cobblestones –– Bands
–– Hypertrophy of Brunner’s glands –– Atresia/webs/stenosis
–– Oedema –– Annular pancreas
–– Crohn’s disease –– Superior mesenteric artery syndrome
–– Varices • Paralytic ileus
–– Carcinoma • Scleroderma
–– Lymphoma
• Small cobblestones Intestinal lymphoma
–– Food residue
–– Duodenitis • Commonest malignant small bowel
–– Nodular lymphoid hyperplasia tumour
–– Heterotropic gastric mucosa • Commonest cause of intussusception in
children aged > 6 years
Causes of decreased or absent • Incidence is 4–20%; 10% have bowel
duodenal folds involvement
• Amyloidosis • Median age at time of onset is 60 years
• Crohn’s disease • Risk factors include coeliac disease, AIDS,
• Cystic fibrosis systemic lupus erythematosus, Crohn’s
• Scleroderma disease and chemotherapy
• Strongyloidiasis • Seen as a large, cavitating, ulcerating or
nodular mass with aneurysmal dilatation
Causes of thickened duodenal of the bowel lumen
folds • Primary lymphoma of the bowel can be
localised or diffuse, and is associated with
• Inflammatory causes
coeliac disease
–– Crohn’s disease
• Secondary lymphoma of the bowel occurs
–– Duodenitis
in generalised systemic disease
–– Pancreatitis
• Areas of spread (in descending order
–– Zollinger–Ellison syndrome
are the stomach (2.5% of all gastric
• Neoplasia
neoplasms), the small bowel (20% of
–– Metastases
malignancies), the rectum, the colon and
–– Lymphoma
the oesophagus
–– Infiltrations
–– Eosinophilic enteritis Pathology
–– Amyloidosis • Hodgkin’s lymphoma
–– Mastocytosis • Non-Hodgkin’s lymphoma (T cell or B cell)
–– Whipple’s disease –– High-grade and intermediate-grade
• Vascular causes tumours carry a poor prognosis
–– Intramural haematoma –– Low-grade tumours carry a good
–– Ischaemia prognosis
• Oedematous causes –– Low grade B-cell tumours are MALT
–– Hypoproteinaemia lymphomas
–– Venous obstruction –– Can be secondary to Helicobacter pylori
gastritis

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174 Gastrointestinal system

Imaging features valvulae and aneurysmal dilatation


• Polypoidal or nodular tumour in 47% of –– Mesenteric or retroperitoneal
cases lymphoma: may be single or multiple
• Diffuse infiltrating disease in 11%: hose- extraluminal masses, in a ‘cake’
like thickening with reduced peristalsis configuration engulfing multiple bowel
• Ulcerative tumour in 42% of cases, which loops or a ‘sandwich’ configuration in
can perforate which a mass surrounds mesenteric
vessels that are separated by
CT staging perivascular fat; can also occur as a
• Stage I: tumour confined to the bowel wall mesenteric and retroperitoneal mass
• Stage II: local nodes –– Endoexoenteric lymphoma: a large
• Stage III: widespread nodes mass with small intramural component,
• Stage IV: disseminated to the liver, marrow which can cause fistulas
or other sites
Lymphoma of the colon
Lymphoma of the stomach • Mainly affects the caecum (in 85% of
• 2.5% of all lymphomas present with gastric cases)
neoplasms • Can be seen as a single mass, as diffuse
• Commonest site of extranodal spread infiltration or as a polypoidal lesion
• Arises from the lamina propria after • Focal soft tissue thickening
Helicobacter pylori infection
• Extends to the pancreas, spleen, transverse Carcinoid tumour
colon and liver • Commonest primary malignant tumour of
• Diffuse mucosal thickening with or the small bowel
without ulcers in 50% of cases • 33% occur in the small bowel; 45% occur in
• Smooth nodular mass with or without the appendix
mucosal thickening (segmental • 33% of patients have a second primary
involvement is seen in 25% cases) malignancy
• Ulcers, which may be single or multiple, in • Distribution of GI carcinoid: 81% ileum,
8% of cases 7% jejunum, 2% duodenum, 10% gastric
• Linitis-like picture with thickened wall and • Arises from neuroendocrine cells of the
reduced motility submucosa
• May be seen as a duodenal ulcer • Secretes a variety of products, including
associated with a gastric mass, as an 5-hydroxyindoleacetic acid (5-HIAA),
ulcerated mass adrenocorticotropic hormone (ACTH),
histamine and serotonin
Lymphoma of the small
intestine Gastric carcinoid
• Usually solitary lesions located in
• Multiple sites of involvement
the gastric fundus or body and show
• Commonest cause of intussusception
metastasis at presentation in 50–70% of
• 51% are located in the ileum, 47% in the
cases
jejunum and 2% in the duodenum
• Best seen on double-contrast upper GI
• Arise from Peyer’s patches
examination as smoothly marginated
• Types
mural multifocal lesions with/out mucosal
–– Single or multiple polypoidal masses:
ulceration. They may be seen as thickened
seen as cobblestoning; associated with
mucosal folds. There may be flocculation
ulceration and intussusception
of contrast due to fluid in the stomach
–– Infiltrating lymphoma involving < 5 cm
• Mucosal or submucosal masses that
of the bowel wall: associated with a
enhance on CT, with mucosal ulceration
desmoplastic response, thickened

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Large and small bowel 175

Duodenal carcinoid • Strong hereditary component


• An intraluminal polypoid mass (in 50% of • Immune-mediated, inflammatory
cases) or an intramural mass (in 40%) response in the mucosa, resulting in
• On upper GI series they are seen as well- maldigestion and malabsorption
defined, rounded masses • Bimodal age distribution: 1–3 years in
• On CT they enhance on the arterial phase children and 30–50 years in adults
of the scan and lose contrast on delayed • Females are twice as commonly affected as
images males
• In the ampullary region they are found to • Most prevalent in western Europeans; rare
enhance, whereas adenocarcinomas and in Africans and Asians
adenomas do not • Primarily involves the mucosa of the small
intestine
Jejunal and ileal carcinoid • The submucosa, muscularis and serosa are
• Account for the majority of cases usually not involved
• Clinically presents with cramping • Villi are atrophic or absent, and crypts are
abdominal pain or with features caused by elongated
local effects • Cellularity of the lamina propria is
• On small bowel meal or enema, they are increased, with a proliferation of plasma
seen as single or multiple mural lesions, cells and lymphocytes
which may cause the overlying mucosa to • Disease progresses in a proximal-to-distal
ulcerate fashion, with more severe disease in the
• Larger lesions are better seen on CT, but jejunum than in the ileum
there may be bowel wall thickening and • Jejunal atrophy causes secondary
distortion hypertrophy of the ileum, resulting in a
• Occlusion or invasion of the small bowel reversal of fold patterns
vessels may give rise to ischaemic changes • Patients cannot tolerate gluten, a protein
• If there is fibrosis of the tissues, there may commonly found in wheat, rye and barley
be a kink in the small bowel, known as a • Most patients tolerate oats, but should be
‘hairpin turn’, on CT monitored closely
• Gadolinium-enhanced T1 fat-suppressed • Clinical features
MRI may show these lesions in the wall of –– Gastrointestinal symptoms include
the small bowel diarrhoea, steatorrhoea and flatulence
–– Extraintestinal features include
Metastases anaemia, bleeding, osteopenia,
• Common sites of metastases are the liver, neurological symptoms (resulting
lymph nodes, lung and bone from hypocalcaemia), skin disorders
• 2% of tumours < 1 cm metastasise; 85% of (including dermatitis herpetiformis),
tumours > 2 cm metastasise hormonal disorders (including
• Rare in carcinoid of the appendix; amenorrhoea, delayed menarche,
commoner in carcinoid of the ileum infertility and impotence)
Adenocarcinoma • Complications
–– Ulcerative jejunoileitis: multiple ulcers
• Solitary that are usually fatal
• Located in proximal small bowel –– Peptic duodenitis
• Imaging features include ulceration, –– Intussusception
annular constriction with shouldering and –– Cavitary mesenteric lymph node
marked desmoplastic reaction, and polyps syndrome
Coeliac disease –– Hyposplenism and splenic atrophy (in
30–50% of cases)
• Chronic disease that interferes with the
–– Small bowel lymphoma
digestion and absorption of nutrients

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176 Gastrointestinal system

–– Adenocarcinoma of the small bowel, positive with periodic acid–Schiff (not


rectum or stomach pathognomonic)
–– Squamous cell cancer of the • Almost universally fatal after 1 year in
oesophagus patients who are not diagnosed and who
–– Endocrine diseases, seen in up to do not receive therapy with antibiotics, e.g.
10% of cases, including autoimmune ceftriaxone followed by oral trimethoprim–
thyroiditis and Sjögren’s syndrome sulfamethoxazole
• Management is by a gluten-free diet
Imaging features
Imaging features Barium study
• Investigated radiologically by a small • Minimal bowel dilatation
bowel series or a small bowel enema • No ulceration
(best imaging test and suggestive of the • Thickening of duodenal or jejunal folds
diagnosis in 75% of cases)
• Upper endoscopy with duodenal biopsy is CT
considered the criterion standard • Thickening of the small bowel wall
• Jejunoileal fold reversal is diagnostic; in thickening and bulky hypodense
addition at least three of the following abdominal nodes
need to be seen • Associated systemic or articular disease is
–– Fold thickening in favour of the diagnosis
–– Jejunal atrophy: three folds or fewer per Radiation enteritis
2.5 cm in the first loop of the jejunum,
with a concomitant increase in the • Radiation injury, either in an acute form or
number of folds in the ileum, are highly chronic form
diagnostic • Injury follows a dose 45–60 Gy for the
–– Mosaic pattern of mucosa is maintained small intestine and colon and 55 80 Gy for
in the jejunum in 10% of cases the rectum
–– Dilatation of bowel • Acute injury is a function of the fraction
–– Barium flocculation: a sign of of the dose, field size and the type of
hypersecretion and malabsorption, radiation
seen in 20–30% of cases • Chronic injury is a function of the total
–– Jejunisation of the ileum (hypertrophy dose of radiation
of ileum with thickening of folds) • Injury results in an inability to repopulate
• Gastric metaplasia in the duodenum may the surface epithelium as well as in
give rise to mucosal nodules (the ‘bubbly collagen deposition and fibrosis, leading
bulb’ sign) to bowel wall thickening, obliterative
endarteritis and neural injury, all of which
Whipple’s disease lead to impaired mucosal and motor
• Systemic disease caused by infiltration of function
tissues by the Gram-positive bacterium • Acute injury occurs within 6–24 months of
Tropheryma whippelii the initial radiation therapy
• Disordered host response • Risk factors include hypertension, diabetes
• Seen in immunocompromised patients mellitus, atherosclerosis, adhesions,
• Males predominate, roughly in a ratio of peritonitis, previous abdominal surgery,
8:1 or 9:1 pelvic inflammatory disease and
• Affects the small bowel, joints, central combination radiation–chemotherapy
nervous system and cardiovascular system Radiation injury of the
• Clinical presentation: muscle wasting,
arthralgia, arthritis, fever, diarrhoea,
stomach
pericarditis • Usually occur with doses of 45–60 Gy given
• Diagnosis is by macrophages that stain over 5 weeks

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Large and small bowel 177

• Acute phase is seen 2–8 weeks after –– Discrete masses are not observed
treatment –– Mesenteric changes include increased
• Chronic phase occurs 4 weeks to 7 months attenuation and thickening of the
after treatment mesentery
• Two main types seen, prepyloric or pyloric –– The changes are non-specific, but
ulceration and antral narrowing correlation with history and radiation
• Prepyloric or pyloric ulcers ports are important
–– More typical of the acute phase and
indistinguishable from benign ulcers Radiation injury of the colon
• Antral narrowing and rectum
–– Seen with chronic injury • The colon and rectum are commonly
–– Appears as narrowing of the antrum affected by irradiation to the pelvis in
and pylorus without ulceration gynaecological malignancies
–– The mucosa is irregular and may mimic • Time interval to appearance of changes is
gastric cancer similar to that seen with the small intestine
–– Similar to linitis with little peristalsis • Acute radiation colitis presents non-
–– CT shows non-specific gastric wall specifically as mucosal irregularity and
thickening, loss or widening of the submucosal thickening, resulting in
gastric folds and perigastric fat cobblestone pattern on barium enemas
stranding • Increase in the presacral space and
–– Associated duodenal injury may also be thickening or effacement of the haustral
present folds or rectal valves of Houston may be
seen
Radiation injury of the small • The chronic form manifests itself as
intestine strictures that may be long or short and are
• Although radiosensitive, because of its generally tapered
relative mobility the small bowel is less • Fistulation can be seen
commonly affected by radiation changes • Bowel obstruction may be seen
• Injury occurs with doses above 50–Gy over • Colonic hypoplasia is a rare complication
a 6-week period in children
• Three phases are seen
• Acute phase (during and immediately after
Imaging features
therapy) CT
–– Hyperaemia, oedema and • Increased attenuation of the prerectal fat
inflammation of the mucosa and fibrous tissue, giving a halo effect
–– Crypt abscess and sloughing of the • Increase in the presacral space to > 1 cm in
mucosa the anteroposterior diameter
• Subacute phase (2–12 months after MRI
therapy) • Early increase in signal on T2WI of the
–– Obliterative endarteritis of the small submucosa, with a normal low signal outer
vessels wall of the rectum
• Chronic phase (> 1 year after therapy) • Further injury results in the outer wall
–– Progressive fibrosis and serosal changes also becoming high signal, with loss of
with adhesions and fistula formation differentiation of the mucosa and muscle
–– In order to evaluate small bowel
changes, good distension and Graft-versus-host disease
opacification of the bowel is needed • Loss of haustrations
–– Loops of bowel that are thickened and • Effacement of small bowel with tubular
matted with a serpentine appearance appearance
are indicative of irradiated bowel • Fold thickening

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178 Gastrointestinal system

• Persistent coating of bowel mucosa by Thick folds seen on imaging


barium • Crohn’s disease
• Reduced transit time • Ischaemia
• Lymphoma
Causes of bowel obstruction • Radiotherapy
Small bowel obstruction • Zollinger–Ellison syndrome
• Adhesions • Extensive small bowel resection
• Hernias • Amyloidosis
• Intussusception
• Crohn’s disease
Appendicitis
• Gallstone ileus • Inflammation of the appendix
• Ileus • Caused by luminal obstruction from
• Tumour faecolith (in 33% of cases), lymphoid
• Foreign body (e.g. smuggled cocaine) hyperplasia, foreign body, parasites or a
primary tumour
Large bowel obstruction • Presents clinically with mild periumbilical
• Faeces pain that then localises to the right iliac
• Sigmoid volvulus or caecal volvulus fossa, anorexia, nausea, vomiting and low-
• Tumour grade fever
• Diverticulitis • Consider perforation if the temperature is
> 38.3°C)
Symptoms
• Complications include perforation,
• Anorexia, nausea, vomiting with relief
abscess formation, peritonitis, sepsis,
• Colicky abdominal pain with distension
infertility and bowel obstruction
• Constipation if distal obstruction
• Active, ‘tinkling’ bowel sounds Imaging features
Imaging features AXR
• Abnormalities seen in < 50% of cases
AXR • May reveal a faecolith, a thickened
• Small bowel obstruction
caecum, small bowel obstruction or
–– Central gas shadows
extraluminal gas
–– Collapsed large bowel
• Large bowel obstruction Ultrasound
–– Gas proximal to the obstruction, • Graded compression ultrasound is 85%
collapsed bowel distally sensitive and 92% specific for appendicitis
• Mural wall thickness 2 mm or more
Management
CT
• Strangulation and large bowel obstruction
• 87–100% sensitive and 89–98% specific for
→ urgent surgery (within 1 hour)
appendicitis
• Paralytic ileus and incomplete small
• Abnormal appendix (> 7 mm in diameter)
bowel obstruction can be managed
with periappendicular inflammation
conservatively initially

Causes of dilated small bowel Meckel’s diverticulum


• Persistence of the omphalomesenteric
Normal folds seen on imaging duct on the antimesenteric border of the
• Mechanical obstruction ileum
• Ileus • Commonest congenital abnormality of the
• Scleroderma gastrointestinal tract
• Iatrogenic • Contains ectopic mucosa (gastric,
• Coeliac disease pancreatic and colonic) in 50% of cases
• Tropical sprue • The ‘rule of twos’: 2% of the population, 2
• Dermatitis herpetiformis inches long, 2 feet from the terminal ileum,

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Large and small bowel 179

symptomatic before the age of 2 years Ultrasound


• Asymptomatic in 20–40% of cases • Mesenteric Doppler ultrasound is a non-
• Complications include bleeding, invasive method of analysing vessel flow
diverticulitis, bowel obstruction
secondary to intussusception, malignancy Epiploic appendagitis
(carcinoma, carcinoid or sarcoma) and • Rare condition
chronic abdominal pain • Inflammation of the epiploic appendage
• Cause
Imaging features –– Primary epiploic appendagitis is caused
Technetium-99m pertechnetate scan by torsion or venous thrombosis
• 85% sensitive and > 95% specific –– Secondary epiploic appendagitis is
• Sensitivity drops after adolescence caused by inflammation of an adjacent
because adults are less likely than children organ
to have gastric mucosa in the Meckel’s • Presents clinical with an abrupt onset of
diverticulum localised abdominal pain (in the right
Angiogram lower quadrant in 50% of cases); there is a
• Identification of vitelline artery is palpable mass in 10–30% of cases
pathognomonic • Management is conservative

Chronic mesenteric ischaemia Imaging features


• Cause by diffuse atherosclerotic disease Ultrasound
in > 95% of cases • Solid, hyperechoic, non-compressible
• All three major mesenteric arteries usually ovoid mass with a hypoechoic margin
involved CT
• Clinical presentation is with weight loss, • Pericolic, oval-shaped, pedunculated mass
postprandial epigastric or periumbilical 1–4 cm in diameter
pain, a fear of eating and a history of
vascular disease involving other risk Causes of small bowel
factors strictures
• Risk factors include smoking, • Adhesions
hypertension, diabetes mellitus and • Tumours, e.g. lymphoma, carcinoid,
hypercholesterolaemia carcinoma, sarcoma, metastases
• Medical treatment involves primary • Crohn’s disease
prevention and anticoagulation • Radiation enteritis
• Surgical treatment • Ischaemia
–– Transaortic endarterectomy of the • Enteric-coated potassium tablets
celiac or superior mesenteric artery
–– Retrograde bypass from the external Causes of multiple small bowel
iliac artery nodules
–– Anterograde bypass, which provides the • Inflammatory causes
best orientation of the graft to the aorta –– Nodular lymphoid hyperplasia
–– Mesenteric artery reimplantation, –– Crohn’s disease
which has been performed but is not • Infiltrative causes
widely recommended because of the –– Whipple’s disease
technical difficulties of the procedure –– Waldenström’s macroglobulinaemia
Imaging features –– Mastocytosis
• Neoplasia
Arteriogram –– Lymphoma
• Arteriography is the criterion standard and –– Polyposis
will show occlusion and collateral flow –– Metastases

Ch-03.indd 179 8/12/2010 12:18:32 PM


180 Gastrointestinal system

• Infective causes mesenteric artery and the inferior


–– Typhoid mesenteric artery at the splenic flexure
–– Yersinia infection –– Sudeck’s point: the anastomotic plexus
between the inferior mesenteric artery
Causes of lesions in the and the hypogastric supply at the
terminal ileum rectosigmoid junction
• Inflammatory causes –– The right colon is involved in 30% of
–– Crohn’s disease cases, the left colon in 45–90%
–– Ulcerative colitis
Imaging features
–– Radiation enteritis
• Infective causes AXR
–– Tuberculosis • Segmental ‘thumb-printing’
–– Yersinia infection Barium enema
–– Actinomycosis • Thickening of the bowel wall, loss of
–– Histoplasmosis haustrations and ‘thumb-printing’
• Neoplasia
–– Lymphoma CT
–– Carcinoid • Segmental thickening
–– Metastases • Enhancement of the colon wall
• Ischaemia • Portal or mesenteric air
• Thrombus in the superior mesenteric
Causes of small bowel artery or the superior mesenteric vein
aphthoid ulceration • Haemorrhage
• Crohn’s disease Angiogram
• Polyarteritis nodosa • Mild acceleration of arteriovenous transit
• Yersinia enterocolitis time
• Small tortuous ecstatic draining veins
Colon Pseudomembranous colitis
Ischaemic colitis • Results from a disturbance of the normal
bacterial flora of the colon, colonisation
• Non–occlusive ischaemic disease that
with Clostridium difficile and release of
reduces blood flow to 20% of normal
toxins that cause mucosal inflammation
• Acute and rapid onset
and damage
• Reperfusion injury is likely when blood
• Clostridium difficile is a Gram-positive,
flow is re-established
anaerobic, spore-forming bacillus
• Causes include bowel obstruction,
• Pathogenic strains of Clostridium difficile
thrombosis and trauma; it may also be
produce two distinct toxins: toxin A is an
idiopathic
enterotoxin and toxin B is a cytotoxin
• Presents clinically with an abrupt onset of
• Clostridium difficile-associated diarrhoea
lower abdominal pain, rectal bleeding and
can be a serious condition with a mortality
diarrhoea
rate of up to 25% in elderly, frail patients
• Prognosis
• Risk factors include advanced age (> 60
–– Transient ischaemia resolves in 1–3
years) and hospitalisation
months (76% of cases)
• 20% of hospital patients Clostridium
–– Stricturing ischaemia leads to
difficile during their hospital stay, and
incomplete and delayed healing
more than 30% of these patients develop
–– Overall mortality is 10–30%
diarrhoea
• Affected segments
• The commonest antibiotics implicated
–– Griffith’s point (80% of cases): the
in Clostridium difficile colitis include
junction between the superior

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Large and small bowel 181

cephalosporins (especially second- and observed in the colon and most often
third-generation agents), ampicillin, affect the rectosigmoid region
amoxicillin and clindamycin • Associated with a low-fibre diet,
• Disease distribution constipation and obesity
–– Pancolitis in 50% of cases • Affect 70–80% of people aged 80 years
–– Right-sided colitis in 27% of cases
–– Isolated rectosigmoid disease in 12% of
Diverticulitis (inflammation of one
cases (the rectosigmoid area is spared or more diverticula)
in 67% of cases) • Faecal material or undigested food
• Stool cytotoxin test has high sensitivity particles may collect in a diverticulum
(94–100%) and high specificity (99%), and • Obstruction of the neck of the
is the test of choice diverticulum results in distension of the
• Endoscopy may demonstrate the presence pouch secondary to mucous secretion and
of raised, yellowish–white plaques overgrowth of normal colonic bacteria
2–10 mm in diameter (pseudomembranes) • The thin-walled diverticulum, consisting
overlying an erythematous and solely of mucosa, is susceptible to
oedematous mucosa vascular compromise and subsequent
• Medical care: microperforation or macroperforation
–– Most patients recover, even without • 10–25% of people with colonic diverticular
specific therapy disease develop diverticulitis
–– The decision to treat Clostridium • Once the inflamed mucosa has healed,
difficile infection and the type of colonoscopy or contrast enema is
therapy may depend on the severity of important to rule out a malignancy
the disease masquerading as diverticular
–– No treatment is necessary for bowel thickening, phlegmon or
asymptomatic carriers postinflammatory stricture
–– Cessation of the causative antibiotic is • Complications
essential when possible –– Abscess
–– Patients with more severe diarrhoea or –– Intestinal fistula (in 14% of cases)
colitis should receive antibiotic therapy –– Intestinal perforation
directed at Clostridium difficile, e.g. –– Intestinal obstruction
metronidazole or vancomycin –– Peritonitis
• Complications include fulminant colitis –– Sepsis and septic shock
(in 3% of cases), toxic megacolon, colonic –– Bleeding (more common in
perforation and peritonitis diverticulosis than diverticulitis)
• Persistent diarrhoea may be debilitating
Imaging features
and can last for several weeks
CT
Imaging features • Modality of choice
CT • Pericolic fat stranding
• Non-specific mural thickening with bowel • Colonic diverticula and thickening of the
dilatation and a paucity of pericolonic bowel wall
inflammation • Soft tissue inflammatory masses or
phlegmon
Diverticular disease • Abscesses or fistula formation may also be
• Diverticula are small mucosal herniations seen
protruding through the intestinal layers
and smooth muscle along the natural Colonic polyps
openings created by the vasa recta or • Slow-growing overgrowths of the colonic
nutrient vessels in the wall of the colon mucosa that carry a small risk (< 1%) of
• Diverticula can occur anywhere in the becoming malignant
gastrointestinal tract but are usually • Can occur as part of inherited polyposis

Ch-03.indd 181 8/12/2010 12:18:32 PM


182 Gastrointestinal system

syndromes, in which their number Imaging features


is greater and the risk for malignant
Air-contrast barium enema
progression is much greater
• Detects larger polyps (> 1 cm in diameter)
• 30% of middle-aged and elderly people
but can miss smaller ones
have colonic polyps
• Low false-positive rate
• Management
–– Colonoscopic polypectomy for solitary CT virtual colonoscopy
pedunculated polyp • Detects more than 80% of large polyps
–– Repeat colonoscopy in 5 years after Colonoscopy
complete removal of a low-risk • Preferred test to detect colonic polyps,
adenomatous polyp obtain biopsies, and/or perform
–– Repeat colonoscopy in 3 years if the endoscopic resection
polyp has high-risk features • 80–90% sensitivity for large polyps
Types of polyp Colorectal carcinoma
• Hyperplastic polyps
• Commonest cancer of the GI tract
–– Account for 90% of all polyps
• Risk factors
–– Benign protrusions
–– Family history or personal history of
–– Usually < 0.5 cm in diameter
colonic adenoma (5% tubular, 30–40%
–– Most commonly occur in the
villous)
rectosigmoid area
–– Carcinoma
–– Possess some malignant potential in
–– Inflammatory bowel disease
the setting of the hyperplastic polyposis
–– Prominent lymphoid follicular pattern
syndrome
–– Pelvic irradiation
• Adenomas
–– Ureterosigmoidostomy
–– Account for 10% of polyps
–– Diet low in fibre and high in fat and
–– Most (approximately 90%) are small,
animal protein
usually < 1.5 cm in diameter, and have a
–– Obesity
very small potential for malignancy
–– Asbestos exposure
–– The remaining 10% of adenomas are
–– Lynch’s syndrome (hereditary non-
> 1.5 cm in diameter and have about
polyposis colorectal cancer syndrome)
a 10% chance of containing invasive
• Staging (modified Dukes’ staging)
cancer
–– Stage A: limited to the mucosa
–– Traditionally divided into three
–– Stage B: involvement of the muscularis
subtypes: tubular, tubulovillous and
propria
villous
–– Stage C: lymph node metastases
–– Tubular adenomas are the commonest
–– Stage D: distant metastases
subtype
• Location
–– Rectum (in 30% of cases)
Polyposis syndromes
–– Sigmoid (in 30% of cases)
–– Descending colon (in 10% of cases)
• Hereditary conditions, including –– Transverse colon (in 10% of cases)
familial adenomatous polyposis, –– Ascending colon (in 10% of cases)
Gardner’s syndrome, Turcot’s syndrome, –– Caecum (in 10% of cases)
Peutz–Jeghers syndrome, Cowden’s • Metastasises to the liver (in 75% of cases),
disease, familial juvenile polyposis and the adrenal glands (in 10% of cases), the
hyperplastic polyposis lung (in 5–50% of cases), the ovary (in 5%
• Complications include bleeding, of cases), bone (in 5% of cases) and the
diarrhoea, intestinal obstruction and brain (in 5% of cases)
progression to cancer • 5-year survival depends on disease stage
• Colonic resection remains the only –– Stage A: 85%
feasible treatment option –– Stage B: 70%

Ch-03.indd 182 8/12/2010 12:18:33 PM


Large and small bowel 183

–– Stage C: 33% –– Colitis and enteritis


–– Stage D: 5% –– Collagen disorders
• Peritoneal involvement is present in 25% –– Leukaemia
of cases and is a poor prognostic sign –– Corticosteroids and other
• Recurrence occurs in 33% of cases: 50% immunosuppressive therapy
recur within 1 year and of these 60% recur
locally Causes of colonic aphthoid
• 5% of recurrent tumours are endoluminal ulceration
and 50% are extraluminal • Crohn’s disease
• Preoperative radiotherapy reduces local • Ischaemia
recurrence • Amoebic colitis
• Complications include bowel obstruction, • Yersinia enterocolitis
perforation, intussusception, abscess • Behçet’s disease
formation and fistula formation
Causes of anterior indentation
Sigmoid volvulus of the rectosigmoid junction
• Sigmoid colon twists on the mesenteric • Tumours
axis • Haematoma
• Seen as greatly distended, paralysed loop • Hydatid
of bowel with fluid–fluid levels mainly on • Abscess
the left side, extending to the diaphragm • Ascites
• Distinct midline crease (‘coffee bean’ sign) • Endometriosis
• Management is with a flatus tube • Previous surgery
Causes of colonic strictures Causes of a widened retrorectal
• Neoplasia space (> 1.5 cm at the S3–S5
–– Carcinoma
–– Lymphoma
level)
• Inflammatory causes • Normal variant
–– Ulcerative colitis • Neoplasia
–– Crohn’s disease –– Rectal carcinoma
–– Pericolic abscess –– Rectal metastases
–– Radiotherapy –– Sacral tumours
• Infective causes • Inflammatory causes
–– Tuberculosis –– Crohn’s disease
–– Amoeboma –– Ulcerative colitis
–– Schistosomiasis –– Abscess
–– Lymphogranuloma venereum –– Radiotherapy
• Cathartic colon –– Diverticulitis
• Ischaemia • Pelvic lipoma
• Extrinsic masses • Anterior sacral meningocoele
–– Inflammatory masses • Enteric duplication cysts
–– Tumours Crohn’s disease
–– Endometriosis
• Uncertain aetiology
Causes of pneumatosis • Pathologically the disease affects the bowel
intestinalis in a transmural pattern and can affect any
part of the GI tract from the mouth to the
• Primary pneumatosis intestinalis accounts
anus
for 15% of cases
• Early mucosal involvement consists of
• Secondary pneumatosis intestinalis
longitudinal and transverse aphthous
accounts for 85% of cases
ulcers
–– Necrotising enterocolitis

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184 Gastrointestinal system

• Progression to fissuring and fistulation • Most cases occur in the rectum and travel
follows, eventually resulting in proximally
communication with adjacent segments of • May involve the terminal ileum (back-
bowel wash ileitis caused by an incompetent
• Risk factors include a positive family ileocaecal valve)
history, smoking and oral contraceptives • Risk of colorectal cancer increases by 0.5 to
• Complications 1% per year after 10 years of the disease
–– Sinus tracks (in 15% of cases) • Toxic megacolon occurs in 2% of cases
–– Bowel obstruction (in 20% of cases)
–– Fistula formation (in 50% of cases)
Imaging features
–– Malignant transformation; cancer is a AXR
leading cause of death (the majority of • Colonic dilatation
malignancies are adenocarcinomas) • Dilatation >9 cm in the caecum and 10 cm
in the transverse colon means that there
Imaging features is a toxic megacolon, which carries a high
AXR chance of perforation and is a surgical
• Main role is to assess for obstruction or emergency
perforation • Colon may demonstrate ‘thumb-printing’
Contrast studies Barium enema
• Used to diagnose and differentiate disease • Usually performed in the quiescent phase
• Characterised by aphthoid ulcers, which • Left colon is usually involved and the
are separated by normal bowel but which disease is segmental but skip lesions are
can become more confluent and give rise not seen
to serpigenous and linear ulcers affecting • Mucosal oedema is seen with a fine
the mesenteric borders granular pattern
• Cobblestoning is seen with transmural • In more advanced disease, ulceration is
inflammation, giving a reticular pattern seen, characteristically ‘rose thorn’ ulcers
• As transmural inflammation increases, • Other types of ulcers include ‘collar
there is eventual narrowing of the lumen, button’ ulcers (with undermining of the
giving rise to the ‘string sign’ edges) and longitudinal ulcers
CT • Confluent ulceration is seen as a coarse,
• Used in the context of an acute granular appearance
abdomen in Crohn’s disease to assess for • Symmetrical haustral thickening resulting
complications and for surgical planning from oedema produces the impression of
and postoperative management ‘thumb printing’
• Ulceration and mucosal oedema can be • Pseudopolyps are seen in severe cases and
seen on thin-section CT, although contrast result from normal mucosa adjacent to
examinations are better for this areas of ulceration
• Associated mesenteric stranding is not • Recurrent inflammation leads to
reliable in evaluating active disease narrowing and shortening of the colon
because it is also found during periods of • Rectal narrowing is seen as an increase in
remission the presacral space
• Identification of abscess and fistula • Benign strictures occur in 10% of patients
formation is useful for management with long-standing disease
CT
Ulcerative colitis • Now become the primary imaging
• Involves the mucosa with formation of modality
crypt abscesses • Circumferential, symmetrical thickening
• Severe cases affect the other layers of the of the bowel well, with thickened haustral
large bowel wall folds

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Large and small bowel 185

• Target sign (alternating high and low Ultrasound


attenuation of the colonic wall, caused by • Used to diagnose ulcerative colitis but is
oedema) operator-dependent
• Submucosal fat deposition is seen in • Thickening of the bowel wall is seen as
ulcerative colitis more often than Crohn’s a hypoechoic wall, but it may become
disease inhomogeneous as a result of fatty
• Main use of CT is to identify the deposition
complications of ulcerative colitis, • Haustral folds are lost and there is reduced
including perforation, abscess formation, peristalsis and compressibility
obstruction secondary to strictures and • Ultrasound can differentiate between
malignant change, as well as for surgical Crohn’s disease and ulcerative colitis:
planning and postoperative imaging Crohn’s disease is a transmural disease
whereas ulcerative colitis affects the
superficial layers

Ch-03.indd 185 8/12/2010 12:18:33 PM


Chapter 4
Genitourinary system,
adrenal gland, obstetrics
and gynaecology,
and breast

4.1 Renal system


4.2 Urinary bladder
4.3 Testis
4.4 Prostate gland
4.5 Adrenal gland
4.6 Uterus
4.7 Ovary
4.8 Breast

Ch-04.indd 187 8/9/2010 1:42:51 PM


4.1 Renal system

Anatomy • 99m
Tc-DTPA (diethylene triamine penta-
acetic acid) is used to assess renal blood
• The kidneys develop from the pronephros, flow and urinary excretion
mesonephros and metanephros of which • 99m
Tc-MAG3 (mercapto acetyl tri-glycine)
the pronephros and mesonephros regress; is used to assess renal function
some segments of the mesonephros • 99m
Tc-DMSA (dimercaptosuccinic acid)
develop into segments of the genital is used to depict focal renal parenchymal
system lesions
• The ureteric bud develops as an outgrowth • 99m
Tc-MAG3 is the agent of choice for
from the mesonephric duct proximal to urinary tract obstruction, but DTPA is an
the cloacal entry. The ureter, renal pelvis, alternative agent
calyces and collecting tubules develop • 99mTc-sulphur colloid is the agent of choice

from the ureteric bud for demonstrating vesicoureteric reflux


• The metanephric blastema arises from
the caudal part of the nephrogenic cord.
Physical contact with the ureteric bud
Autosomal dominant
induces its development. The excretory polycystic kidney disease
part of the kidney develops from the
• Hereditary disorder in which there is
ureteric bud
variable enlargement of the kidneys with
• The kidneys originally develop in the
multiple cysts
upper sacral region but migrate cranially
• Progressive renal failure and hypertension
receiving successively higher branches
• Usually presents in the third or fourth
from the aorta. The final adult position
decade of life
is reached by 8 weeks of gestation. Renal
maturation continues until the age of 5 Clinical symptoms and signs
years and hence the kidneys are prone to • Flank pain, haematuria,
scarring until this age • Pyelonephritis, urolithiasis, hypertension,
renal failure
Imaging Pathology
Ultrasound • Cysts develop anywhere along the nephron
and are variable in size
• Normal renal cortex is hypoechoic
• Intracystic haemorrhage may occur
compared with normal liver
• Intracystic calcifications may also be seen
• Renal medulla is hypoechoic compared
• Strong penetrance (nearly all who inherit
with cortex
the gene develop the disease)
• Arcuate arteries may be seen as punctate
• Extrarenal abnormalities are common
echogenic foci at the corticomedullary
• Liver cysts occur in up to 50% of patients
junction and should not be mistaken for a
• Berry aneurysms occur in up to 15% of
calculus
patients
• Renal sinus is hyperechoic, mainly due to
the presence of fat Diagnostic criteria
• Two or more cysts in both kidneys
Radionuclide imaging detected with ultrasound in at-risk patients
• Technetium-99m is the isotope most < 30 years
commonly used • Two or more cysts in each kidney detected
• High gamma photon energy (140 keV) with ultrasound in patients aged 30–60
• Short half-life of 6 hours with even shorter years
biological half-life due to renal excretion • Patients > 60 years must have at least four
• Iodine-123 or iodine-131 are the other detectable cysts in each kidney
commonly used isotopes

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Renal system 189

Nephrocalcinosis Renal sinus cysts


• Radiologically detectable diffuse calcium (parapelvic cysts)
deposition within the renal substance • Benign extraparenchymal cysts located
• Mechanism of calcium deposition can be within the renal sinus
metastatic (in morphologically normal • Most likely lymphatic in origin
kidneys), dystrophic (in injured tissue) or • May be uni- or multilocular
due to urine stasis (as in medullary sponge • May be bilateral
kidney) • Differential diagnoses include
Causes pelviureteric junction obstruction,
• Primary and secondary multicystic dysplastic kidney
hyperparathyroidism Imaging features
• Metastatic carcinoma to bone
• Hypercalcaemia of malignancy Ultrasound
• Prolonged immobilisation • Features of a simple cyst: anechoic, thin
• Sarcoidosis wall, post acoustic enhancement
• Milk-alkali syndrome • No communication with the pelvicalyceal
• Hypervitaminosis D system
• Renal tubular acidosis • Intravenous urogram
• Medullary sponge kidney • Soft tissue density in the renal sinus that
• Hyperoxaluria causes focal displacement and smooth
• Bartter’s syndrome effacement of the adjacent pelvicalyceal
• Prolonged furosemide (frusemide) system
administration, e.g. in premature infants CT
• Nephrotoxic drugs • Homogeneous, near water density and
• Papillary necrosis non-enhancing
• Displacement of renal pelvis and calyces
Imaging features
• Surrounding halo of renal sinus fat is a
• Extent of parenchymal calcification is characteristic feature
variable • MRI: water signal intensity lesion (low T1
• In hypercalcaemic states, only a few and high T2 signal) surrounded by high-
scattered punctate densities in the signal renal sinus fat
medullary portions of the kidneys may be
seen
• In renal tubular acidosis, often very dense Acquired cystic kidney
and extensive calcification of the medulla disease
is seen
• Development of multiple renal cysts
in patients with chronic renal failure
Medullary sponge kidney (uraemia) but without a history of
• Dysplastic cystic dilatation of papillary and inherited renal cystic disease
medullary portions of the collecting ducts • 90% of patients with end-stage renal
• Symptoms can occur secondary to stasis disease, > 5–10 years of dialysis and 8–13%
but condition is often asymptomatic patients not on dialysis
• Bilateral in 75% • Usually asymptomatic
• Medulla appears echogenic on ultrasound • Diagnosis based on detection of 3–5 cysts
due to the medullary calcification in each kidney in a patient with chronic
• Intravenous urogram: ‘striated’ renal failure not caused by an inherited
nephrogram phase, ‘bunch of flowers’ cystic disease
appearance may also be seen • Affected kidneys are usually small, but due
to progression, nephromegaly eventually
develops

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190 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Haemorrhagic cysts occur in 50% of Subcapsular haematoma


patients with acquired cystic kidney (limited by capsule)
disease • Recent haemorrhage is of higher
• Renal cell carcinoma develops in up to 7% attenuation than adjacent parenchyma
of patients • Flattening of kidney
• Subcapsular and perinephric haematomas • Elevation of capsule
can occur due to rupture of haemorrhagic • Medial displacement of collecting system
cysts • May eventually calcify
• Large chronic subcapsular haematoma
Imaging features can cause hypertension (Page kidney)
CT
Perinephric haematoma (involves fat
• High attenuation values may be seen
between capsule and fascia)
within cysts as a result of haemorrhage
• Can simulate a subcapsular haematoma,
• Lack of enhancement post-contrast
but usually extends below the kidney
Ultrasound margin
• Multiple simple cysts • If CT reveals no cause for haemorrhage
MRI angiography is recommended
• Haemorrhagic cysts display high signal on
T1WI and T2WI in the subacute phase Renal infarction
• No enhancement following gadolinium
Causes
• Renal artery thrombosis or embolism
Renal haemorrhage • Vasculitis, e.g. polyarteritis nodosa
• May be suburothelial, intraparenchymal, • Trauma
subcapsular, perinephric, pararenal or in • Sickle cell disease
the renal sinus • Aortic dissection
Causes CT features
• Trauma • Depends upon extent and age of infarct
• Shock wave lithotripsy
Global renal infarction (main renal artery
• Spontaneous: anticoagulation, blood
occlusion)
dyscrasias (usual cause for suburothelial
• Non-enhancing kidney with high-density
and renal sinus haemorrhage)
cortical rim from perfusion by collateral
• Renal infarction
vessels
• Polyarteritis nodosa
• Renal aneurysms and arteriovenous Large branch occlusion
malformations • Sharply demarcated hypodense wedge-
• Tumours: renal cell carcinoma shaped defect with the base of the wedge
(commonest cause of spontaneous on the renal capsule and apex towards the
subcapsular and perinephric renal hilum
haemorrhage) • Cortical rim may enhance
• Renal vein thrombosis Small vessel occlusion
• Rupture of a haemorrhagic cyst (emboli, vasculitis, sickle cell)
CT features • Multiple, often bilateral, focal infarcts
• Multiple ‘slit-like’ areas of low attenuation
Suburothelial haemorrhage (sickle cell)
• Thickened wall of renal pelvis and upper • Main differential diagnosis is acute
ureter by high attenuation blood pyelonephritis (cortical rim is not usually
seen with pyelonephritis)

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Renal system 191

Renal artery stenosis • Septic abortion


• Severe trauma with shock
• Accounts for 1–5% of hypertension in • Transfusion reaction
young adults • Severe dehydration
• Causes: • Haemolytic–uremic syndrome
–– Atherosclerosis (up to 90%) • Acute aortic dissection
–– Fibromuscular dysplasia • Toxins including snake venom
• Usually bilateral
Imaging features
CT CT features
• Prolonged corticomedullary • Acute phase: zone of unenhanced
differentiation in comparison with normal cortex between enhancing medulla and
kidney on a conventional dynamic CT is subcapsular cortical rim
suggestive of significant stenosis • Chronic phase: small smooth kidneys. The
• Multidetector CT angiography has cortex may calcify
improved spatial resolution compared
with magnetic resonance angiography and Renal vein thrombosis
can be used to evaluate renal vascularity
when imaged in the arterial phase Causes
MRI • Extrinsic occlusion by an adjacent
• Gadolinium-enhanced magnetic neoplasm
resonance angiography has a high • Direct extension of a renal or adrenal
sensitivity for detection of proximal renal carcinoma
artery stenosis • Primary renal disease: membranous
glomerulonephritis (most common in
Catheter angiography adults) also systemic lupus erythematosus
• Gold standard test and amyloidosis
• In renal artery stenosis secondary to • Secondary renal vein occlusion from
atherosclerosis the ostium or proximal thrombosis of the inferior vena cava
2 cm of the renal artery is affected • In infants: sickle cell disease,
• In fibromuscular dysplasia the mid/ haemoconcentration states such
distal main renal artery is involved and as diarrhoea and sepsis, hypoxia in
characteristically has a ‘string of beads’ cyanotic congenital heart disease and
appearance hypercoagulable states and maternal
• Cannot be used to assess haemodynamic history of diabetes
significance of lesion • Clinical consequences depend on the
Colour Doppler ultrasound rapidity of development of obstruction and
• Peak systolic velocity > 150 cm/sec development of collateral circulation
• Post stenotic spectral broadening and/or • If completely occluded acutely, renal
flow reversal infarction occurs, eventually causing a
• Parvus tardus waveform of interlobar small, smooth non-functioning kidney
artery Imaging features
• Acceleration time > 0.07 sec
Intravenous urogram
• Large and smooth kidney
Acute cortical necrosis • Variable contrast excretion
• Ischaemic necrosis of the renal cortex with • Attenuation of the collecting system by
sparing of the medulla swollen parenchyma
• Increasingly dense nephrogram in some
Causes cases
• Haemorrhage (classically in third trimester • Medullary striations on intravenous
of pregnancy) urogram and CT have been described

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192 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Cortical rim sign may be seen as in acute Acute bacterial


arterial infarction
• Varices can cause indentation of the renal pyelonephritis
pelvis and ureter • Imaging is indicated if severe and/or
Doppler ultrasound refractory to treatment
• Loss of normal low-resistance pattern of • Aim is to determine complications and
renal artery waveform diagnose any predisposing abnormalities
• Visualisation of thrombus within the renal Imaging features
vein
Intravenous urogram
CT • Usually normal
• Prolonged corticomedullary • Abnormal in 30%, but with non-specific
differentiation features
• Focal areas of non-enhancement where • Enlargement of the kidney, delayed or
infarction has occurred poor excretion of contrast and a striated
• Retroperitoneal haemorrhage nephrogram may be seen
• Expansion and enlargement of renal vein • Hydroureteronephrosis can occur
with low-attenuation thrombus secondary to the effect of bacterial
• Fine linear densities radiating from endotoxins
the kidney to the perirenal space are
CT
characteristic and enhance on contrast
• ‘Striated nephrogram’: discrete rays of
administration
alternating attenuation extending to the
• Enlargement of the kidney in acute renal
cortex (better demonstrated on CT than
vein thrombosis
intravenous urogram)
• No contrast flow may occur in acute cases
• Diffuse renal involvement: poor contrast
enhancement, delayed/absent excretion of
Renal artery occlusion contrast, global renal enlargement
• Unifocal or multifocal renal involvement
Causes may be seen, with areas of apparently
• Embolism normal renal parenchyma
• Thrombosis • Wedge-shaped areas of decreased
• Dissection attenuation with straight borders, widest at
• Trauma the periphery of the kidney, represent the
• Involvement of the main artery or major abnormal areas
branches occur only in a small number of • Low-attenuation masses with bulging of
cases the renal surface in severe inflammation
Signs and symptoms (do not enhance following contrast
• Variable and can be asymptomatic administration)
• Abrupt onset of severe abdominal or flank • Liquefaction (central low attenuation)
pain, nausea, vomiting, haematuria and in involved regions suggests abscess
albuminuria formation
• Fever and leucocytosis
• Enlarged tender renal mass Renal abscess
Imaging features • Acute pyelonephritis can progress to tissue
necrosis and abscess formation
Intravenous urogram
• Small abscesses may respond to antibiotics
• Non-opacified, normal to enlarged kidney,
• Larger abscesses and perinephric
with a normal pelvicalyceal system is
involvement require drainage (may
characteristic
be performed under ultrasound or CT
CT guidance)
• ‘Cortical rim’ sign on nephrogram phase

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Renal system 193

CT features • Often associated with severe sepsis


• Attenuation value of around 30 HU • Treatment with percutaneous
• Distinct rounded margins and thick nephrostomy or ureteric stenting to bypass
enhancing walls the obstruction is first line
• Non-enhancing contents CT features
• Occasionally gas bubbles may be seen
• Thickening of renal pelvic wall
• Focal thickening of the adjacent renal
• Inflammatory changes in perinephric fat
fascia and perinephric stranding
• Rarely, layering of contrast medium
• Perinephric abscess if infection extends
anterior to pus in a dilated renal pelvis
through the capsule
• Pelvicalyceal system gas in the absence of
instrumentation
Emphysematous • CT may be able to demonstrate the site of
pyelonephritis obstruction

• Life-threatening infection of renal


parenchyma by gas forming organisms
Xanthogranulomatous
• Most patients are diabetic pyelonephritis
• Escherichia coli is the most common
• Chronic inflammatory disease associated
bacterial species involved
with indolent bacterial infection
• Inflammatory process begins in the renal
Emphysematous pyelonephritis pelvis and extends into the renal medulla
and cortex
Type 1 • Lipid-laden macrophages replace the renal
• Destruction of more than one third of parenchyma
parenchyma • Usually diffuse but can be focal
• Mortality rate approximately 70% • Typical patients are middle-aged females
• Streaky mottled gas radiating • E. coli or Proteus mirabilis are frequently
peripherally cultured from urine
• May require nephrectomy if
conservative measures fail Symptoms
• Malaise, fever, chills and weight loss
Type 2 • Flank pain, frequency, dysuria and
• Destruction of less than one third of nocturia
the parenchyma
• Renal or perirenal fluid collection with CT features
locules of gas within the collecting Diffuse form
system • Diffuse reniform enlargement with ill-
• Mortality rate approximately 20% defined central low attenuation
• Apparent cortical thinning
Utility of CT • Staghorn calculus in up to 70% of cases
• Most sensitive modality for diagnosis • Absence or decrease of contrast excretion
• Can identify gas in the collecting on affected side
system, parenchymal or perinephric • Less commonly a small contracted kidney
tissue with replacement lipomatosis
• Multiple fluid-density, rounded areas
almost completely replacing the kidney
• Rim enhancement of the low-density areas
Pyonephrosis • Extrarenal extension is common
• Perinephric and psoas muscle abscesses
• Accumulation of pus in an obstructed are common
pelvicalyceal system

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194 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Focal form CT features


• Poorly enhancing mass in one pole or • Contrast-enhanced CT is modality of
adjacent to a calyx choice
• May be associated with calculus • Scans are obtained at 70 seconds and
• Sometimes misdiagnosed as a neoplasm 3 minutes after the start of contrast
injection
Renal trauma Contusion
• Can be caused by blunt or penetrating • Amorphous interstitial extravasation of
injuries blood and oedema
• Unenhanced CT: focal swelling and
Renal trauma irregular high-density infiltrates
• Enhanced CT: ill-defined rounded or
Category I ovoid areas of low attenuation
• 75–85% • Small interstitial accumulations of contrast
• Contusions and small corticomedullary material on delayed images
lacerations that do not communicate Laceration
with collecting system • Superficial: limited to cortex
• Clinically insignificant • Deep: extends to medulla
• Managed conservatively • Contrast extravasation is often seen into
the perinephric tissues
Category II
• 10%
Infarction
• Focal area may be caused by thrombosis or
• Laceration through the renal cortex
laceration of the segmental arteries
extending into the medulla or
• Typically appears as non-enhancing
collecting system with or without
peripherally based wedge-shaped areas
urinary extravasation
• May be managed conservatively Haematoma
• Can be intrarenal, subcapsular or
Category III perinephric
• 5%
Main renal artery injuries
• Multiple deep lacerations and injury to
• Absence of contrast medium enhancement
the renal pedicle
• Cortical rim sign may or may not be seen
• Haematoma surrounding the renal hilum
Category IV
may be seen
• Rare
• Abrupt cut-off of contrast-filled renal
• Pelviureteric junction avulsion
artery
• Laceration of the renal pelvis
Acute renal vein occlusion
Subcapsular and perinephric • Enlarged kidney
haematomas may occur with any of these • Thrombus visualised in the renal vein
grades but when isolated are categorised • Venography may be performed if injuries
as category I to the renal vein and inferior vena cava are
suspected
Pelviureteric junction injuries
• Rare
Symptoms and signs
• Excellent excretion of contrast material in
• Gross haematuria
the intrarenal collecting system
• Absence of haematuria does not preclude
• Medial perinephric urinary extravasation
serious injury
• Circumrenal urinoma may be seen
• Absence of haematuria is reported in 24%
of renal artery thrombosis and one third of
pelviureteric junction injury

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Renal system 195

Simple renal cyst Imaging features


• Most common renal masses CT features
• Frequency increases with age • Well-defined masses with smooth rounded
• Usually asymptomatic margins
• May cause pressure symptoms or • Occasionally contain calcifications
symptoms due to haemorrhage or • May be multiple and bilateral
infection • Small oncocytomas are usually displayed
• Solitary or multiple and commonly homogeneous enhancement and may be
bilateral impossible to distinguish from small slow-
growing renal cell carcinomas
Ultrasound features • 25–33% of large oncocytomas have central,
• Sharp margination and demarcation from sharply defined stellate scar which strongly
surrounding parenchyma suggests the diagnosis
• Smooth and thin walled • ‘Spoke wheel’ enhancement pattern in
• Homogeneous anechoic content larger lesions, but this does not reliably
differentiate from renal cell carcinoma

Bosniak classification Catheter angiography


• Homogeneous blush
• Cannot reliably distinguish from renal cell
• Category 1: classical ‘simple cysts’
carcinoma
• Category 2: thin (< 1 mm) septae, fine
calcifications in cyst wall or septa or MRI
high density. Can be monitored with • Iso/hypointense to normal parenchyma on
serial imaging. MRI may be helpful to T1-weighted image (T1WI)
characterise high-density lesions • Variable appearance on T2-weighted
• Category 3: thick irregular mural or image (T2WI)
septal calcification, numerous or thick • Central scar
irregular septae, Uniform or slightly • Enhances less than normal renal
nodular wall thickening. These lesions parenchyma following gadolinium
shall be explored surgically unless administration
contraindicated • Fine-needle aspiration is helpful in
• Category 4: clearly malignant lesions discriminating between oncocytoma and
with cystic components renal cell carcinoma

Angiomyolipoma
• Benign hamartomas composed of blood
Renal adenoma vessels, smooth muscle and fat
• May be seen in isolation or in patients with
• Solid lesion < 3 cm in size detected on tuberous sclerosis (usually multiple)
ultrasound or CT • Sporadic form (accounts for 80–90% of
• Previously considered to be benign but cases)
is now considered to be renal carcinoma –– Typically seen in middle age
in evolution and treated by partial –– More common in females (4:1)
nephrectomy • Usually asymptomatic
• Large masses can cause flank pain,
Oncocytoma haematuria and anaemia, and carry a
high risk of intratumoural or perinephric
• Solid epithelial neoplasm with a generally
haematoma
benign course
• Conservative for lesions smaller than 4 cm
• Represents 10–15% of small (< 3 cm) solid
with typical features
renal neoplasms
• Arises from proximal tubular epithelium

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196 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Partial nephrectomy or selective catheter • Pancreatic cysts, islet cell tumours,


embolisation for masses larger than 4 cm phaeochromocytomas, retinal
is advocated angiomas and central nervous system
• Annual follow up with CT or ultrasound haemangioblastomas are other
in asymptomatic patients with lesions features. Endolymphatic sac tumours,
approaching 4 cm in diameter; larger cystadenomas of the epididymis and broad
lesions are followed up more frequently ligament cysts and tumours are recognised
manifestations
Imaging features • Pancreatic cysts occur in around 30% of
CT patients
• Unenhanced: fatty attenuation (values less • Pheochromocytomas occur in about 10%
than -10 HU) of patients (often multiple and ectopic;
• Around 5% do not contain fat and 50–80% are bilateral)
therefore cannot be differentiated from • Abdominal CT screening of first-degree
renal cell carcinoma relatives is sometimes advocated from the
• Extensive intratumoural haemorrhage can second decade of life
obscure the presence of fat
• Well-circumscribed mass
• Vary in size from tiny nodules to large
Tuberous sclerosis
tumours • Autosomal dominant with variable
• Extensive bilateral involvement in patients penetrance
with tuberous sclerosis • More than half of cases are sporadic
• Presence of intratumoural fat is almost • Classic triad: epilepsy, mental retardation
diagnostic and adenoma sebaceum
• Retroperitoneal lymph node involvement • Renal lesions are seen in 50% of patients
may be seen • Bilateral renal cysts
• Extrarenal extension may be seen • Bilateral angiomyolipomas that may
• Extension into the renal vein and inferior appear at an early age and grow faster
vena cava may be seen • Multiple renal angiomyolipomas are the
sine qua non of tuberous sclerosis
MRI
• Occur in about 15% of patients, mostly
• Presence of fat is demonstrated by
females
chemical shift imaging
• 1–2% of patients with tuberous sclerosis
develop renal cell carcinoma
Von Hippel–Lindau disease • Perirenal cystic collections or
• Autosomal dominant with almost 100% lymphangiomas
penetrance • Other associated abnormalities include
• Cysts are the most common renal subependymal nodules, giant cell
manifestation astrocytoma, peripheral tubers, retinal
• 25–40% of affected patients develop clear hamartomas, cardiac rhabdomyomas,
cell renal carcinoma lymphangioleiomyomatosis, shagreen
• Often develop renal cell carcinoma in the patches, subungual fibromas and bone
fourth or fifth decade of life cysts
• Synchronous and metachronous renal cell
carcinomas are common Renal carcinoma
• Transformation of simple cysts to solid
• Most common urological malignancy in
lesions is rare
adulthood
• Complex lesions require follow up as solid
• Can occur at any age but most common in
elements may transform
seventh and eighth decades
• Predominantly cystic lesions with solid
components are characteristic

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Renal system 197

• Known risk factors: smoking, petroleum Upper tract transitional


products, obesity, acquired cystic renal
disease, Von Hippel–Lindau disease, cell carcinoma
tuberous sclerosis and hereditary papillary • 10% of neoplasms of the upper tract
renal cancer • Usually present as haematuria; can present
• Almost 50% are detected incidentally as renal colic
on imaging for symptoms other than • Multicentric
haematuria or flank pain • 2–4% of cases are bilateral
• Most commonly metastasises to the lungs, • Most common sites of metastases are liver,
bones and liver bone and lungs
• Less commonly to the adrenal glands,
kidney, brain, pancreas, mesentery and Imaging features
abdominal wall Intravenous urogram
Imaging features • Calcification is uncommon (2–7%) and
may mimic calculi
CT • Enlargement of the kidney with large
• Unenhanced, corticomedullary and infiltrating tumour or ureteric obstruction
nephrographic phase imaging are • Filling defect in the collecting system or
necessary ureter
• Corticomedullary phase is best for • ‘Stippling sign’ : tracking of contrast
evaluating venous extension medium into the interstices of a papillary
• Nephrographic phase is best for evaluating lesion
the primary mass lesion • Stricturing (if multiple can mimic
• Calcification in up to 30% of tumours (may tuberculosis)
be amorphous or curvilinear)
Ultrasound
• Enhancement is usually less than that of
• Usually slightly hyperechoic compared
the normal renal parenchyma
with normal renal parenchyma
• Tumour haemorrhage or necrosis can
• Typically infiltrative and does not cause
cause heterogeneous enhancement
contour distortion
• Lobulated or irregular margin may be
seen, though small tumours often have CT
distinct smooth margins • Typically hyperattenuating compared with
renal parenchyma
MRI
• Sessile filling defect seen on the excretory
• Hypo- to isointense to renal parenchyma
phase of CT urography
on T1WI
• Centrifugally expanding mass causing
• Heterogeneously hyperintense on T2WI
compression of the renal sinus fat
• Less enhancement than normal
• Pelvicalyceal irregularity, focal or diffuse
parenchyma after gadolinium
thickening or focally obstructed calyx
• Presence of fat, haemorrhage and necrosis
• Reniform shape is typically preserved
affects signal intensity
• Ureteric lesion may manifest as
Post-nephrectomy evaluation hydronephrosis
• Recurrence in the renal bed occurs MRI
in about 5% patients after radical • Not commonly used technique for
nephrectomy assessment of transitional cell carcinoma
• Renal bed recurrence is most likely within • Nearly isointense to renal parenchyma on
first 2 years following nephrectomy T1WI and T2WI
• Moderate enhancement following
gadolinium
• Static or dynamic MR urography may be
employed

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198 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Renal lymphoma • Occasionally solitary lesions, but if


there are metastases in other regions a
• Usually secondary to systemic disease solitary renal lesion can be assumed to be
• Bilateral in 75% metastasis
• More common in non-Hodgkin’s • Colonic metastases are commonly solitary
lymphoma and exophytic
• Seen in about 8% of patients with • Melanoma metastases typically have
lymphoma perinephric extension
Imaging features
CT Chronic renal failure from
• Five patterns are recognised: diffuse parenchymal disease
–– Multiple renal masses (59%)
• Also known as chronic medical renal
–– Solitary mass (3%)
disease
–– Renal invasion (contiguous
• Marked parenchymal atrophy with smooth
retroperitoneal spread) (28%)
reniform contour
–– Perirenal disease (10%)
• Hyperechoic on ultrasound
–– Diffuse renal infiltration (rare)
• Homogenously enhancing nodule which
are less dense than normal parenchyma on Renal papillary necrosis
contrast-enhanced CT • Chronic tubulointerstitial nephropathy
• Renal arteries and veins remain patent • Predominantly affects the renal medulla
despite tumour encasement (characteristic
sign) Causes
• Obstructive hydronephrosis may be • Diabetes
seen when there is retroperitoneal • Analgesic abuse or overuse
lymphadenopathy • Sickle cell disease
MRI • Pyelonephritis
• Same morphologic features as on CT • Renal vein thrombosis
• Hypointense relative to the cortex on • Tuberculosis
T1WI and heterogeneously hypointense to • Obstructive uropathy
isointense on T2WI Imaging features
• Minimal enhancement following • Intravenous urogram
gadolinium • Normal in the initial period of early
ischaemic change
Renal metastases • Clefts extending from the fornices to the
papillary tips
• The kidneys are the fifth most common site
• Cavity in the papilla filled with contrast
of metastasis in the body
• Sloughed papilla may calcify over the
• Mainly haematogenous spread
course of time
• Lung, breast and contralateral kidney are
the most common primary sites CT
• Usually discovered incidentally • Nephrographic phase: poorly marginated
• Some patients present with haematuria areas of diminished enhancement at the
and flank pain tip of medullary pyramid in the early
ischaemic phase
CT features • Excretory phase: clefts and cavities filled
• Multiple discrete bilateral lesions are most with contrast medium
common • Calyceal blunting at the papillary tip
• Usually homogeneous enhancement • Scarring and atrophy of the kidney
pattern following healing
• Central necrosis may be seen with large • Diffuse papillary calcifications
lesions

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Renal system 199

Genitourinary tract • Epididymitis, hydrocoele, palpable


testicular mass, discharging scrotal
tuberculosis or perineal sinuses may be the first
• Second most common site of tuberculosis presentation of male genital tract
• Usually haematogenous spread to the involvement
kidneys • Rectal or pelvic pain may be the presenting
• May be asymptomatic symptom of prostatic tuberculosis
• Pelvic pain, menstrual irregularity and
Pathogenesis sterility may be the presenting features of
• Begins as small tubercles and progresses to female genital tract involvement
necrotic lesions/irregular cavities
• Cavities communicate with calyces, which Radiographic features
can undergo structuring • Plain radiographs
• Usually bilateral asymmetrical renal • Signs of extrarenal tuberculosis (e.g. lung
involvement consolidation, lymphadenopathy)
• Unilateral in 25% • Active or healed pulmonary tuberculosis
• If not appropriately treated, the kidney in 50%
becomes atrophic scarred and densely • Dystrophic renal calcification may
calcified sometimes be seen
• Ureteric involvement may occur secondary Intravenous urogram
to renal involvement
• Mucosal granulomas form which Kidneys
eventually fibrose • ‘Smudged papillae’ due to surface
• Upper and/or lower thirds of the ureters irregularity
are usually involved • ‘Moth-eaten’ calyx
• Vesicoureteric reflux can occur from a • Irregular tract formation from the calyx to
fixed patulous vesicoureteric junction the papillae
• Bladder infection usually occurs • Large irregular cavities
secondary to renal infection • Enlarged kidneys initially; later become
• Bladder mucosal ulceration can lead atrophic
to scarring and fibrosis, resulting in a • Hydrocalyces with no pelvis dilatation or
thickened contracted bladder an atrophic pelvis is suggestive
• Bladder wall calcification is uncommon • ‘Hiked-up renal pelvis’: cephalic retraction
• Seminal vesicle involvement is usually of the infero-medial margin of renal pelvis
haematogenous in nature; mucosal at the pelviureteric junction
tuberculomas, ulceration and fibrosis can • Stricture at pelviureteric junction can
occur cause hydronephrosis or delayed function,
• Prostatic infection is usually secondary to clubbed calyces or no function
renal infection. Can cause fistulae into the • Associated renal calculi seen in 20% of
surrounding tissues cases
• Can cause chronic epididymitis and • Autonephrectomy (small scarred non-
epididymoorchitis functioning kidney with calcification)
• Female genital tract involvement may be • Renal calcification seen in 50%
secondary to haematogenous or lymphatic (amorphous granular associated with
spread. Can cause infertility active infection; dense punctate associated
with healed tuberculomas)
Clinical features
Ureters
• Presentation is usually late
• Usually unilateral (if bilateral appears
• Frequency, urgency and dysuria may
asymmetrical)
occur with tuberculous cystitis
• Intraluminal filling defects
• Secondary infection of hydronephrosis
• Irregularity of the mucosa
cause symptoms

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200 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Beaded or ‘corkscrew’ appearance from MRI features


scarring alternating with dilatations • Good for depicting tuberculous cavities,
• Rigid, aperistaltic, shortened ureter sinuses, fistulae, and extrarenal and
• Bilateral hydroureters extraprostatic spread
Bladder Ultrasound features
• Small trabeculated bladder
• Hypoechoic or cystic masses
• Vesicoureteric reflux
communicating with collecting system,
• Wall calcification rare
representing excluded calyces
• Fistulae or sinus formation
• Renal tuberculomas usually appear as a
Prostate solid mass
• Dense calcification in the prostatic bed • Calcifications may be seen as echogenic
CT features foci with post-acoustic shadowing
• Thick-walled small-volume bladder
• Calyceal changes are better seen on
• Enlarged heterogeneous epididymis,
intravenous urogram
hypoechoic lesions in the testis or diffusely
• CT is superior for demonstrating
hypoechoic testis
calcification and extrarenal extension
• Thickening of scrotal wall and tunica
albuginea, and moderate hydrocoele

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4.2 Urinary bladder

Ureters • Associated with incontinence (chronic


leakage), normal voiding and infections
Anatomy and embryology • Chronic infection can lead to fibrosis
• The pelvicalyceal system and ureter and stenosis of the ureter resulting in
develop from the ureteric bud which in hydronephrosis of the upper moiety
turn arises from the mesonephric duct Retroperitoneal fibrosis
• 10–25 calyces develop per kidney
• Rare fibrotic retroperitoneal process
• The ureters usually lie anterior to the
transverse processes of the lumbar Causes and associations
vertebrae • Idiopathic form known as Ormond’s
• In the pelvis the ureters cross the common disease (more common in men)
iliac vessels • Approximately two thirds are considered
• The ureters enter the bladder from its idiopathic
posterolateral surface • Peak age fifth and sixth decades of life
• The ureters and pelvis are lined by • Often coexists with inflammatory bowel
transitional epithelium disease, sclerosing cholangitis, fibrosing
mediastinitis, Riedel’s thyroiditis,
Ureteral duplications sclerosing mesenteritis and orbital
• Result from more than one ureteric bud pseudotumour
forming from the mesonephric duct • Can occur secondary to aortic aneurysm,
Incomplete retroperitoneal metastasis from
• Usually not clinically significant lymphoma, breast carcinoma or carcinoid
tumour, retroperitoneal haematoma,
Complete abscess, urinoma, diverticulitis or
• Much less common than incomplete appendicitis
duplication • Drugs known to be associated are ergot
• Weigert–Meyer rule: ureter draining the alkaloids and hydralazine
upper moiety of the kidney inserts inferior • Typically originates below the aortic
and medial to the other ureter bifurcation and extends superiorly
• Upper moiety ureteric insertion can be • Fibrosis begins just lateral to the aorta
extravesical • Usually involves the left ureter before the
• The ureter draining the lower moiety of the right
kidney is prone to reflux • Fibrotic process extends to the aortocaval
• The upper moiety ureter is prone to and pericaval region
develop a ureterocoele • Usually does not extend between the aorta
• May present with infection, incontinence and vertebral bodies
or renal impairment from obstruction • The ureter can be involved at any level, but
L3–5 most common
Ectopic ureterocoele • Usually does not encase the renal pelvis
• Submucosal dilatation of the intramural • Limited anteriorly by the peritoneum
ureter at the vesicoureteric junction
• Most occur in association with duplication
Symptoms and signs
• May project into the bladder lumen • Dull back, flank and abdominal pain
• Can obstruct the other ureter if a duplex • As ureters are most frequently involved,
system is present pain, oliguria, anuria and eventually renal
• Seen more commonly in females failure may occur
• Lower extremity oedema, scrotal oedema
Extravesical insertion of ureter or deep vein thrombosis from compression
• Bladder neck, urethra or vagina in females of the inferior vena cava

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202 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Imaging features Imaging features


• Soft tissue layered around the aorta and
Intravenous urogram
inferior vena cava
• Bladder assumes an inverted pear shape,
• Usually does not lift the aorta off the
with narrowing most prominent in the
vertebral bodies
centre or inferiorly
• Encases ureteric wall, leading to loss of
• Elevation of bladder base
peristalsis of the involved segment
• Reduction of bladder capacity
• Ureteric mucosa is always spared
• Medial deviation of pelvic ureters
• Absence of peristalsis leads to functional
• Dilatation of proximal ureters may also be
obstruction, while the lumen remains
seen
patent
• Degree of hydronephrosis does not Barium enema
correlate with degree of renal insufficiency • Elongation and narrowing of rectum and
• Passage of ureteral stents is unusually easy sigmoid colon
• Presence of other lymphadenopathy or • Intact colonic mucosa
metastatic disease, osseous destruction • Enlarged retrorectal space
• Variable enhancement characteristics
CT
Malignant retroperitoneal fibrosis • Confirms the presence of fat in the pelvis,
• Ill-defined margin or inhomogeneity of with mass effect on the pelvic organs
plaque is suggestive
MRI
• Malignant lymphadenopathy usually lifts
• Signal intensity of mature fat on all pulse
the aorta from the vertebral bodies and
sequences
displaces the ureters laterally
MRI features Trauma to the ureter
• T1WI: low to intermediate signal intensity • Penetrating injury is the most common
• T2WI: mechanism
–– Low signal: benign retroperitoneal • Acceleration or deceleration trauma
fibrosis (mature fibrotic plaque) can cause avulsion of the ureter,
–– High signal: early benign and malignant the commonest site involved is the
retroperitoneal fibrosis pelviureteric junction
• This type of injury is more common in
Pelvic lipomatosis children
• The true pelvis is infiltrated by • Imaging findings include urinoma
unencapsulated benign mature adipose formation, contrast extravasation and
tissue discontinuity of the ureter
• African-American men are affected in two
thirds of cases Ureteritis cystica
• Peak age third and fourth decades • Associated with chronic urinary tract
infections
Symptoms • Sterile submucosal fluid collections
• Non-specific abdominal or low back pain caused by intramural inflammation
• Urinary frequency infections • Leads to encystment and submucosal
• Urinary tract obstruction in 40% of cases extension of transitional epithelium
• Constipation • Typically multicentric smooth and round
• Associated with proliferative cystitis, • Seen as filling defects in the ureter on
particularly cystitis glandularis (75% of intravenous urography (intramural
cases) haemorrhage can have a similar
• Increased risk of bladder adenocarcinoma appearance)

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Urinary bladder 203

Bladder and urethra Inflammatory pseudotumour


• Non-neoplastic proliferation of
Embryology myofibroblastic spindle cells and inflam­
• The bladder develops from the anterior matory cells with myxoid components
urogenital sinus of the cloaca • Symptoms: haematuria, iron deficiency
• It communicates with the allantois anaemia, fever
through the urachus • Can mimic malignancy clinically and at
• The urachus becomes obliterated and cystoscopy and imaging
forms the median umbilical ligament
• Most of the female urethra, the prostatic Imaging features
and membranous portions of the male • Single bladder mass that may be exophytic
urethra develop from the urethral part of or polypoid and may be ulcerated
the urogenital sinus • Intramural solid and cystic variants
• A small portion of the urethra and • Tends to spare the trigone
vestibule in the female and the penile • Invasion through the bladder wall and
urethra in the male develops from the extravesical extension is possible
phallic part of the urogenital sinus. • Contrast enhancement on CT and MRI
• Internal vascularity may be seen on colour
Schistosomiasis Doppler sonography
• Schistosoma haematobium infestation
causes bladder and ureteric disease
Endometriosis
• The eggs of the fluke cause a foreign body • Bladder is the most common site of
reaction in the bladder mucosa or distal urinary tract involvement
ureter • Prevalence of 1–5% in women with
• Affects men more commonly than women endometriosis
• Symptoms include haematuria, dysuria, • Reported in premenopausal women only
frequency and urgency • Cyclic haematuria occurs in only 20% of
• Major differential diagnosis is tuberculosis cases
• High incidence of associated squamous • Asymptomatic or symptoms of cyclic pain,
cell carcinoma dysuria, urgency and pain
• Bladder endometriosis is deeply
Imaging features infiltrating
• Plain film: calcification in bladder wall and • Most lesions are found in the posterior
distal ureters may be seen wall of the bladder above the trigone or at
• Intravenous urogram: mucosal the dome
irregularity, ureteritis cystica, ureteral
dilatation and stricture (mostly limited to Imaging features
distal ureters) • Can be non-specific
• Persistent filling/beading in lower ureters • Lesion location is most helpful for
• Linear or parallel calcification in distal diagnosis
ureters • Posterior and may be inseparable from the
• Mucosal irregularity and reduced bladder anterior aspect of the uterus
capacity • MRI: haemorrhagic foci with high signal
• Bladder wall calcification can completely intensity on fat suppressed and non-fat
encircle the bladder (initially seen at the suppressed T1WI
bladder base) • High signal intensity on T2WI may be seen
• Even though fibrotic the bladder still • Contrast enhancement can be either
remains distensible and retains normal homogeneous or peripheral
volume
• Calcifications are better delineated with
Nephrogenic adenoma
CT and are more common on the anterior • Benign reactive process to chronic
bladder wall irritation

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204 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Involves the lamina propria but spares the Cystitis


muscular layer
• Cystitis cystica and cystitis glandularis
• Not premalignant
(chronic reactive inflammatory disorders
• May recur after resection in up to 63% of
• Can occur at any age
cases
• Symptoms: frequency, dysuria, urgency,
• Symptoms: irritative voiding symptoms or
haematuria
haematuria
• Imaging features are nonspecific Imaging features
• Polypoid or sessile masses or simply • Filling defects at urography
mucosal irregularity • Hypervascular polypoid mass on CT and
Malacoplakia MR
• Low signal on T1WI; predominantly low
• Chronic granulomatous condition, most signal on T2WI with central branching
frequently affecting the bladder high signal
• Predominantly seen in women • Muscle layer is intact
• Peak occurrence in middle age
• More common in diabetics or Malignant tumours
immunocompromised patients of the bladder
• Highly associated with E. coli infection
• Accounts for 4% of all malignancies
• Symptoms: gross haematuria, signs of
• Peak age sixth and seventh decade
urinary tract infection
• More common in men (3:1)
Imaging features • 95% are carcinomas
• Multiple, polypoid, vascular, solid masses • The most common non-epithelial
or circumferential wall thickening malignancy is leiomyosarcoma
• Can be extremely aggressive, invading the • Other pelvic malignancies can infiltrate
perivesical space or even bone destruction into the bladder
• Stomach and breast cancers may
metastasise to the bladder

Ch-04.indd 204 8/9/2010 1:42:52 PM


4.3 Testis

Anatomy • The pampiniform plexus drains the


scrotum into the ipsilateral testicular vein
• Ovoid in shape • The cremasteric nerve, genital branch of
• 3.5–4.0 cm in length the genitofemoral nerve and the testicular
• Covered by a fibrous capsule called the sympathetic plexus are the nerves in the
tunica albuginea cord
• Mediastinum testis: vertical • Lymphatic vessels ascend with the
invagination of the fibrous capsule in testicular vessels and drain into the lateral
the posterosuperior portion of the testis and preaortic nodes
(spermatic cord enters testis here)
• Multiple lobules form each testis, the Scrotum
apices of which converge into the • Wall is derived from the layers of the
mediastinum abdominal wall
• The seminiferous tubules in each lobule • The tunica vaginalis is in continuity with
coalesce and enter the mediastinum testis the peritoneal processus vaginalis. The
• These form 12–20 efferent ductules after tunica vaginalis is reflected on itself
multiple anastomosis forming a visceral and parietal layer. The
visceral layer is closely applied to the testis,
Epididymis epididymis and posterior scrotal wall. The
• Continuation of the ductal system of the parietal layer is in contact with the scrotal
testis wall
• Lies posterolateral to testis • The dartos is a highly vascularised muscle
• Head of the epididymis (globus major) layer of the scrotum
is lateral to the upper pole of the testis
(normally 7–8 mm in diameter)
• The body and tail of epididymis extend Embryology
inferiorly and continue as the vas deferens
• Genital ridges extend from the sixth
Vas deferens thoracic to the second sacral segments
• Ascends on posterior aspect of spermatic • Coelomic epithelial cells migrate into and
cord proliferate to form primitive sex cords
• Leaves spermatic cord at deep inguinal • Germ cells in the wall of the yolk sac
ring migrate into the sex cords through along
• Extends anterior to internal iliac artery the hindgut and dorsal mesenteric root
• Descends between the posterior surface • In the presence of a Y chromosome,
of bladder and the superior pole of the primitive sex cords form the seminiferous
seminal vesicle tubules
• Forms ejaculatory duct by joining seminal • The migrated germ cells form the
vesicle at base of prostate spermatogonia
• Ejaculatory duct opens into urethra • The mesenchyme between the
adjacent to veru montanum and prostatic seminiferous tubules forms the Leydig’s
utricle cells
• The tunica albuginea forms along the
Spermatic cord surface of the primitive testis
• Composed of the vas deferens and vessels, • At about 8 weeks the Leydig’s cells begin to
lymphatics and nerves secrete testosterone
• Internal spermatic (testicular) artery, • Under hormonal influence, the
external spermatic (cremasteric) artery mesonephric ducts develop into the
and artery to the vas are the arteries in the epididymis, vas deferens, seminal vesicles
cord and ejaculatory ducts

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206 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• The paramesonephric ducts regress Varicocoele


• Between 7 and 8 weeks the testes descend
into the pelvis and remain near the deep • Compressible tangle of veins in the
inguinal ring until the seventh month scrotum measuring > 2 mm in diameter
• The testes descend through the inguinal • Abnormal dilatation of pampiniform
canal into the scrotal sac venous plexus
• Results from incompetent valves of the
internal testicular vein; rarely caused by
Acute epididymitis venous obstruction
• Commonest treatable cause of male
• Commonest acute scrotal pathology in
infertility
postpubertal age group
• More common on left side (95%)
• Retrograde infection from the urethra or
• Ultrasound: compressible, tortuous
prostate is thought to be the cause
vessels, made more prominent by standing
• Symptoms: presents as acute or subacute
up or Valsalva manoeuvre. Colour Doppler
pain and fever, dysuria
demonstrates flow
• Signs: scrotal erythema, pyuria
• Orchitis develops a complication in 20% of
patients Orchitis
Ultrasound features • Infection of the testis
• Most common complication of mumps
• Swollen epididymis (> 5 mm thickness)
infection in postpubertal males
• Usually hypoechoic (rarely hyperechoic)
• Mumps orchitis is more commonly
• Thickening of the scrotal skin and/or
unilateral
hydrocoele
• Increased vascularity on colour Doppler is Imaging features
suggestive of inflammation
Ultrasound
• Hyperaemia may be the only sign in up to
• Diffuse enlargement of the testis
20% of patients
• Homogeneously hypoechoic
• Hyperaemia may be focal
• Can also appear normal or have a diffusely
• Complex fluid collection around the testis
inhomogeneous texture
is suggestive of pyocoele
• Vascularity may be increased especially
• Hypoechoic area in the testis may indicate
when compared with the normal side
orchitis
• Epididymis or scrotal skin may be
abnormal
Chronic epididymitis • Testis may return to normal once the
inflammation resolves or may atrophy
Imaging features • Atrophy is usually detectable within 6
Ultrasound months
• Swollen epididymis which may be • Unilateral atrophy in one third of patients
hyperechoic on ultrasound with mumps orchitis and bilateral in 10%
• Focal orchitis is difficult to distinguish
MRI from cancer, but usually does not distort
• Epididymal inflammation as evidenced the contour of the testis
by increase in size, increased signal on • Discrete intratesticular fluid collection,
T2WI, sympathetic hydrocoele, increased which is typically complex
number of vessels seen with signal voids • Gas if present will produce ‘dirty’ acoustic
• Patchy areas of low signal intensity may be shadowing
seen in areas of orchitis. Normal testis is of
high signal intensity

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Testis 207

Malignant testicular • A right testicular cancer first metastasises


to the paracaval lymph node group at or
neoplasm below the right renal vein
• Approximately 1% of all male malignancies • Paralumbar nodes below the renal hilum
• Commonest malignancy in 15- to 30-year- and thoracic sites are involved later
old males • If local spread into the epididymis or
• Usually presents as painless scrotal scrotum has occurred, iliac and inguinal
swelling lymph nodes may become involved
• Infarction or haemorrhage into the tumour • Choriocarcinoma is prone to early
can mimic torsion or epididymoorchitis haematogenous metastasis
• Haematogenous spread is most common
Classification to the lungs
• Primary germ cell • Liver, bone and brain are other sites
• Primary non-germ cell involved
• Metastatic • Brain metastases are common with
• Germ cell tumours constitute 95%, of choriocarcinoma
which 40% are seminomas and 40% have • Germ cell tumour metastases may have
mixed histology (teratocarcinoma is the histology characteristics different from
commonest among mixed) those of the original tumour
• Non-seminomatous germ cell tumours
are more aggressive. These include Imaging features
embryonal cell carcinoma, teratoma, Ultrasound
choriocarcinoma and yolk sac carcinoma • Most tumours are hypoechoic
• Serum alpha-fetoprotein levels are
Seminoma
increased in 60% patients with testicular
• Uniform decreased echogenicity
cancer
• Usually focal but may be diffuse
• Serum beta-human chorionic
• May cause bulging of tunica albuginea if
gonadotropin levels are increased in 50%
focal and peripheral
patients with testicular cancer
• Usually sharp interface with normal
• Pure seminomas do not cause increase in
parenchyma
alpha-fetoprotein
• Generalised hypoechoic appearance in
• Non-germ cell tumours are usually benign
diffuse infiltration
• Leydig cell tumours may produce
testosterone and 30% may be associated Lymphoma or metastatic disease
with precocious virilisation or • Usually focal hypoechoic mass
feminisation. Sertoli cell tumours can • Mixed histology and non-seminomatous
produce oestrogen and can present with germ cell tumours
gynaecomastia • Heterogeneous due to cystic areas or
• Lymphoma is the most common tumour in hyperechoic foci
older men and may be bilateral • Less distinct tumour margin
• Prostate, lung, kidney, gastrointestinal • Contour of the testis may be lobulated
tract, myeloma and leukaemia are
other recognised primary sites that
metastasise to the testis (lymphatic and
haematogenous spread)
Differential diagnosis for a focal
• Haematogenous spread does not occur
intratesticular mass
before lymphatic spread
• Sentinel lymph node group for a left • Orchitis
testicular cancer is the left renal perihilar • Haematoma
group, immediately below the left renal • Abscess
vein

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208 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Enlargement of the epididymis, scrotal Simple cysts


skin thickening and hydrocoele are more • Tend to occur in men 40 years or older
commonly associated with inflammatory • Usually 2 mm to 2 cm in diameter
processes • More often arise near mediastinum testis
• Small hydrocoele may be seen in about • Usually not palpable (when palpable are
10% of tumours not firm)
• Usually single but can be multiple
Staging (TNM) • Imperceptible wall on ultrasound with
through transmission
• Low-stage disease: tumour confined
Epidermoid cysts
to the testis, epididymis or spermatic
cord (T1–T3) and mild to moderate • Germ cell origin (so called keratocysts)
adenopathy (N1and N2) • Non-tender and usually palpable
• Advanced-stage disease: tumour that • Age usually 20–40 years
invades the scrotal wall (T4), significant • Size usually 1–3 cm
retroperitoneal adenopathy (N3), or Ultrasound
visceral metastasis (M1) • Varies with cyst content
• A solid mass with an echogenic rim and
onion ring appearance
• Avascular
Testicular calcifications
and microlithiasis Undescended testis
• Large calcifications may be seen with • Root of scrotum (50%)
tumours (teratocarcinoma, seminoma, • Within inguinal canal (20%)
embryonal cell carcinoma, and Sertoli and • Abdominal: any location between lower
Leydig cell tumour) pole of the kidney and internal ring (10%)
• Large irregular calcifications seen with • Bilateral (10%)
teratoma • Ultrasound is useful to identify the testes
• Treated cancers, old infections, in extra-abdominal location (it is essential
haematomas and infarcts can also calcify to identify the mediastinum testis)
• Microlithiasis (tiny, 1–2 mm), diffuse • Laparoscopy and MRI are used for locating
calcifications in the seminiferous tubules the testes within the abdomen
• May be bilateral but asymmetrical
• Seen also in cryptorchid or atrophic testis
Testicular trauma
Testicular cysts • Intratesticular: may need surgical
intervention
• Benign cysts: simple cysts, tubular ectasia, • Extratesticular: usually managed
epidermoid cyst, tunica albuginea cyst conservatively
• Intratesticular varicocoele, abscess and • Ultrasound readily distinguishes between
haemorrhage the two types
• Malignant cyst: teratoma
Tunica albuginea cyst Testicular torsion
• Mean age at presentation: 40 years
• Also termed torsion of the spermatic cord
• 2–5 mm in diameter
• Causes obstruction of the blood supply to
• Location—upper anterior or lateral aspect
the testis
of testis
• Uni- or multilocular Extravaginal
• If palpable, firm to palpation • Occurs in fetuses and neonates

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Testis 209

• The testis, epididymis and tunica vaginalis Ultrasound


twist in the spermatic cord • Grey scale is sensitive but not specific
Intravaginal • Colour Doppler ultrasound approaches
100% specificity
• More common in the peripubertal period
• Associated with bell clapper deformity Differential diagnoses
(tunica completely surrounds the testis) • Acute epididymitis
and seen in around 12% of males • Epididymo-orchitis
Clinical features • Testicular abscess
• Torsion of testicular appendix
• Acute onset of severe scrotal pain and
• Incarcerated hernia
associated nausea and vomiting
• Scrotal haematoma
• Scrotal swelling and erythema
• Ruptured varicocoele
• Difficult to palpate the testis
• Scrotal tumour (rarely)
• 10% may be bilateral
• Spontaneous detorsion may occur

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4.4 Prostate gland

Anatomy Transrectal ultrasound


• Usually possible to differentiate the central
• Derived from the urethral epithelium and transitional zones
• Prostatic utricle develops from the • Adenomatous nodules may appear as
epithelium of the urogenital sinus, hyper- or hypoechoic areas; biopsy is
Wolffian ducts and Müllerian ducts required to exclude cancer
MRI
Benign prostatic • Glandular hyperplastic nodules are of high
hyperplasia signal intensity on T2WI
• Low or high signal intensity on T1WI
• Unusual below 40 years • Stromal proliferation gives decreased
• Affects 50–75% of men over 60 years signal on T1WI and T2WI
• More common in Afro-Caribbean
patients and in patients with diabetes and
hypertension
Prostatitis
• Usually presents with pain
Symptoms
• Imaging is not usually indicated
• Usually results from obstruction of the • MRI: low signal intensity on T2WI (in
urethra at the level of the prostate peripheral zone) without focal mass or
• Hesitancy, diminution in the strength of capsular irregularity
the urinary stream, post-void dribbling, • Chronic inflammation can appear similar
sensation of incomplete emptying to cancer
• Frequency and urgency from uninhibited
contractions of the detrusor muscle
• Overflow incontinence and nocturia with Prostate carcinoma
increasing volumes of residual urine • Most common malignancy in men
• Chronic urinary retention • Tumour differentiation and size are
Pathology important for predicting behaviour and
prognosis
• Involves the glandular tissue surrounding
the prostatic urethra, in the transitional
zone Gleason grading system
• Commonly situated closer to the proximal
end of veru montanum Grades 1–5 depend on tumour
differentiation:
Imaging features • Grade 1 well differentiated, grade 5
Intravenous urogram poorly differentiated
• ‘J-hook’ configuration of the distal ureters • Grades 4 and 5 have potential for
• Visualisation of the entire ureter in a single lymphatic spread
film
• Dilatation of the ureter and tortuosity of Grades of the two predominant
the ureter if obstruction is long standing histologic patterns in any specimen are
• Pelvicalyceal dilatation with renal cortical summed to give the Gleason score:
thinning if obstructive nephropathy has • 2–4 is well differentiated
developed • 5–7 moderately differentiated
• Trabeculation of the bladder and • 8–10 poorly differentiated
diverticula formation
• Prominent prostatic impression on the Size: larger tumours are more often
bladder associated with metastases

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Prostate gland 211

• 85% are located in the peripheral zone • Mucinous adenocarcinoma and small
infiltrating cancers may be very difficult to
Staging detect
• A: Occult cancer
• B: Confined to the capsule; suitable for Patterns of metastases
radical surgery • Nodal metastasis to local lymph nodes
• C: Extracapsular spread; non-surgical • Obturator, internal iliac and external iliac
therapy groups
• D: Distant metastasis • More than 80% of patients with lymph
node metastases develop bone metastases
Imaging features within 5 years
Transrectal ultrasound Osseous metastasis
• Round or oval hypoechoic lesion in the
• Majority are osteoblastic
peripheral zone (classic appearance)
• 5% are purely lytic
• Iso- or hyperechoic lesions can also occur
• 10% are mixed osteolytic and osteoblastic
• Distortion of the regular contour of the
• The pelvic bones, lumbar spine, femur,
prostate is used to direct biopsy
thoracic spine and ribs are involved, in
MRI descending order of frequency
• Transaxial T1WIs are used to assess • Prostate-specific antigen levels > 58 ng/ml
periprostatic extension are indicative of bone metastasis
• Axial and sagittal T2WIs are also routinely
acquired Intrathoracic metastasis
• Foci of low signal in the peripheral zone • 6% of patients at first diagnosis have
(fibrosis, prostatitis and calcification) can intrathoracic metastasis
also cause a similar appearance • 25% of patients with stage D cancer have
• Haemorrhage after biopsy can cause low lung or pleural involvement
or high signal on T2WI (high signal on • Lymphangitis carcinomatosis is more
corresponding T1WI will also be seen) common pattern than lung nodules

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4.5 Adrenal gland

Anatomy • CT: contrast washout properties can be


used to help characterise adrenal masses
• The adrenal gland is 4–6 cm in length. • MRI: T1WI, T2WI and chemical shift
Its limbs are 3–6 mm thick. It has two imaging is employed for characterisation
parts—cortex and medulla of masses
• Blood supply is via the inferior, middle and • Angiography: only used in certain specific
superior adrenal arteries conditions
• Cortex is derived from mesoderm—forms
about 90% of the gland Adrenal adenomas
• Cortex is subdivided into zona
glomerulosa (aldosterone), zona • 2–10% normal people at autopsy and 1% of
fasciculata (cortisol) and zona reticularis patients undergoing abdominal CT
(epiandrosterone) • In the absence of known malignancy,
• Medulla is derived from the adrenal masses < 1.5 cm are almost always
neuroectoderm and secretes adrenaline adenomas
and noradrenaline (epinephrine and • Adenomas remain stable in size over time
norepinephrine) • The majority are non-functional (non-
• Right gland is posterior to the inferior secreting)
vena cava and lateral to the right crus of Imaging features
diaphragm
• Attenuation values < 10 HU at unenhanced
• Left gland is lateral to the aorta and left
CT (71% sensitive and 98% specific for
crus of diaphragm
adenoma)
• Venous drainage from the right adrenal
• Contrast material washout occurs rapidly
gland is to the inferior vena cava. The left
in adenomas, resulting in 60% or more
adrenal gland drains into the superior
washout (< 30 HU) at 15-minute delayed-
aspect of the left renal vein, hence adrenal
phase imaging
vein sampling may be used to localise
the side of the abnormality in the case of MRI
a functioning adenoma not detected by • Characteristic high-signal rim on fat-
imaging saturated SE sequence
• Non-functioning adenomas: isointense to
Imaging liver on T1WI and iso- or hyperintense on
T2WI with relative loss of signal intensity
• Abdominal X-ray: calcifications may be on out-of-phase imaging
detected
• Ultrasound: normal glands are often not
visible Adrenal myelolipoma
• Scintigraphy: • Uncommon, benign, encapsulated lesion
–– Iodine-131 labelled 6 beta-iodomethyl- • Incidental detection; may be large at
19-norcholesterol used to investigate presentation
adrenocortical pathology • No malignant potential
–– Iodine-131 labelled meta- • Large tumours can bleed spontaneously
iodobenzylguanidine/indium-111 and become centrally necrotic
octreotide used to investigate functional • Always non-functioning
lesions of the adrenal medulla
–– Fluorine-18 labelled Imaging features
fluorodeoxyglucose (FDG) positron • Hallmark on imaging is the demonstration
emission tomography (PET) is useful in of lesional fat
differentiating benign from malignant • Ultrasound: increased echogenicity
pathology

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Adrenal gland 213

• CT: heterogeneous; foci of calcification common extra-adrenal site is the organ


in 20% (especially if haemorrhage has of Zuckerkandl situated at the aortic
occurred) bifurcation
• MRI: high signal on T1WI in areas of fat • Can occur as part of a syndrome: multiple
and previous haemorrhage. Chemical shift endocrine neoplasia (MEN) types 2 and
artefact on out-of-phase imaging 3, neurofibromatosis, von Hippel–Lindau
syndrome, Sturge–Weber syndrome,
Adrenal cyst Carney’s triad and tuberous sclerosis
• Clinical features: headaches, palpitations,
• 45% endothelial cysts; 40% pseudocysts excessive sweating, tremor, anxiety,
(secondary to previous haemorrhage, hypertension
infarction or cystic degeneration) • Occasional hypertensive crisis can be
• May be large (> 3 cm) at presentation induced by contrast medium
Imaging features Imaging features
• Peripheral or curvilinear calcification in • Usually > 3 cm
15% • Characteristically solid and hypervascular.
• Thick irregular walls and septations may Central necrosis, calcification and cystic
be seen with pseudocysts changes may rarely be present
• MRI: low, intermediate or high signal
Adrenal haemorrhage intensity on T2WI
• Atypical features such as areas of fat
• Acute haemorrhage can be spontaneous, density and contrast washout may be
post-traumatic (more common on right encountered
side) or due to coagulation disorders • Scintigraphy: meta-iodobenzylguanidine
• Metastases (e.g. melanoma) are prone to (mIBG) and octreotide uptake. FDG avid
spontaneous haemorrhage • Laboratory: increased vanilmandelic acid
• Up to 20% of haemorrhage is bilateral: levels in 24-hour urine sampling
this requires assessment for adrenal
insufficiency
• Spontaneous haemorrhage should
Adrenal carcinoma
become smaller on follow up • Very rare; can be functioning or non-
functioning
Imaging features • Associated with MEN type 1
• MRI: varying signal intensity depending on • Two peaks: one in paediatric population;
age of bleed other in fourth and fifth decades of life
• Calcifications may develop beyond 1 year • Large tumour size (> 12 cm), intratumoural
haemorrhage and high mitotic rates are
Phaeochromocytoma predictors of poor prognosis
• Cushing’s syndrome and virilisation are
• Rule of tens: 10% extra-adrenal, 10% frequently present
malignant, 10% extra-abdominal, 10% • Metastasises by haematogenous and
familial and 10% bilateral lymphatic spread
• Catecholamine-secreting tumours, derived
from chromaffin cells of the adrenal Imaging features
medulla • Calcification seen in 20–30% of cases
• 90% of phaeochromocytomas are located • CT: large inhomogeneous mass with
within the adrenal glands heterogeneous enhancement pattern
• 98% occur in the abdomen
• Usually unilateral and benign Adrenal metastasis
• Extra-adrenal phaeochromocytomas
are considered to be paragangliomas; • 1–2% of patients have incidental adrenal
up to 40% of these are malignant. Most masses on cross-sectional imaging

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214 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• In patients with known cancer of any type, Causes


38–57% of detected adrenal nodules are • 70% due to adrenocorticotropic hormone
metastatic (ACTH) secreting pituitary adenoma
• Lung, breast, renal and melanoma are • 5% due to ectopic ACTH secretion by lung,
commonest primary sites islet cell pancreatic or ovarian tumours
• Metastases are commonly larger; • 20% due to adrenal adenomas (usually
heterogeneous and poorly marginated 2–4 cm in size)
• Frequently bilateral. Thick irregularly • 5% due to adrenal cortical carcinoma (only
enhancing rim may be seen 10% of these cause Cushing’s syndrome)
Imaging features Imaging features
• CT: delayed contrast washout • CT is the modality of choice in
• PET: considered positive if FDG uptake corticotropin-independent Cushing’s
greater than or equal to liver uptake syndrome
–– False positives occur in around 5% of • Cortisol-producing adenomas have
adenomas, especially those that are a characteristic low density and the
functional remainder of the glands becomes atrophic
–– Phaeochromocytoma, inflammatory due to ACTH-induced suppression of
conditions and cysts may also cause activity
false positive PET results • In adrenal hyperplasia due to excess ACTH
–– False negatives can occur with lesions secretion, diffuse bilateral enlargement
< 1 cm, metastasis from carcinoid without a focal lesion is the most
and bronchioloalveolar carcinoma, commonly encountered pattern
haemorrhage, necrosis and metastasis • Adrenal venous sampling is a useful
from clear cell renal carcinoma additional test if no cause is identified on
• PET-CT has better diagnostic accuracy CT
than either dynamic CT or MRI
• Chemical shift MRI: most metastatic
lesions do not show signal dropout Conn’s syndrome
unlike adenomas. False positives can • Results from hypersecretion of aldosterone
occur with ‘fat poor’ adenomas and • Causes hypertension, hypokalaemia,
phaeochromocytomas polyuria and hypernatremia
• Female predilection with peak incidence
Adrenal lymphoma in the fourth and fifth decade
• 70% due to aldosterone-secreting
• Most commonly occurs in patients with
adenoma
non-Hodgkin’s lymphoma
• Remainder mostly due to adrenal cortical
• Bilateral involvement is common (70%)
hyperplasia
• Diffuse glandular enlargement or more
• Usually < 2 cm in size if caused by an
discrete solid masses
adenoma
• Commonly associated with
lymphadenopathy Imaging features
• CT: solitary adenomas are usually small,
Cushing’s syndrome with low attenuation and eccentrically
situated
• Due to a chronic excess of circulating
• In adrenal hyperplasia, the gland is mildly
glucocorticoids
enlarged with an irregular outline (limb of
• Clinical features: obesity, plethora,
adrenal > 10 mm, but shape is maintained)
bruising, striae, muscle wasting, diabetes
• Up to one third of patients will have
mellitus (10–15%), osteoporosis,
morphologically normal glands
hypertension
• If the CT appearance is normal, venous
sampling can be useful as the tumour can
be very small

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Adrenal gland 215

Adrenal hypofunction – MEN type 1: adrenal cortical nodules


or functioning adenoma, parathyroid
• Primary: results from destruction of the hyperplasia or adenoma, pancreatic
cortex islet cell tumour, pituitary adenoma and
• Secondary: results from inadequate carcinoid tumour. Multiple lipomas and
stimulation by the pituitary gland thyroid nodules may also occur
– MEN type 2A: phaeochromocytomas,
Causes of primary adrenal
medullary carcinoma of the thyroid and
cortical failure parathyroid hyperplasia or adenoma
• Autoimmune disorders – MEN type 2B: phaeochromocytomas
• Infection (fungal or tuberculous); of the adrenal medulla and thyroid
glandular enlargement when acute; medullary carcinoma, soft tissue
calcifications if chronic neuromas, ganglioneuromatosis of the
• Drug induced intestine and Marfanoid features
• Bilateral adrenal haemorrhage • Increased incidence of extra-adrenal
• Metastases phaeochromocytomas in MEN Type 2
• Sarcoidosis • Carney’s triad: gastric leiomyogenic
• Haemochromatosis neoplasms, extra-adrenal
• Amyloidosis phaeochromocytomas or paragangliomas
and pulmonary chondromas
Multiple endocrine
neoplasia Adrenal collision tumour
• Conditions in which tumour or • Co-existing histologically different
hyperplasia of two or more endocrine tumours within the same gland as in MEN
organs occur. There are three main types. syndromes (both benign and malignant in
All are autosomal dominant the same gland)

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4.6 Uterus

Anatomy • Potential for irradiation of a very early,


unsuspected pregnancy
• Inner mucosa called the endometrium
• Middle muscular layer called the
myometrium
MRI of the uterus
• Outer serosal layer called the perimetrium and ovaries
• Internal os divides the uterus into corpus
• T2WI demonstrates the zonal anatomy
and cervix
very well in the reproductive age group
• Fallopian tubes enter the uterus at the
• Endometrium is of high signal intensity
cornua
• Junctional zone is of low signal intensity
• Fundus is located superior to the cornua
• Peripheral myometrium has intermediate
• Cervix projects into the vagina
signal intensity (higher than striated
• Bladder lies anteriorly and rectum
muscle)
posteriorly
• Cervical zonal architecture is also well
• Broad ligaments are peritoneal folds that
seen on T2WI
extend from the lateral sides of the uterus
• Epithelium and mucus are seen as central
to the pelvic side walls
high signal intensity
• Fallopian tubes and uterine arteries run in
• The fibrous stroma in the middle has low
the broad ligaments
signal intensity
• Size of uterus and endometrial thickness
• The peripheral myometrium has medium
vary with age and menstrual cycle
signal intensity
• Zonal anatomy becomes indistinct post-
Hysterosalpingography menopause
• Radiographic evaluation of uterus and
fallopian tubes
Ovary in the reproductive age
• Best method for visualising and evaluating group (T2WI)
fallopian tubes • Low signal stroma
• High signal follicles
Indications
• Infertility
• Recurrent spontaneous abortions Congenital anomalies
• Postoperative evaluation following reversal (see box opposite)
of tubal ligation or tubal ligation
• Preoperative evaluation prior to • Prevalence of 2–3%
myomectomy
Contraindications
Female infertility
• Pregnancy Causes
• Active pelvic infection • Ovulatory dysfunction in 30–40% cases
• Hyperprolactinaemia due to drugs or
Technique prolactin-producing pituitary tumours
• Performed between days 7 and 12 of the • Polycystic ovarian syndrome
menstrual cycle • Disorders of fallopian tubes in 30–40% of
• Water-soluble contrast material is used cases
Complications • Damage or obstruction to the tubes
• Bleeding • Peritubal adhesions
• Infection • Adenomyosis
• Contrast medium reaction • Leiomyoma
• Uterine injury • Endometriosis

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Uterus 217

American fertility society classification of anomalies


• Class I (absence of Müllerian ducts) • Class V
Agenesis of the vagina, uterus or uterine Septate uterus (commonest uterine
tubes, either alone or in combination anomaly). A fibrous septum may divide the
endometrial cavity alone (partial septate)
• Class II
or extends into the endocervical canal
Agenesis or incomplete development of
one Müllerian duct, e.g. unicornuate uterus • Class VI
Arcuate uterus (considered a normal
• Class III
variant)
Complete lack of fusion of the Müllerian
ducts (uterus didelphys) • Class VII
Uterine anomalies associated with
• Class IV
diethylstilbestrol exposure
Partial failure of Müllerian duct fusion
(bicornuate uterus)

Gestational Ultrasound features


• Enlarged uterus
trophoblastic disease • Multiple small anechoic areas (3–10 mm
• Chorionic tissue that undergoes hydropic diameter) in uterine cavity
change but retains the capacity to produce
chorionic gonadotrophins Differential diagnoses
• Consists of a spectrum of diseases: • Hydropic placental degeneration after
hydatidiform mole, chorioadenoma incomplete abortion
destruens (invasive mole) and malignant • Myxoid degeneration of a leiomyoma
choriocarcinoma • Retained products of conception
• Complete or classic hydatidiform mole: • Endometrial proliferative disease
hydropic enlargement of chorionic villi,
resulting in multiple vesicles of varying Choriocarcinoma
size
• Rarely associated with fetal tissue • Need not necessarily follow a pregnancy
• The majority of hydatidiform moles are • Can arise in the ovary or testis
complete moles • Pathologically there are no recognisable
• Presents with heavy painless vaginal villous structures
bleeding in the first trimester • Syncytial and cytotrophoblasts
• Hydropic placental tissue may be passed are interspersed between areas of
per vaginum haemorrhage and necrosis
• Severe hyperemesis gravidarum may be • Haematogenous dissemination to
the presenting feature the lungs, liver, kidneys, brain and
• Uterine size is usually large for gestational gastrointestinal tract can occur
age Ultrasound features
• Ovarian cysts (theca lutein) are common • Sonographic appearance is similar to
• Partial or incomplete moles present with a hydatidiform mole
dysmorphic, frequently triploid, fetus • Myometrial invasion and metastases may
• Chorioadenoma destruens is locally be evident
invasive, but nonmetastatic (represents • Theca lutein cysts that fail to resolve 3–4
< 10% of gestational trophoblastic disease) months after evacuation of the uterus are
suggestive of residual or metastatic disease

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218 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Pelvic inflammatory disease due to obstruction at the fimbria


• Tubes may not be seen if the obstruction is
• Multibacterial infectious diseases of the at the uterine end
genital tract, ovary or pelvic peritoneum • Tubal sacculation with infiltration of
• Symptoms: lower abdominal pain, vaginal contrast material around the fallopian tube
discharge, cervical motion tenderness, • Focal irregularity and areas of tubal
adnexal tenderness, dyspareunia calcification
• Usually begins as cervicitis • Rigid shortened fallopian tubes
• Secondarily involves the endometrial • Synechiae in the uterine cavity
cavity and fallopian tubes • Complete obliteration of the uterine cavity
• Tubal spillage results in local peritonitis may occur in late-stage disease
and oophoritis
• Adnexal adhesions can result causing
fusion of the tube and ovary
Endometrial hyperplasia
• Necrosis of the inflamed mass can result in • Overgrowth of normal endometrium due
tubo-ovarian abscess to unopposed persistent oestrogen
• May occur during periods of infrequent
Imaging features ovulation, administration of exogenous
• Variable appearance depending on oestrogens and oestrogen-producing
extent and severity of involvement and ovarian neoplasms
development of complications • Tamoxifen therapy can cause endometrial
Ultrasound hyperplasia
• Up to one third of patients have normal • Low risk of progression to endometrial
examination carcinoma
• Enlarged and ill-defined uterus
• Excessive fluid in the endometrial cavity
• Indistinct central endometrial echo
Endometrial polyp
complex • Polypoid mass originating in the
• Tubes visible with thick echogenic walls endometrium
• Hydro- or pyosalpinx visualised as cystic • May be a true polyp, submucosal fibroid or
adnexal masses endometrial carcinoma
• Loculated collections in the adnexa or • Can cause bleeding
pouch of Douglas • In postmenopausal women, 10% of
endometrial polyps are malignant
CT
• Nonspecific features
• Bilateral low-attenuation adnexal masses Endometrial carcinoma
with thickened walls representing tubo-
• Most common gynaecological malignancy
ovarian complex
• Predominantly affects postmenopausal
• Enlarged ill-defined uterus
women
• Hydrosalpinx may appear as fluid-filled
• Prolonged unopposed oestrogen activity is
multicystic mass
a major risk factor
• Increased density of pelvic fat
• Usually presents with abnormal vaginal
• Thickening of uterosacral ligaments
bleeding
• Pelvic ascites
• 85% are adenocarcinomas
• Histologic grade of neoplasm, depth of
Uterine tuberculosis myometrial invasion and presence of
metastatic lymphadenopathy influence
• Commonly involves the fallopian tubes
prognosis
Hysterosalpingogram features • Local invasion, lymphatic spread to
• Almost always bilateral and asymmetrical aortocaval and pelvic lymph nodes, and
• Flask-shaped dilatation of fallopian tubes haematogenous metastasis to liver, lungs
or brain are common routes of spread

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Uterus 219

Ultrasound features Complications


• Thickening of the endometrial stripe Gastrointestinal implants
> 8 mm (postmenopausal), > 15 mm • Double-contrast barium enema: variable
(premenopausal) appearance. Often asymmetrical, with a
• Thinning of the inner myometrium puckered or crenulated appearance to the
adjacent to the echogenic endometrium is affected wall
suggestive of myometrial invasion • Adhesions: difficult to define radiologically
• Obliteration of the hypoechoic layer (fixed pelvic organs are suggestive)
suggests deep myometrial invasion
Cyst rupture
• Uncommon (can occur during pregnancy)
Endometriosis
Malignant transformation
• Primarily affects women of reproductive • Rare (< 1%)
age (mean age at diagnosis 25–29 years) • 75% arise from endometriosis of the ovary
• 24% of women with pelvic pain may have • Endometrioid carcinoma is the most
endometriosis, 20% of women undergoing common
laparoscopy for infertility investigation
may have endometriosis Imaging features
• Obstructive Müllerian duct anomalies Ultrasound
of the uterus or vagina account for most • Indicated for diagnosis of endometriotic
cases of endometriosis in girls aged < 17 cysts
years • Majority of cysts exhibit diffuse low-level
• Around 5% of cases occur in internal echoes
postmenopausal women • Classic endometrioma is a homogeneous,
• Caused by functioning endometrium hypoechoic focal ovarian lesion
located outside the uterus • Some cysts are anechoic an can mimic a
• Ovaries are the most common sites simple ovarian cyst
involved • May be unilocular or multilocular
• May also involve the gastrointestinal tract, • Thin or thick septations may be seen
urinary tract, chest and soft tissues • Solid wall nodules may be seen and mimic
• Endometriosis of the gastrointestinal neoplasia
tract occurs in 12–37% patients with • Hyperechoic foci in the wall may be seen
endometriosis (rectosigmoid colon is most and is predictive of endo­metrioma
common site involved) • Lesions that resolve on follow-up
• Pathologic findings depend on duration of examinations are unlikely to be
disease and depth of penetration of lesions endometriomas
• Mature endometriotic tissue initiates
an inflammatory response with areas
Differential for ultrasound
of haemorrhage, fibrosis and adhesion
appearances in endometriosis
formation
• Dermoid cyst
Symptoms
• Haemorrhagic cyst
• Some patients are asymptomatic
• Cystic neoplasm
• Others may present with pelvic pain,
dysmenorrhoea, dyspareunia, infertility,
adnexal masses or rectal discomfort
• Symptoms may not correlate with the MRI
severity of the disease • T1, T2 and fat-suppressed T1WI are
routinely performed
Diagnosis • Homogeneous high signal on T1WI
• Laparoscopy is the gold standard for
diagnosis

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220 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Contrast enhancement is not particularly MRI features


useful and it should be reserved for cases • Short axis measurement of the junctional
suspicious of malignancy zone ≥ 12 mm
• Degenerated blood products may give high • Thickness of 8–12 mm is considered
signal on T1WI and T2WI indeterminate
• Loss of signal (shading) seen on T2WI is an • Hyperintense 2–4 mm foci in the thickened
important feature junctional zone on T2WI
• Shading can range from faint, dependent • Often oriented parallel to the endometrial
layering to complete signal void strip
• The fibrous nature of the cyst wall gives a
low signal on both T1WI and T2WI
• Other high signal intensity lesions on Fibroid uterus (leiomyoma)
T1WI include dermoids, mucinous cystic • Common benign neoplasm, occurring in
neoplasms and haemorrhagic masses 20–40% of women
• Dermoid cysts demonstrate chemical • Derived from myometrial smooth muscle
shift artefact and signal dropout on fat • Most women are asymptomatic
suppression • May present as palpable abdominal
• Signal intensity of mucinous tumours is masses or with pelvic pain, abnormal
less than that of fat or blood bleeding or infertility or with symptoms
• Haemorrhagic corpus luteal cysts have related to pressure on the bladder or
similar appearance to endometriomas, rectum
however these are usually unilocular and • Can be subserosal, submucosal or
unilateral. Haemorrhagic cysts do not intramural
exhibit shading and resolve with time • May undergo degeneration, necrosis or
calcification
Adenomyosis • Variable appearance on imaging
depending on location and degeneration
• Presence of endometrial tissue and stroma • Coarse dystrophic calcification is a
in the myometrium relatively specific feature
• Induces overgrowth of surrounding
smooth muscle Imaging features
• Typically presents in women aged 40–50 Ultrasound
years • Majority appear as focal hypoechoic
• Two thirds present with menorrhagia or masses
dysmenorrhoea • Increased echogenicity may be seen in
Focal and diffuse forms some fibroids
• Distortion of the endometrial cavity may
• Diffuse form is more common, distributed
be seen with submucosal fibroids
asymmetrically in the uterus
• Difficult to differentiate from leiomyomas MRI
on ultrasound • Low signal intensity on T2WI
• Adenomyosis is less well defined than • One third of leiomyomas demonstrate a
leiomyomas high signal intensity rim on T2WI
• Always contiguous with the junctional • Well circumscribed with well-defined
zone margins
• Margins appear ‘shaggy’ • Subserosal exophytic fibroids demonstrate
• Minimal mass effect on the endometrial a ‘bridging-vessel sign’ (presence of flow
canal voids on T1WI and T2WI from branches
of the uterine artery located between the
mass and the uterus)

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Uterus 221

Cervical carcinoma • Most are of squamous cell type


• Locally invasive neoplasm
• Invasive cervical cancer is the third most • Lymphatic spread occurs initially to
common gynaecological malignancy in the parametrial, obturator and presacral
UK nodes followed by internal, external and
• Peak age 35–50 years common iliac nodes
• Vaginal bleeding is commonest presenting • Haematogenous spread to liver, lung and
symptom bone occurs in late-stage disease

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4.7 Ovary

Anatomy Serous and mucinous


• Ovoid intraperitoneal structures, variable tumours
in size depending on age, hormonal status • Can be uni- or multilocular
and menstrual cycle • Varying amounts of solid tissue
• Volume around 3 ml before menarche,
10 ml in menstruating women and 6 ml Benign serous cystadenoma
when postmenopausal • Can be uni- or multilocular
• Mesovarium anchors ovary to posterior • Homogeneous CT attenuation or MR
surface of broad ligament signal intensity in all locules,
• Ovarian ligament anchors ovary to uterus • Thin regular wall or septum
• Suspensory ligament anchors ovary to • No endo/exocystic vegetations
pelvic side wall
• Position of ovaries is highly variable Benign mucinous
• Internal iliac artery and the ureter lie cystadenoma
posterior to the ovary
• Almost always multilocular; locules of
• External iliac vein lies superiorly and
varying intensity/density, smooth walls/
anteriorly
septae
• Ovarian artery originates from the
• Mucinous cystadenomas tend to be larger
abdominal aorta below the renal artery
than serous cystadenomas at presentation
• Left ovarian vein drains into left renal vein
• Right ovarian vein drains into inferior vena
cava Endometrioid carcinoma
• 10–15% of all ovarian carcinomas
Ovarian tumours • 15–30% associated with endometrial
(general features) carcinoma/endometrial hyperplasia
• Most common malignant neoplasm
Imaging features suggestive of a arising from endometriosis
benign tumour • Bilateral involvement in 30–50%
• Diameter < 4 cm
• Entirely cystic components Clear cell carcinoma
• Wall thickness < 3 mm • 5% of ovarian tumours
• Lack of internal structure • Majority are stage I at time of presentation
• Absence of ascites/adenopathy • Nearly all patients have previous
Imaging features suggestive of a endometriosis
• A large endometrioma with solid
malignant tumour
components suggests malignancy
• Thick irregular wall
• Thick septa MRI features
• Papillary projections • Unilocular or large cyst with solid
• Large soft tissue component with necrosis protrusions
• Pelvic organ invasion • Smooth cyst margin
• Peritoneal, mesenteric or omental • Low to high signal intensity on T1WI
implants
• Ascites
• Lymphadenopathy
Brenner tumour
• Usually small and discovered incidentally

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Ovary 223

• Rarely malignant Immature teratoma


• Associated with other ovarian tumours in
30% of cases • Contains immature tissue from all three
germ layers
Imaging features • Occurs in first two decades of life
• Multilocular cystic mass or a small mostly • Large complex mass with cystic and solid
solid mass components and scattered calcifications
• Solid components may enhance to a mild • Rapid growth
or moderate extent • Perforation of capsule is frequently
• MRI: low signal intensity (similar to demonstrated
fibroma)
• CT: extensive amorphous calcifications
may be seen
Dysgerminoma
• Occurs predominantly in young women
Germ cell tumours • Ovarian counterpart of seminoma of the
testis
• 15–20% of all ovarian tumours • Calcification may be present (typically
• Mature and immature teratomas, with a ‘speckled’ pattern)
dysgerminomas, endodermal sinus
tumour, embryonal carcinoma and Ultrasound features
choriocarcinoma • Multilobulated, solid masses with
• Only mature teratoma is benign, but it is prominent fibrovascular septa
the most common type • Necrosis and haemorrhage may appear
• Elevated serum alpha-fetoprotein and as anechoic, low signal intensity or low
human chorionic gonadotropin is seen attenuation areas
with the malignant germ cell tumours
Endodermal sinus tumour
Mature teratoma • Second decade of life
• Most common benign ovarian tumour in • Large complex pelvic mass that extends
women < 45 years of age into the abdomen
• Composed of mature tissue from two or • Both solid and cystic components
more embryonic germ cell layers • Mature teratomas may coexist with the
• Cystic teratomas are unilocular tumour
• 88% of tumours are filled with sebaceous • Rapid growth and poor prognosis
material and lined by squamous
epithelium
• Usually there is a raised protuberance
Sex cord-stromal tumours
projecting into the cavity called a • Derived from granulosa cells, theca cells,
Rokitansky nodule fibroblasts, Leydig’s cells and Sertoli’s cells
• Complications: torsion, rupture, malignant • Affects all age groups
degeneration • Represent 8% of all ovarian neoplasms
• Most are benign or confined to the ovary at
Imaging features
the time of diagnosis
• Can be purely cystic, cystic–solid, solid
• Sebaceous material, hair, bone or fat Granulosa cell tumour
appears as echogenic material on • Most common malignant sex-cord stromal
ultrasound tumour
• CT: fat attenuation with or without mural • Most common oestrogen-producing
calcification is diagnostic of mature cystic ovarian tumour
teratoma • Occurs predominantly in peri- and
• MRI: sebaceous component is high signal postmenopausal women
on T1WI

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224 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• The hyperoestrogenaemia can cause • Scattered high signal areas represent


endometrial hyperplasia, polyps or oedema or cystic degeneration
carcinoma
• Endometrial carcinoma is associated with Sclerosing stromal cell tumour
3–25% of these tumours • Benign tumour occurring predominantly
in young women
Imaging features • Large mass with both cystic and solid
• Wide range of appearances components
• Solid or multiloculated cystic tumours • Early peripheral enhancement with
• Intratumoural bleeding, infarcts and centripetal progression on contrast-
fibrous degeneration may be seen enhanced dynamic studies
• Usually confined to the ovary
Sertoli and Leydig cell tumour
Fibrothecoma • Low-grade malignancy occurring
• Lipid-rich thecoma demonstrates predominantly in young women
oestrogen activity • Most common virilising tumour
• Occurs in both pre- and postmenopausal • Composed of heterologous tissue
women • Well-defined, enhancing solid mass with
• Fibroma is the most common sex-cord intratumoural cysts
tumour
• Appears as solid mass mimicking
malignant tumours
Ovarian metastases
• Associated with ascites or Meigs’ • Colon and stomach are the most common
syndrome (unilateral pleural effusion) primary sites
• Breast, lung and contralateral ovary are the
Imaging features other frequent primaries
Ultrasound • Represent 10% of all ovarian tumours and
• Homogeneous hypoechoic mass with develop during the reproductive years
posterior acoustic shadowing • Imaging findings are non-specific

CT Kruckenberg’s tumour
• Homogeneous solid tumour with delayed • Contains mucin-secreting signet ring cells,
enhancement usually originating in the gastrointestinal
• Dense calcifications tract
MRI • Bilateral complex masses with hypointense
• Low signal intensity on T1WI and very low solid components on T1WI and internal
signal intensity on T2WI hyperintensity on T2W1

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4.8 Breast

Anatomy and embryology • Cancers are usually hypoechoic and cause


acoustic shadowing
• Modified skin gland • Implant rupture gives snow storm
• Can extend from clavicle to 8th rib and appearance if extracapsular and step
sternum to midaxillary line ladder pattern if intracapsular
• Formed by about 20 lobes which in turn
are formed of lobules Mammography
• The immediate branches of a major duct • Most widely used screening tool for the
and its lobule form the terminal duct detection of breast cancer
lobular unit—the terminal duct is thought • Needs dedicated equipment
to be where breast cancer originates
• Fibrous tissues support the glandular Anode and filters
tissue, which is progressively replaced by
fat with advancing age • Molybdenum anode is used to produce
• Develop from the mammary ridge which low-energy photons (17.9 and 19.5 keV)
in the early stages extend from the upper • A molybdenum filter is used to filter out
limb bud to the lower limb bud ventrally; the photons of energy > 20 keV
only the mid portion of the upper third of • X-ray tube windows used in
the ridge develops into mammary tissue mammography are made of beryllium to
• Accessory breast tissue may be seen reduce the amount of filtration of the low-
anywhere along the mammary ridge energy photons

Purpose of breast compression


Arterial supply, venous
drainage and lymphatics • Hold breast away from chest wall
• Reduce motion blur
• Axillary artery through the lateral thoracic
• Reduce radiation dose by reducing
artery supplies the upper outer quadrant
thickness
• Internal mammary artery supplies the
• Further reduce dose and motion by
central and medial portions
shorter exposure times
• Intercostal arteries supply the lateral
• Separate out overlapping structures
breast tissue
• Improve resolution by keeping the
• Venous drainage through axillary, internal
object closer to the detector
mammary and intercostal veins
• Reduce scatter
• Lymphatic drainage is primarily to the
• Permit more uniform exposure by
axillary nodes
providing more uniform thickness
• Small percentage into the internal
mammary nodes and the upper abdomen

Investigations Breast calcifications


Ultrasound • Large calcifications with popcorn
• Usually high-frequency transducers are appearance—involuting fibroadenoma or
used less commonly, a papilloma
• Is not useful as a screening tool • Fine curvilinear calcifications delineating
• Can be used to characterise some lesions the walls of a round or oval mass—usually
detected on mammography benign cyst
• May be used to clarify lesions seen on only • Dense lucent centred calcifications in fat
one mammographic view necrosis
• Can be used to guide needle placement in • Vascular calcifications are linear and parallel
aspirations/biopsies • Calcified rods in secretory disease

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226 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Pleomorphic calcifications < 0.5 mm when Breast cysts


associated with a mass are of concern—
intraductal cancer • Peak prevalence 30–50 years
• Fine linear calcifications in • Most represent dilatation of the lobular
comedocarcinoma acini
• Magnification mammography is used to • Some are distended ducts
further analyse calcifications • Solitary or often multiple
• Benign and malignant processes may • Carcinoma is rarely found in cysts; when
produce similar calcifications found most are papillary
• Intracystic cancer is found in < 0.2% of
cysts
Breast MRI • Pericystic leak of fluid can cause
• Breast coils are designed to image patients inflammation and fibrosis
in a prone position • May resolve spontaneously
• Small lesions are better detected with
three-dimensional sequences than two-
Imaging features
dimensional Mammography
• If possible MRI of the breast is best done • Multiple, rounded densities
between days 7 and 14 of the menstrual • May be lobulated
cycle because physiological enhancement • Usually well defined with circumscribed
is most pronounced before and margins
immediately after the menstruation • Margins may be obscured especially with
• Almost all assessments need intravenous pericystic fibrosis from inflammation
contrast • A lucent halo may be seen around (Mach
• Almost all invasive cancers enhance effect)
• Normal tissue and benign pathology also • Does not distort normal breast
may enhance architecture
• Round or oval lesions with non-enhancing • Cyst wall may calcify—egg-shell thin rim
septations are almost always fibroadenoma • Milk of calcium (concave crescent) when
• Heterogeneous enhancement is more viewed tangentially in the upright lateral
common in malignant lesions projection and concomitant amorphous
• Peripheral enhancement is more common dots of calcium when seen en face in the
with malignant lesions caudocranial projection
• Inflammatory lesions that show peripheral Ultrasound
enhancement have thinner rims • Round, ovoid, flattened or lobulated
• Cancers are low in signal intensity in T2WI • Sharply marginated with no internal
• Implant ruptures can be evaluated well with echoes
MRI • Enhanced through transmission of sound
• Bleeding into a cyst give rise to internal
Lesions that enhance on echoes and other complex appearance
contrast MRI • Complicated cysts (with internal echoes,
septations or solid areas) need close follow
• Breast cancer
up or aspiration cytology of the fluid
• Fibroadenomas
• Fibrocystic change including sclerosing MRI
adenosis • Round, oval or lobulated, well defined
• Fat necrosis • High signal on T2WI
• Radial scars • May demonstrate rim enhancement
• Mastitis following contrast (unusual and due to
• Atypical hyperplasia pericystic inflammation)
• Lobular neoplasia
• Normal breast tissue

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Breast 227

Fibroadenoma • Malignant tumours contain sarcomatous


elements
• Most common benign solid lesion of the • Mammography
breast • Generally well defined
• Common in teenage women • Spiculation does not occur
• Freely movable • Microcalcification is not a feature
• Mammography • A halo may be seen
• Sharply circumscribed • Tumours > 3 cm are likely to be malignant
• Round, ovoid or smoothly lobulated
• A thin lucent halo may be seen Imaging features
• May have one or more flattened surface Ultrasound
• Can be multiple and bilateral • Large well-circumscribed lesions
• May calcify—popcorn-shaped large • Variable low-amplitude internal echoes
calcifications are pathognomonic; but • Posterior acoustic attenuation or
fine and irregular calcification as in enhancement can occur
malignancy may be seen
• Cancer can develop in a fibroadenoma or MRI
can engulf it • Enhance rapidly
• Indistinguishable from fibroadenoma
Imaging features
Ultrasound Gynaecomastia
• Extremely variable appearance
• Non-neoplastic enlargement of the male
• Typically well-circumscribed, ovoid,
breast
hypoechoic lesions
• If breast enlargement is purely due to fat
• Lateral wall refractive shadowing may be
deposition, it is pseudogynaecomastia
seen
• With true gynaecomastia, the ducts
• Homogeneous or irregular internal echo
increase in number and may become
pattern
dilated
• Occasionally isoechoic to surrounding
• May be unilateral or bilateral
parenchyma and can be barely visible
• Frequently posterior acoustic Associations/causes
enhancement is seen • Endogenous hormonal imbalance,
• Posterior acoustic shadowing can occur exogenous hormone use, hormone-
with lesion having more fibrosis producing tumours, hepatic disease, renal
MRI disease and hyper­thyroidism
• Almost always enhances • Drugs: reserpine, cardiac glycosides,
• Gross morphology as described before spironolactone, cimetidine, thiazides,
• Non-enhancing septations may be marijuana
virtually diagnostic • Testicular tumours: seminoma,
choriocarcinoma and embryonal cell
carcinoma
Phyllodes tumour • Klinefelter’s syndrome (also associated
(cystosarcoma phyllodes) with increased risk of breast cancer)
• Unusual, usually benign stromal tumour • Lung cancer
• 25% recur locally if incompletely excised
• 10% can metastasise; metastasis is by Intraductal papilloma
haematogenous route
• One of the most common causes of serous
• Peak prevalence between 30–50 years
or bloody nipple discharge
• Rapidly enlarges
• Benign proliferation of the ductal
• Smooth lobulated contours
epithelium that projects into the lumen of
• Relatively mobile even when large
the duct and has a fibrovascular stalk

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228 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

• Usually solitary but may be multiple • Occasionally contains typical spherical


• Solitary papillomas are usually in calcification of fat necrosis
the subareolar region, and multiple
papillomas in the smaller ducts
Imaging features
peripherally Ultrasound
• Solitary papillomas are more common in • Hypoechoic and similar to subcutaneous
peri- and postmenopausal years fat
• Multiple peripheral papillomas are less • Specular reflection from the capsule may
common, usually in younger women and be seen
there is an increased likelihood for atypical • Calcifications may be present
changes and carcinoma MRI
• Imaging features cannot differentiate • Similar appearance to breast fat
between benign and malignant process • Incidental finding on MRI
Imaging features
Mammography Focal fat necrosis
• Most are not visible • May produce a palpable mass and can be
• Occasionally seen as lobulated lesion extremely hard if oil cysts are formed
forming a fairly well-circumscribed mass
• When visible, almost always located in the
Mammography
• Round or oval, lucent lesion defined by its
anterior part of the breast
capsule
• When seen within a cyst, the cyst may be
• Cysts may calcify
evident on the mammogram
• Irregular clustered deposits of calcium can
• Occasionally can produce a non-specific
occur similar to malignancy
cluster of microcalcifications
• ‘Shell-like’, lucent-centred, calcific
densities in the subareolar region Galactocoele
• May be seen as filling defects on
• Milk-containing cystic structure
ductography
• Usually associated with lactation
Ultrasound
• Solid, hypoechoic, usually lobulated
Imaging features
masses when large enough to be seen Mammography
• Occasionally located within a cystically • Indistinguishable from other fat-
dilated duct containing rounded lesions
• When seen within a cystic structure, the • Usually appears radiolucent surrounded
frond-like structure may be more obvious by dense lactating breast tissue
• Fat-fluid levels may be seen
MRI
• May enhance with contrast Ultrasound
• Varied appearance
• Thin well-defined walls
Lipoma • May contain low-level echoes
• Generally superficial, found around the • May cause shadowing
periphery of the parenchyma and always
encapsulated Hamartoma
• Freely movable and soft
• Mammography: (fibroadenolipoma)
–– Typically radiolucent • Proliferation of fibrous and adenomatous
–– Thin capsule nodular elements in fat surrounded by a
• May cause distortion of the surrounding capsule of connective tissue
architecture by displacement or
themselves become moulded by the
surrounding parenchyma

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Breast 229

Imaging features • Rods with central lucency


• Spherical or globular densities with central
Mammography
lucencies
• Sharply marginated
• May look like lipomas with fibroglandular Ultrasound
tissue within • If there is fluid in the distended duct,
• Capsule may be evident if there is fat tubular anechoic branching structure is
outside the lesion seen
Ultrasound • Duct filled with debris produces fairly
• Sharply defined homogeneous solid tubular structure
• Displaces surrounding structures
• Heterogeneous echo pattern Breast abscess
• Results from infection in the breast
Radial scar • More common in young, nursing mothers
• Idiopathic process that produces as scar- • Painful and associated with signs of
like lesion inflammation
• Should be biopsied to establish diagnosis Imaging features
Imaging features Mammogram
Mammography • Rarely indicated
• Appearance is indistinguishable from • Round and well circumscribed or irregular
cancer and ill defined
• Area of architectural distortion with • Skin and trabecular thickening
speculations radiating from a central point Ultrasound
• Microcalcifications may be seen • Can look like a cyst or solid mass
• Generally do not have a central mass • Ultrasound-guided aspiration is generally
needed to make the diagnosis
Ultrasound
• Ultrasound-guided wide-bore needle
• Appearance is indistinguishable from
aspiration is therapeutic
cancer
• Irregularly shaped hypoechoic poorly
defined tissue Ductal carcinoma
MRI • Cancers with cellular features similar to
• May have similar appearance to cancer ductal epithelium
• 90% of breast cancers
• Called ductal carcinoma in situ when
Duct ectasia confined to the duct
• Primarily affects the major ducts in the • Called invasive or infiltrating ductal
subareolar region carcinoma when the basement membrane
• Non-specific dilatation of one or more of the duct is breached
ducts that may be palpable or visible on
mammography Ductal carcinoma in situ
• Ducts become thicker due to periductal
collagen deposition from inflammation • Considered to be a precursor of
invasive ductal carcinoma
Imaging features • 30–50% of cases progress to invasive
Mammography cancer if not treated
• Tubular serpentine structures converging • Heterogeneous calcification along the
on nipple in subareolar region duct lines
• Rod-shaped calcifications may be seen • May extend through large volumes of
that may be thick continuous solid the breast
deposits; rarely branch

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230 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Imaging features is a rapid increase followed by a plateau or


rapid washout
Mammography
• Peripheral enhancement may be seen
• Fine linear branching calcifications are
very characteristic
• Calcifications can be heterogeneous or Paget’s disease
granular • Ductal carcinoma that involves the nipple
Ultrasound • Usually no tumour mass is evident
• A mass may or may not be seen • Generally favourable prognosis due to
• Calcifications may be seen as bright early presentation
reflections in hypoechoic tissue
Imaging features
MRI
• Not always evident on MRI
Mammography
• There may be no mammographic
• Extensive ductal carcinoma in situ
abnormality
can be seen as pronounced segmental
• Occasionally a mass with malignant
enhancement, but can be lost in the
characteristics
background enhancement
• Microcalcification in the subareolar region
directed towards the nipple
Invasive ductal carcinoma • Diffuse pattern of ductal calcification
• Causes desmoplastic response with Ultrasound
cicatrisation and fibrosis • Not indicated when there is nipple
• A hard palpable mass may be found abnormality
• If left untreated the lesion can ulcerate
through the skin
Tubular cancer
Imaging features
• Well-differentiated form of invasive ductal
Mammography carcinoma
• Irregular mass with spiculated margin • Sometimes palpable
• Lobulated contours are common • Frequently detected on mammography
• Calcifications in association with a typical • Slow growing
tumour mass are diagnostic
• Distortion of surrounding architecture Mammography
• Skin or nipple retraction • Small spiculated lesion
• The tumour may be undetectable on • Some contain microcalcification
mammography if the surrounding breast • Axillary lymph-node metastases are rare
tissue is of the same density as the tumour
Ultrasound
Papillary carcinoma
• Irregular hypoechoic structure that • A form of ductal malignancy
produces retrotumoural shadowing • Epithelium proliferates into villous
• Tends to be vertically oriented relative to projections and eventually fills lumen
skin • Frequency increases with age
• Can be irregular and lobulated • Slow growth rate
• Varying internal echotexture • Can infiltrate but not aggressively
• Ultrasound is used to guide needle infiltrative
positioning for interventional procedures • Generally well circumscribed and not
particularly hard
MRI
• Irregularly shaped mass with contrast Imaging features
enhancement
Mammography
• Typical pattern of contrast enhancement

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Breast 231

• May not be evident when small • Frequently has somewhat ill-defined


• Fairly well-circumscribed masses margin
• Lucent halo surrounding the mass may be Ultrasound
seen • Well circumscribed, frequently lobulated
• When within a cyst, appearance similar to • Hypoechoic
cyst • Low-amplitude internal echoes that may
• If the cancer bleeds, the cyst will be very be heterogeneous
dense on mammogram • Posterior acoustic enhancement is not
Ultrasound unusual
• When intracystic, multiple fronds MRI
projecting into the lumen of the cyst • Round with fairly well-defined borders
• Complex mass with cystic or solid • Diffuse enhancement
properties

Inflammatory carcinoma
Colloid or mucinous
• Warmth, erythema and peau d’orange
carcinoma appearance on clinical examination
• Form of ductal carcinoma with more • Appearance can be mimicked by benign
mucinous differentiation inflammatory disease
• When pure, better prognosis • Painless
• Frequency increases with age • Peak incidence around 30 years of age

Imaging features Imaging features


Mammography Mammography
• No differentiating features from other • Unusual to find a mass or calcifications
cancers • Skin thickening
• Tends to be better circumscribed • Diffuse increase in density on the affected
• Small lobulations in the contour may be side due to diffuse trabecular thickening
seen Ultrasound
• Density may be less than more scirrhous • Non-specific skin thickening
forms
MRI
Ultrasound • No distinctive findings
• Nonspecific appearance • Requires needle biopsy for confirmation
• Hypoechoic lesions
MRI Lobular carcinoma
• Lobulated tumours with slowly enhancing
• Neoplastic cells are similar to cells lining
contrast pattern
the lobule
• High signal intensity on T2WI
• Lobular carcinoma in situ if confined to
the lobule
Medullary carcinoma • Invasive lobular carcinoma if invades
• Relative frequency rises in third decade of beyond the lobule
life then decreases steadily • Women with LCIS in one breast are at
• Frequently grows quite large high risk for developing invasive lobular
• Relatively soft on physical examination carcinoma in both breasts
and tends to be freely movable
Imaging features
Mammography
• Fairly well-circumscribed smooth, round
or ovoid masses

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232 Genitourinary, adrenal, obstetric, gynaecological, & breast radiology

Ultrasound
Lobular carcinoma in situ • Hypoechoic tissue with varying degrees of
posterior acoustic shadowing
• Lower risk of progressing to invasive
cancer than ductal carcinoma in situ MRI
• Found more commonly in younger • Enhancement pattern similar to invasive
women than ductal carcinoma ductal carcinoma
• More common found in women with
radiographically dense breasts Mimics of breast cancer
on mammography
Imaging features • Post-surgical scarring
• Fat necrosis
Mammography • Radial scars
• No lesions are usually seen • Extra-abdominal desmoid tumours (very
• Calcification in adjacent benign tissue is rare)
common • Granular cell tumours (very rare)
Ultrasound
• Not well characterised Breast biopsy
• Palpable lesions can be biopsied without
Invasive lobular carcinoma imaging guidance
• More often bilateral • Any lesion that is visible by imaging can be
• Insidious in onset biopsied under guidance
• Less desmoplastic response than ductal • Lesion that is only visible on
cancers mammography can be biopsied under
• Tends to be large at diagnosis stereotactic guidance
• For lesions that need surgical excision
Imaging features biopsy, hook wire localisation can be
Mammography done under ultrasound/mammography
• Difficult to detect early guidance
• Asymmetrical density without definable • MRI can also be used to guide
margins percutaneous biopsy
• Area of progressively increasing density in
a dense breast

Ch-04.indd 232 8/9/2010 1:42:54 PM


Chapter 5

Paediatrics

5.1 Paediatric neuroradiology


5.2 Paediatric gastrointestinal radiology
5.3 Paediatric genitourinary system
5.4 Paediatric respiratory radiology
5.5 Paediatric cardiovascular radiology
5.6 Paediatric musculoskeletal radiology

Ch-05.indd 233 8/12/2010 12:18:44 PM


5.1 Paediatric neuroradiology

Neonatal cranial ultrasound Germinal matrix


Probes haemorrhage
• At least 5 mHz for term infants and 7 mHz
for premature infants Four grades of germinal matrix
haemorrhage
Standard views
Coronal views • Subependymal, no long-term
abnormalities
• Anterior to the frontal horns of the lateral
• Intraventricular, no ventricular
ventricles
dilatation
• At the level of anterior horns and sylvian
• Intraventricular with dilatation
fissure
• Intraparenchymal haemorrhage
• At the level of the third ventricle and the
intraventricular foramen, to include the
thalamus • The thin microvasculature of the germinal
• At the posterior horns to include the matrix is susceptible to rupture
choroid plexuses • Neonates born at 32 weeks’ gestation or
• Posterior and superior to the lateral earlier and those born with birth weight
ventricles (parenchyma) < 1500 g are at particular risk
Sagittal views • Germinal matrix normally involutes at
34 weeks; it is unusual to find germinal
• Midline through the third ventricle, cavum
matrix haemorrhage or intraventricular
septum pellucidum and cerebellum
haemorrhage in infants born after
• Medial to the ventricles for the
34 weeks’ gestation
caudothalamic groove
• Intracranial bleed associated with
• Through the lateral ventricles to include
prematurity
frontal, occipital and temporal horns
• Commonest site is the caudothalamic
• Lateral to each ventricle to include frontal
groove
and parietal white matter
• 90% of periventricular haemorrhages
Normal variants and pitfalls occur in the first 3 days of life
• The cavum septum pellucidum disappears Imaging features
by 2 months of age in 85% of infants
• However, its absence in the preterm
Ultrasound
• Acute blood, < 7 days (old blood appears
baby is always associated with structural
hyperechoic)
abnormalities
• The haematoma becomes less echogenic
• The choroid plexus extends from the
over time, beginning from the central
temporal horns to the foramen of Monro
portion
and then caudally into the third and fourth
• Subsequent to eventual clot retraction,
ventricles
a subependymal cyst may develop or a
• The ventricles in preterm infants appear
linear echo may result
fuller than at term, when they appear slit-
• Intraparenchymal haemorrhage
like
–– Usually located in the frontal and
• Thin-walled frontal horn cysts must
parietal lobes
not be confused with periventricular
–– Appears acutely as an echogenic
leukomalacia
homogeneous ‘mass’

Ch-05.indd 234 8/12/2010 12:18:44 PM


Paediatric neuroradiology 235

–– As the haemorrhage evolves, an Choroid plexus cyst


echogenic rim with an echo-poor
centre forms • Most common at 16 weeks’ gestation,
–– After 2–3 months, a porencephalic cyst when about 2% of fetuses will demonstrate
(if the lesion communicates with a this finding; almost always disappear by
ventricle) 26 weeks
–– Pitfalls • These cysts may be detected antenatally or
–– Intraventricular haemorrhage may postnatally
blend imperceptibly with the choroid • Known association with trisomy 18
plexus, which has a similar echo texture • Small cysts (< 10 mm in diameter) are
–– Asymmetric thickness of the choroid known to resolve spontaneously
plexus should be viewed with suspicion • Rarely, these cysts may obstruct the
–– A lack of abnormality on ultrasound foramen of Monro causing hydrocephalus
does not exclude the possibility of later
neurodevelopmental problems Craniosynostosis
• Premature fusion of one or multiple
Periventricular cranial sutures
leukomalacia • Sporadic and familial forms are known
• The sagittal suture is the most commonly
• Results from perinatal asphyxia
affected (in 50–60% of cases), followed by
• Most common in premature infants born
the coronal suture (20–30%), the metopic
at less than 32 weeks’ gestation with a birth
suture (4–10%) and the lambdoid suture
weight < 1500 g
(2–4%)
• Most commonly affects the white matter
• May result from a primary defect of
adjacent to the frontal horns of the lateral
ossification (primary craniosynostosis) or,
ventricles
more commonly, from a failure of brain
• Associated with seizures, spasticity and
growth (secondary craniosynostosis)
movement disorders
• Craniosynostosis that involves more than
• Seen acutely and subacutely as
one suture and also be associated with
hyperechoic changes around the margins
other body deformities, and is termed
of the ventricles
syndromic craniosynostosis
• With time, these areas become
• Craniosynostosis is sometimes associated
progressively less echoic and finally
with sporadic craniofacial syndromes such
hypoechoic with the development of cystic
as Crouzon’s syndrome, Apert’s syndrome,
changes
Chotzen’s syndrome, Pfeiffer’s syndrome
Imaging features or Carpenter’s syndrome
• Secondary craniosynostosis typically
Ultrasound
results from systemic disorders
• On cranial ultrasound the earliest
–– Endocrine disorders, e.g.
feature is increased echotexture in the
hyperthyroidism, hypophosphatemia,
periventricular white matter
vitamin D deficiency, renal
• The abnormal periventricular echotexture
osteodystrophy, hypercalcaemia,
of periventricular leukomalacia usually
rickets
disappears at 2–3 weeks
–– Haematological disorders that cause
• Periventricular cysts appear after 2–3
bone marrow hyperplasia, e.g. sickle
weeks
cell disease, thalassemia
• Initial cranial ultrasound findings may be
–– Inadequate brain growth, including
normal in some patients who actually go
microcephaly and its causes and
on to develop clinical and delayed imaging
shunted hydrocephalus
findings of periventricular leukomalacia

Ch-05.indd 235 8/12/2010 12:18:45 PM


236 Paediatrics

• Types of craniosynostosis
–– Scaphocephaly: early fusion of the Associations of agenesis of the
sagittal suture corpus callosum
–– Anterior plagiocephaly: early fusion of
one coronal suture • Dandy–Walker cyst
–– Brachycephaly: early bilateral coronal • Interhemispheric arachnoid cyst
suture fusion • Hydrocephalus
–– Posterior plagiocephaly: early closure of • Arnold–Chiari malformation type 2
one lambdoid suture
–– Trigonocephaly: early fusion of the
metopic suture Imaging features
MRI
Widened cranial sutures • The corpus callosum and cingulum are
typically absent or malformed
Widened cranial sutures are • High-riding third ventricle that is usually
pathological in the following contexts open superiorly to the interhemispheric
fissure. This is seen as a ‘candelabra’ on
• > 10 mm at birth coronal imaging
• > 3 mm at 2 years of age • The sulci and gyri on the medial
• > 2 mm at 3 years of age hemispheric surface have a radial or
‘spoke-like’ configuration around the third
Complete closure occurs at 30 years of ventricle
age • Probst’s bundles are longitudinal white
matter tracts that indent and invaginate
into the superomedial aspect of the lateral
• Causes ventricles
–– Normal variant • Three types of midline cysts are associated
–– Metabolic bone disease, e.g. with agenesis or hypogenesis of the corpus
hyperthyroidism, hypothyroidism, callosum:
osteogenesis imperfecta –– Type 1 is a large midline cyst that
–– Raised intracranial pressure communicates with third ventricle and
–– Infiltration of sutures: leukaemia, the lateral ventricles
lymphoma –– Type 2 is similar to type 1 but
–– Recovery from chronic illnesses and associated with cortical anomalies
prematurity (e.g. polymicrogyria, gray matter
heterotopia, schizencephaly)
Agenesis of the corpus –– Type 3 involves complex, multilocular
callosum cysts that are asymmetric and
independent of the ventricles. Cortical
• The corpus callosum forms from the malformations are uncommon.
commissural plate at the seventh week of With large cysts, the ipsilateral
gestation lateral ventricle may be compressed,
• The genu is formed before the truncus and and the contralateral ventricle
splenium may be obstructed and enlarged
• The anterior parts, splenium and rostrum (hydrocephalus).
are completely formed by the 20th week of
gestation

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Paediatric neuroradiology 237

Paediatric brain tumours • Hydrocephalus as a result of ventricular


obstruction is seen in 90% of cases
Classification • Dense homogeneous contrast
• Supratentorial tumours enhancement
–– Account for one third of cases • Atypical features
–– Most often low-grade (I or II) –– 10% are calcified
astrocytomas –– 10% have haemorrhagic components
–– Pleomorphic xanthoastrocytoma –– 10% have cystic components
• Infratentorial tumours –– 10% are non-enhancing
–– Account for two thirds of cases MRI
–– Medulloblastoma • Less specific imaging features than with
–– Juvenile pilocytic astrocytoma CT
–– Ependymoma • Typically low T1 and high T2 signal
–– Brainstem glioma • Most sensitive modality for detecting
‘drop’ metastases
Astrocytoma is the most common brain • FLAIR and T1-weighted images (T1WI)
tumour in children post-contrast are the best sequences
• Nodular surface enhancement, which
follows the gyral contours
• Besides identifying the primary lesion,
Medulloblastoma MRI is beneficial in detecting metastatic
• Commonest childhood posterior fossa lesions
tumour • To rule out drop metastases, MRI of the
• A primitive neuroectodermal tumour spine is obligatory when medulloblastoma
(PNET) is either considered or diagnosed
• Two age peaks • Imaging of the spine is best performed
–– 75% occur in first decade of life: midline before surgery in order to avoid
location (in the vermis or the roof of the postoperative artefacts, which may be
fourth ventricle) interpreted as tumour metastases
–– 25% occur in third decade of life: a • Metastases can occur in the basal cisterns
parasagittal location in the cerebellar • Both recurrent lesions and metastases
hemispheres show sparse enhancement
• Fast-growing, aggressive tumour (grade
IV) Paediatric causes of ‘drop‘
• Leptomeningeal ‘drop’ metastases are metastases
found in 40% of cases at the time of
diagnosis; these metastases can seed • Medulloblastoma
anywhere along the neuroaxis but are • Ependymoma
commonest over the cerebral convexities • Germinoma
and in the lumbosacral region • Pineoblastoma
• Bone metastases (osteoblastic) are found
in 5% of cases at the time of diagnosis
• Treatment is with surgical resection and
full neuroaxis irradiation
Juvenile pilocytic astrocytoma
• Second commonest posterior fossa
Imaging features tumour in children
CT • Slow growing (grade I)
• Non-contrast CT shows a large hyperdense • Peak age is 10 years
midline mass • Associated with neurofibromatosis type 1

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238 Paediatrics

• Commonest location is the cerebellar


hemispheres CT features of ependymoma versus
• Presents with headaches and ataxia medulloblastoma
• Can occur, but rarely, in the optic nerves or
the hypothalamus • High attenuation (ependymoma); low
• High survival rate after resection attenuation (medulloblastoma)
• Rarely calcified (ependymoma);
Imaging features commonly calcified (medulloblastoma)
CT • Rarely cystic (ependymoma);
• Well-defined, cystic mass with an avidly commonly cystic (medulloblastoma)
enhancing mural nodule
• Very similar in appearance to cerebellar
haemangioblastoma
• 10% calcify
Brainstem glioma
• Three distinct anatomical locations:
MRI diffuse intrinsic pontine tumours, tectal
• Cyst is low signal on T1WI and high signal tumours and cervicomedullary tumours
on T2-weighted images (T2WI) • Commonest location is the pons
• Mural nodule displays prominent • High-grade aggressive tumour with a poor
enhancement prognosis
Ependymoma • Intrinsic pontine gliomas carry a
particularly grave prognosis; longer
• Arises from ependymal cells lining the survival is associated with the tectal and
ventricles cervicomedullary gliomas
• 70% occur in the brain; more commonly • Present early with cranial nerve palsies
seen in children • Hydrocephalus uncommon at time of
• 30% occur in the spine; more commonly diagnosis
seen in adults
• Commonest location in the brain is within Imaging features
the fourth ventricle in children and within MRI
the lateral ventricles in adults • Cranial MRI is the diagnostic test of choice
• Slow-growing (grade II) tumour • Typical appearance of a brainstem glioma
• Does not invade through the ventricular is an expansile, infiltrative process with
walls low-to-normal signal intensity on T1WI
• Fourth ventricle tumours typically and heterogeneous high-signal intensity
extend through foramina of Luschka and on T2WI, with or without contrast
Magendie enhancement
Imaging features • Can delineate the extent of infiltration of
the leptomeninges and the surrounding
CT structures
• Large, low-attenuation midline tumour • High midbrain tumours, especially those
obliterating the fourth ventricle arising in the tectum, are typically low-
• Non-communicating hydrocephalus grade lesions by histological criteria and
• 50% contain calcifications commonly appear hypointense on T1WI
• 50% contain cystic elements and hyperintense on T2WI even without
MRI contrast enhancement
• Heterogeneous signal on T1WI and T2WI CT
• Solid components enhance • Appearance of brainstem gliomas is
variable
• CT is less sensitive than MRI and less
useful for characterising the tumours

Ch-05.indd 238 8/12/2010 12:18:45 PM


Paediatric neuroradiology 239

• Calcifications, cystic changes and Imaging features


displacement of the ventricular system can
Ultrasound
be identified; however, lower brainstem
• Echo-bright mass
lesions are often not apparent on CT scan
• Foci of calcification (in 80% of cases)
Giant cell astrocytoma • Retinal detachment (in 100% of cases)
• Subependymal tumour located at the MRI
foramen of Monro • Modality of choice
• Tumour enlarges, causing hydrocephalus • Appears as a contrasting mass
• Can transform into a high-grade • On T1WI, the vitreous humour is dark and
astrocytoma the tumour is bright
• 15% of all paediatric brain tumours • On T2WI, the vitreous humour is bright
• Other associations include tuberous and the tumour is dark
sclerosis, cardiac rhabdomyomas and • Calcifications are seen as areas of signal
bone cysts void
Imaging features Poor prognostic signs in
MRI retinoblastoma (> 50% mortality)
• Tumour is isointense on T1WI and
hyperintense on T2WI • Invasion of the choroidal layer
• Tumour avidly enhances • Lack of calcifications
• Extension along the optic nerve
Retinoblastoma • Contrast enhancement
• Commonest ocular malignancy of
childhood
• One third are hereditary, and are usually
bilateral
• Two thirds occur sporadically, and are
Arnold–Chiari
usually unilateral malformations (1 and 2)
• Clinically patients have leukocoria (white
pupil) Arnold–Chiari malformations
• Tumour mass arises from posterior aspect type 1
of the globe • Presents clinically in adults
• Propensity for extension along the optic • Small posterior fossa
nerve • Cerebellar tonsils > 5 mm below a normal-
• Meningeal metastases can occur via the sized foramen magnum
subarachnoid space • Elongated fourth ventricle, but in a normal
position
Causes of leukocoria • Obliterated cisterna magna
• Associations
• Retinoblastoma (50% of cases of –– Syringomyelia (in 25% of cases)
leukocoria) –– Hydrocephalus (in 25% of cases)
• Retinopathy of prematurity –– Fused cervical vertebrae (Kippel–Feil
• Coats’ disease syndrome)
• Persistent hyperplastic vitreous –– Fused atlanto-occipital junction
• Toxocara infection –– Platybasia
• Congenital cataract –– No myelomeningocele
–– No supratentorial abnormalities

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240 Paediatrics

Arnold–Chiari malformations • Key feature is agenesis of cerebellar vermis


with small and widely separated cerebellar
type 2 hemispheres
• Presents in newborns with respiratory • Non-communicating hydrocephalus in
distress 75% of cases
• Small posterior fossa
• Wide foramen magnum with variable
herniation of tonsils, vermis, medulla or Vein of Galen malformation
pons • Central arteriovenous malformation and
• Scalloped clivus and petrous ridges resulting varix of vein of Galen
• Dysplasia of membranous skull, which • Prenatal diagnosis with MRI and
disappears by 6 months age ultrasound
• Fourth ventricle may be elongated and • Brain hypoperfusion ensues from
low-lying or obliterated increased pressure in the central veins
• Obliterated cisterna magna • Congestive heart failure develops as a
• Associations result of the high cardiac output
–– Myelomeningocele (in 95% of cases) • Treatment is with glue embolisation
–– Non-communicating hydrocephalus (in
95% of cases)
–– Syringomyelia Tuberous sclerosis
–– Tethered cord • Autosomal-dominant condition (70% are
–– Supratentorial anomalies: thinning new mutations)
of the tentorium with a wide incisura, • Clinically presents with epilepsy and
upward herniation of the cerebellum, mental retardation
dysgenesis of the corpus callosum, a
large massa intermedia with a small Skin abnormalities
third ventricle, small gyri –– Adenoma sebaceum (butterfly
distribution)
–– Shagreen patches
Dandy–Walker –– Ash-leaf macules
malformation –– Periungual fibromas
–– Café-au-lait spots
• A sporadic condition
• Dysembryogenesis of the fourth ventricle Renal abnormalities
• Risk in subsequent pregnancies < 5% –– Multiple angiomyolipomas
• Associated anomalies seen in 90% of cases –– Multiple renal cysts
–– Lipoma or agenesis of the corpus –– Renal cell carcinoma
callosum
–– Holoprosencephaly CNS abnormalities
–– Klippel–Feil syndrome –– Multiple cortical hamartomas (in 90%
• 50% mortality quoted in first year of life of cases); the majority calcify but there
is no malignant potential
Imaging features –– Subependymal nodule
• Large posterior fossa –– Subependymal giant cell astrocytoma
• Large posterior fossa cyst connected to the
fourth ventricle Cardiac abnormalities
• Elevated tentorium cerebelli –– Cardiac rhabdomyoma (single or
multiple)

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Paediatric neuroradiology 241

Imaging features Neonatal spine


MRI • Hypoechoic central tubular structure with
• The tubers are isointense on T1WI, parallel echogenic walls
hyperintense on T2WI and very rarely • Conus is at the T12–L1 level and is
enhance continuous with the echogenic filum
• CSF pulsation is a normal finding on
Heterotopic grey matter imaging
islands
• Caused by abnormal neuronal migration
Tethered spinal cord
• Lesions are of grey matter signal intensity • Associated with spinal dysraphism,
• Typically seen as curvilinear bands fibrolipoma and a thickened ‘tight’ filum
radiating out from the ventricles (> 2 mm)
• May calcify and approximately 20% • MRI is confirmatory
enhance with contrast.

Ch-05.indd 241 8/12/2010 12:18:45 PM


Paediatric
5.2 gastrointestinal radiology

Oesophagus cysts but duplication cysts usually lie more


posteriorly, anywhere along the length of
Tracheoesophageal fistula and the oesophagus
oesophageal atresia • Associations include pulmonary cystic
malformations, oesophageal atresia and
• Presents at birth, usually after failure to
vertebral defects
pass a nasogastric tube, or with excessive
oral secretions, choking and at times Gastro-oesophageal reflux
cyanosis
• A normal intermittent phenomenon,
• Incidence is 2.4 per 10,000 births
common after meals
• Classification
• Termed GORD when the above becomes
–– Oesophageal atresia without a fistula
symptomatic with or without oesophagitis
–– Oesophageal atresia with a proximal
• Most cases of infantile reflux resolve by the
fistula
age of 1–2 years
–– Oesophageal atresia with a distal fistula
(commonest type) Imaging features
–– Oesophageal atresia with a fistula to • Retrograde flow of contrast during contrast
both pouches studies
–– H-type fistula without atresia (usually • Presence of a hiatus hernia
presents later in childhood with • Technetium-99m sulphur colloid scan
recurrent chest infections) offers direct evaluation of gastric emptying
• Associations include VACTERL (vertebral and reflux, with or without swallowing
defects, anorectal malformations, dysfunction, strictures and Schatzki’s rings
cardiovascular defects, tracheo-
oesophageal defects, renal anomalies, limb
deformities) and in 5% of cases a right-
Small intestine
sided aortic arch Pyloric stenosis
• No known association with pulmonary
• Incidence is one in 300 live births
hypoplasia
• Four times as common in males as females
Imaging features • Most commonly seen in first-born male
• Blind, air-filled upper oesophageal pouch children
causing anterior displacement and • Peak age is 3–4 weeks, and it is never after
compression of the tracheal air shadow is 3 months of age
typical • Causes a hypochloraemic metabolic
• Gas in the bowel indicates a distal fistula alkalosis
• Aspiration typical with H-type fistula • The adult form is associated with peptic
• Contrast examination, if performed, is ulcer disease and chronic gastritis
done with the child prone • Associations include tracheo-oesophageal
fistula, trisomy 18, Turner’s syndrome,
Duplication cyst rubella and erythromycin ingestion
• Congenital cysts caused by abnormal
Imaging features
embryological development leading to
sequestration of cells from the primitive Plain X-ray
foregut • Stomach > 7 cm
• Difficult to distinguish from bronchogenic • Gastric pneumatosis is seen, secondary to
increased pressure

Ch-05.indd 242 8/12/2010 12:18:45 PM


Paediatric gastrointestinal radiology 243

Ultrasound Imaging features


• Performed in the right posterior oblique
Plain X-ray
position, without emptying the stomach
• Dilated loops of bowel
• Target sign on transverse section
• Bubbly lucencies in the right lower
• Pyloric muscle thickness > 3.5–4 mm
abdominal quadrant
• Pyloric canal length > 15–18 mm
• Free gas when perforation has occurred
Contrast study • Curvilinear calcifications on peritoneal
• Indented antrum (‘shoulder’ sign) surface or lining a pseudocyst
• Narrow elongated pylorus (‘string’ sign) • Microcolon due to disuse may be seen
• Compression of the duodenal bulb
Contrast study
Duodenal atresia or stenosis • Dilute water-soluble ionic contrast enema
agents used
• Commonest cause of upper bowel
• Microcolon is seen
obstruction in neonates
• Reflux contrast in the terminal ileum
• Common locations are in the second
• Meconium pellets in the terminal ileum
or third part of the duodenum or in the
region of the ampulla of Vater Malrotation and midgut
• Strong association with Down’s syndrome volvulus
• Other associations include annular
pancreas, malrotation, congenital heart • Abnormal fixation of the small bowel
diseases, absence of the gallbladder, situs mesentery leads to a short mesentery that
inversus, preduodenal portal vein is prone to twisting
• Twisting of the small bowel about the
Imaging features superior mesenteric artery is termed
Plain X-ray midgut volvulus
• Distended stomach and proximal • Classically presents with bilious vomiting
duodenum Imaging features
• ‘Double bubble’ • Confirm abnormal position of caecum by
• In atresia, there is no distal gas; in distal barium studies (follow-through or enema)
stenosis, gas is present • In volvulus, expect a cork screw or ‘Z’-
• A diagnosis of isolated duodenal atresia shaped oesophagus that does not cross to
can only be made once midgut volvulus the left of the midline
has been excluded • Signs of perforation and ischaemia, as
Annular pancreas complications, may be present
• Ring of pancreatic tissue encircling the Omphalocoele
duodenum • Herniation of the abdominal contents into
• Child presents with nausea, vomiting, the umbilical cord
epigastric pain and jaundice • Predisposing factors
• Classical ‘double-bubble’ sign on plain –– Babies born out of in vitro fertilisation
films –– Obese mother
• Seen in the second part of the duodenum –– Mothers with bicornuate uterus
in 85% of cases • Mostly contains liver and spleen
• Associated with gastric or duodenal • Covered by peritoneum
ulceration in up to 50% of cases and • Associated with multiple-organ
recurrent pancreatitis in 15–30% of cases pathologies in about 50% of cases
Meconium ileus –– Pulmonary hypoplasia
–– Pulmonary lobar collapse
• Obstruction of the distal small bowel by
–– Lumbar lordosis
thick, tenacious meconium
–– Absent radial ray
• A typical feature of cystic fibrosis
–– Renal ectopia

Ch-05.indd 243 8/12/2010 12:18:46 PM


244 Paediatrics

–– Hydronephrosis • Important to demonstrate a normal


–– Aortic coarctation appendix in these cases
–– Absent inferior vena cava (IVC)
Acute appendicitis
Gastroschisis Imaging features
• Abdominal wall defect, off the midline and
usually right-sided Plain film
• No peritoneal investment • May be normal
• Does not contain liver • Appendicolith is seen in up to 10% of cases
• Less common occurrence than • Air–fluid levels within the bowel in the
omphalocoele right lower quadrant is a feature
• Antenatal ultrasound demonstrates free • Loss of the right psoas margin may be
floating loops of bowel in amniotic fluid shown
Ultrasound
Intussusception • Graded-compression ultrasound with a
• Telescoping of the proximal bowel into a high-frequency transducer is used
contiguous bowel segment • Appendicolith, if present, is echogenic
• Peak age is 2–20 years • Appendix is a non-compressible, blind-
• Commoner in males ended tubular structure > 6 mm in
• Ileocolic and ileoileocolic intussusceptions diameter
are the commonest forms • Fluid, phlegmon or abscess in the right
lower quadrant
Imaging features
• Length of intussusception is crucial in Necrotising enterocolitis
determining management; > 3.5 cm • Disease of premature babies. 10% of
is indicative of the need for surgical affected babies may be term
intervention • Prevalence up to 2.4 cases per 1000 live
• Target or ‘bull’s eye’ or pseudo-kidney sign births
is typical • Child typically presents with abdominal
Reduction of intussusception distension, bloody diarrhoea, sepsis and
• The ‘rule of threes’: bag 3 feet high, 3 increased gastric aspirates, typically in the
minutes per try, 3 tries first week of life
• Dilute contrast medium is generally used; • Overall mortality is 20%
air reduction may also be attempted Imaging features
• Continue reduction till there is free flow of
contrast into the terminal ileum Plain X-ray
• Contraindications to the procedure • Presence of pneumatosis is the best clue
–– Perforation • Asymmetric bowel dilatation with
–– Peritonitis separated, dilated loops of bowel
–– Henoch–Schönlein purpura • Commonest location is the right colon and
• Success rate has been quoted at 18–90% the terminal ileum
• Complications of the procedure • Portal vein gas present; its presence,
–– Perforation in 0.4% of cases however, is not associated with any higher
–– Recurrence in 10% of cases, half of mortality
which occur within 48 hours • Pneumoperitoneum with perforation is an
absolute indication for surgery
Mesenteric adenitis Contrast study
• Self-limiting lymphadenitis in the • Mucosal irregularity and ulceration, with
mesentery widely separated bowel loops
• Three or more clustered nodes measuring • Mucosa becomes permeable to contrast
> 5 mm are are diagnostic media in the presence of necrosis, and

Ch-05.indd 244 8/12/2010 12:18:46 PM


Paediatric gastrointestinal radiology 245

subsequent renal excretion of oral contrast associated with genitourinary and cardiac
is noted anomalies
• Enema is contraindicated in acute • Low malformations (infralevator) have a
necrotising enterocolitis low association with other anomalies
Ultrasound • Associations include tethered cord,
• Thickened, dilated loops of bowel VACTERL, and duodenal and oesophageal
• Vascularity of the loops of bowel is atresia
increased when they are inflamed and Imaging features
decreased when they are ischaemic
Plain X-ray
Hirschsprung’s disease • Low colonic obstruction
• Absence of parasympathetic ganglia in the • Gas bubbles in the urinary bladder and the
submucosal and intramuscular colonic vagina
plexuses • Intraluminal calcification
• Strong male preponderance • Fistulogram is performed to confirm the
• Associations include trisomy 21 and diagnosis
meconium ileus
• Extremely rare in premature infants Liver and biliary system
• Transition zone to normal ganglionic
colon is located in the rectosigmoid in up Choledochal cyst
to 80% of cases • Cystic dilatation of the extrahepatic bile
• Total colonic aganglionosis is a familial duct with segmental aneurysmal dilatation
variant in which male and females are of the common bile duct
equally commonly affected • More common in females
• Complications include necrotising
enterocolitis, caecal perforation, Todani’s classification
obstructive uropathy and enterocolitis
Imaging features • Type I: segmental or diffuse fusiform
dilatation of the common bile duct
AXR • Type II: cysts represent a diverticulum
• Features of distal bowel obstruction of the duct
• Pneumoperitoneum with perforation is • Type III: cysts represent a
seen in 4% of cases choledochocoele occurring in the
Contrast study with water-soluble medium duodenal wall and protruding as a
• Inverted cone like transition point is mass into the duodenal lumen
typical • Type IV: multiple extrahepatic bile duct
• A rectosigmoid index < 1 is diagnostic cysts
• Delayed radiographs should be done if the • Type V: cystic dilatation of the
features are not typical intrahepatic ducts; also known as
Caroli’s disease
Anorectal malformation
• Occurs as a result of failure of descent
and separation of the hindgut and the • Associations
genitourinary system in the second –– Double gallbladder
trimester –– Failure of union of left and right hepatic
• One in 5000 births ducts
• Presents with failure to pass meconium or –– Polycystic liver disease
with meconium per vagina or per urethra • Classical presentation triad as seen in 30%
• High malformations (supralevator) are of cases
–– Intermittent obstructive jaundice

Ch-05.indd 245 8/12/2010 12:18:46 PM


246 Paediatrics

–– Right upper quadrant pain


–– Palpable mass Differential diagnosis
• Complications
–– Stones • Neonatal hepatitis
–– Portal vein thrombosis • Cystic fibrosis (inspissated bile in cystic
–– Cholangiocarcinoma fibrosis may be indistinguishable from
–– Hepatic abscess biliary atresia)
–– Pancreatitis
–– Biliary cirrhosis
Imaging features MRI
• High-signal periportal fibrosis
Ultrasound • Absent hepatic ducts
• Porta hepatis cyst communicating with the
common hepatic or intrahepatic ducts
• Dilatation of the intrahepatic duct
Gastrointestinal tumours in
Hepatobiliary imino-diacetic acid (HIDA)
children
scan Infantile
• Fills in 1 hour haemangioendothelioma
• Prominent ductal activity
• Commonest benign paediatric tumour
MR cholangiogram • Presents in infants < 6 months of age in
• Helps in preoperative planning 85% of cases
• Twice as common in girls
Biliary atresia • There is a tendency to involute
• Severely deficient extrahepatic biliary tree spontaneously over months or years
• Presents in the first week of life with • Often followed with sequential ultrasound
conjugated hyperbilirubinemia • Association with cutaneous haemangioma
• Associations include trisomy 18, (in 50% of cases)
polysplenia and hepatocellular carcinoma • Complications
• Causes include hepatitis and sclerosing –– Arteriovenous shunts causing
cholangitis, in some cases the cause is congestive heart failure
unknown –– Intraperitoneal haemorrhage
• Liver biopsy provides definitive –– Disseminated intravascular coagulation
histological diagnosis –– Thrombocytopenia
• Correctable in 12% of cases, using Kasai’s
portoenterostomy (prompt diagnosis is Imaging features
essential) Ultrasound
Imaging features • Complex, hypoechoic mass

Ultrasound CT
• Normal gallbladder in 20% cases • Low-attenuation mass in the liver
• Triangular cord sign • Haemorrhage or calcification may alter the
• Echogenic fibrous tissue above the characteristics
bifurcation of portal vein • Contrast enhancement is variable
• No intrahepatic ductal dilatation MRI
HIDA scan • T1WI: low signal compared with normal
• Non-visualisation of bowel at 24 hours liver and spleen, with high-signal, intense
• Good hepatic visualisation at 5 minutes haemorrhagic areas
• Increased renal excretion • T2WI: typically high signal, but may be
heterogeneous

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Paediatric gastrointestinal radiology 247

Hepatoblastoma • Associations
–– Chronic wasting diseases
• Commonest malignant primary tumour in
–– Use of anabolic steroid medication
children
–– Sprue
• Median age of presentation < 2 years
–– Diabetes mellitus
• Commoner in males than females
–– Vasculitis
• Commonest site is the right lobe of the
–– Bartonella species infection in HIV
liver
positive patients
• Metastasises to lymph nodes, brain and
lung Imaging features
• Associated with Beckwith–Wiedemann
CT
syndrome and hemihypertrophy
• Hypodense lesions with peripheral
Imaging features enhancement on non-enhanced scans
• Strong contrast enhancement on delayed
Ultrasound
imaging, with a branching appearance
• Large mass with mixed echogenicity
caused by the vascular component
• Calcification seen in 50% of cases
• May have ‘spoke-wheel’ appearance, Double-bubble sign (causes)
owing to the presence of fibrous septa
• Duodenal atresia
CT • Annular pancreas
• Hypodense lesion with peripheral rim • Duodenal diaphragm
enhancement • Midgut volvulus
Angiogram • Preduodenal vein
• Hypervascular lesion • Superior mesenteric artery syndrome
• No arteriovenous shunting • Duplication cyst
• Adhesions
Hepatocellular carcinoma
Neonatal pneumoperitoneum
• Second commonest tumour in children
• Presents in children aged > 3 years
(causes)
• Associations • Rupture in necrotising enterocolitis
–– Glycogen storage disease • Rupture of the posterior urethral valves or
–– Galactosemia the bladder
–– Tyrosinaemia • Pneumopericardium (which may dissect
–– Biliary atresia downwards)
• Difficult to distinguish radiologically • Ruptured pneumatosis cystoides
from hepatoblastoma other than by intestinalis
clinical presentation, though it has a
lower incidence of calcification than
Cystic fibrosis
hepatoblastoma Imaging features
Plain X-ray
Miscellaneous pathologies • Meconium ileus
and tables • Dilated bowel loops with ‘soap bubble’
appearance in the right lower quadrant
Peliosis hepatis • Peritoneal or scrotal calcifications and
• Presence of multiple, blood-filled lacunar meconium pseudocyst formation seen,
spaces in the liver with intrauterine bowel rupture
• Benign lesion but may cause • Meconium plug syndrome
complications, including liver failure, • Dilated loops of bowel with distal
cholestasis, portal hypertension and liver obstruction
rupture • Signs of constipation
• Intussusception

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248 Paediatrics

Contrast study Ultrasound and CT


• Reflux • Gallstones
• Thickened duodenal folds • Fatty liver
• Peptic ulceration • Atrophic pancreas
• Microcolon (small calibre but normal • Features of intussusception, if it is present
length) in meconium ileus • Biliary cirrhosis
• Bowel wall thickening

Ch-05.indd 248 8/12/2010 12:18:46 PM


Paediatric genitourinary
5.3 system

Kidney and ureter • Horseshoe kidneys are at particular risk of


bleeding secondary to minor trauma
Renal agenesis • Associations include caudal ectopia,
• Congenital absence of the kidneys vesicoureteral reflux and hydronephrosis
• Can be unilateral or bilateral
• Renal agenesis is associated with genital
Pelvic kidney
malformations • Ascent of kidneys is hindered by the
–– Hydrometrocolpos inferior mesenteric artery
–– Vaginal atresia and vaginal septum in • Commoner on the left side
girls • Renal pelvis becomes anteriorly facing
–– Cryptorchidism, hypospadias and • Blood supply to a pelvic kidney comes
absent testes in boys from the aorta or iliac vessels

Duplex kidneys
Unilateral renal agenesis
• Duplex kidneys have 2 collecting systems
• One in 125
• One in 500 • Duplex kidneys without dilatation do not
• Failure of ureteric bud to induce need to be followed
metanephric bud • Duplex kidneys with dilatation should be
• Ipsilateral adrenal is present in 85% of evaluated by ultrasound and micturating
cases cystourethrogram after the infant has been
• Ipsilateral absence of the trigone and placed on prophylactic antibiotics
ureter
The Weigert–Meyer rule for duplex
kidneys
Bilateral renal agenesis
• The rule states that the upper pole
• Incompatible with life ureter inserts ectopically, medially and
• One in 8000 inferior to the normal position
• Commoner in boys • This ectopic ureter may terminate
• Associated with oligohydramnios, in a ureterocoele and can become
prematurity, Potter’s facies with low obstructed, causing hydronephrosis
set, floppy ears, prominent epicanthal • The lower pole ureter may reflux and
folds, micrognathia and pulmonary rarely it may cause hydronephrosis
hypoplasia

Multicystic dysplastic kidney


Horseshoe kidney • Most common cystic lesion of the kidney
• Fusion of the lower poles prevents • Results from early and complete
complete ascent of the kidneys obstruction of the ureteropelvic junction
• Increased risk of adenocarcinoma, Wilms’ • One in 4300 live births, more common in
tumour and hypertension boys

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250 Paediatrics

• Non-functional kidney replaced by


multiple cysts and dysplastic tissue Bilateral echo-bright kidneys in
• There are no connections between the neonates
cysts and no normal renal parenchyma
intervenes Never a normal variant
• Contralateral kidney shows abnormalities
in up to 50% of cases, including Causes include
ureteropelvic junction obstruction, renal • Acute tubular necrosis
dysplasia, vesicoureteral reflux and • Renal vein thrombosis
multicystic dysplastic kidney • Autosomal-recessive polcystic kidney
disease
Autosomal-recessive polycystic • Autosomal-dominant polcystic kidney
kidney disease disease
• Autosomal-recessive disorder • Tuberous sclerosis
• Bilateral symmetric cystic disease • Cytomegalovirus infection
• Medullary ductal ectasia is characteristic • Congenital syphilis
• Kidneys have decreased concentrating • Bilateral nephroblastomatosis
ability and tubular atrophy, with systemic
hypertension
• Associated with biliary duct hyperplasia Vesicoureteral reflux
and portal fibrosis
• Occurs in up to 15% of all children
• Renal changes are inversely proportional
• Primary reflux is due to immaturity or
to liver changes in terms of severity
maldevelopment of the ureterovesical
• Normal number of glomeruli
junction
Grades of reflux
Patterns of autosomal-recessive
–– Grade I: distal ureteral reflux
polycystic kidney disease
–– Grade II: reflux into the ureter and
pelvis, with normal calyces
• Perinatal: 90% of tubules affected, –– Grade III: reflux into a mildly dilated
death in <–30 days ureter and renal pelvis, with blunt
• Neonatal: 50% of tubules affected, live calyces
up to 9 months –– Grade IV: reflux into a moderately
• Infantile dilated, tortuous ureter, with significant
• Juvenile blunting of the calyces
–– Grade V: loss of papilla

Imaging features Indications for a micturating


cystourethrogram
Ultrasound
• Bright, bilateral nephromegaly
• Poor corticomedullary differentiation • Thick bladder wall
• Tiny punctuate, hyperechoic foci develop • Antenatally detected hydronephrosis
with time and correlate with renal failure • All boys < 1 year of age with a urinary
tract infection
Intravenous urogram • All boys < 3 years of age with a small
• Prolonged pyelogram, brush-like tubules kidney
and linear contrast streaks • Terminal haematuria
CT • Sibling refluxing
• Nephromegaly • Ureteric dilatation in infants
• Trapping of contrast in dilated tubules
• May be diffusely microcystic
• Small bladder

Ch-05.indd 250 8/12/2010 12:18:47 PM


Paediatric genitourinary system 251

Megaureter Tumours of the renal tract


• Primary megaureter represents an
obstructive dilatation of the ureter
Wilms’ tumour
above an aperistaltic segment near the • Commonest renal tumour of childhood
ureterovesical junction • Genetic mutations have been identified
• Resolves spontaneously • Male and females equally affected
• However, prophylactic antibiotics should • Median age at presentation is 3 years
be given • Bilateral in 5% of causes
• Usually synchronous when bilateral
• 5-year survival rates can be up to 95% in
Urethra children with favourable histology
Posterior urethral valve • Associations
– WAGR (Wilms’ tumour, Aniridia,
• Only boys are affected
Genitourinary anomalies and mental
• Abnormal mucosal folds between the
Retardation)
urethral wall and the distal end of the
– Denys–Drash syndrome
verumontanum
– Beckwith–Wiedemann syndrome
• When severe, may present in utero
– Perlman syndrome
with severe oligohydramnios,
– Hemihypertrophy
hydroureteronephrosis or ascites
• Reflux occurs in up to 60% of cases Imaging features
Imaging features Plain X-ray
• Mass predominantly in the flank with
Micturating cystourethrogram displacement of bowel gas
• ‘Gold-standard’ diagnostic study
• Abrupt transition from a dilated posterior Ultrasound
urethra to a small bulbous urethra at the • Heterogeneous mass with IVC invasion.
level of the valves Doppler ultrasound is better than MRI for
• The actual valve tissue may not be visible assessing venous involvement
CT
Prune belly syndrome • Calcification is seen in 10% of cases
• Triad of abdominal wall hypoplasia, • Enhances poorly post-contrast
cryptorchidism and dilatation of the
urinary tract Neuroblastoma
• One in 40,000, not inherited • Tumours arising from the neural crest
• Only boys are affected along the sympathetic chain, or adrenal
• 20% of affected babies die in infancy medulla
• Associations • Commonest solid neoplasm in children
–– Pulmonary hypoplasia and infants, and the third commonest
–– Polydactyly or syndactyly malignant disease in children overall
–– Malrotation • Location is unknown in 10% of cases
–– Scoliosis • Almost always bilateral
–– Congenital heart disease • Boys and girls are equally affected
–– Pneumothorax • Median age at presentation is 2 years
–– Microcephaly • Metastases are found at time of
–– Imperforate anus presentation in up to 60% of cases
Imaging features • Spreads via lymphatic and haematogenous
routes to bone marrow and skeleton
• Hypertrophied bladder with severe
• Better prognosis when the child is < 1 year
vesicoureteral reflux
old
• Tortuous ureters and renal dysplasia

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252 Paediatrics

Female pseudohermaphroditism
Staging of neuroblastoma
• 46XX
• Fetus has two ovaries
• Stage 1: localised, with complete gross
excision Male pseudohermaphroditism
• Stage 2a: localised, with incomplete • 46XY
gross excision • Causes include androgen insensitivity and
• Stage 2b: localised, with ipsilateral decreased synthesis of testosterone
nodes positive for metastases • Testes present, but no müllerian structures
• Stage 3: unresectable unilateral or • Bilateral cryptorchidism
localised unilateral disease with • Presents in adolescence with
contralateral nodes, or in the midline ‘amenorrhoea’
with bilateral extension
• Stage 4: any primary tumour with • ‘Streak gonads’ should be removed
dissemination to distant nodes promptly (because of the risk of
• Stage 4s: ‘s’ for special dissemination to gonadoblastoma)
skin, liver or bone marrow (limited to • Calcification suggests malignancy
infants < 1 year old) • Increased risk of Wilms’ tumour in true
gonadal dysgenesis

Imaging features
Plain X-ray Cryptorchidism
• Mass in abdomen
• Undescended testis
• Shows mass in the lungs in cases of
• Prevalence is 3.5% at birth, which
extension, and metastases
decreases to 0.8% by 1 year
Ultrasound • Commoner in premature children
• Solid tumour with echogenic foci of • Familial predisposition
calcification • 70% of cases are right-sided; most of the
• Echo-poor areas indicate haemorrhage or rest are bilateral
necrosis • Locations
• Encasement of vessels –– In the inguinal canal (in 70% of cases)
CT and MRI –– Prescrotal in (15% of cases)
• For full assessment of extension and –– Intra-abdominal in (15% of cases)
spread • Associations include prune belly, Prader–
Willi syndrome, Beckwith–Weidemann
Methylene diphosphonate (MDP) or syndrome, Noonan’s syndrome and
metaiodobenzylguanidine (MIBG) Laurence–Moon–Biedl syndrome
scintigram • High incidence of malignancy and
• Assesses for bone metastases infertility
• Ultrasound confirms the diagnosis
Disorders of sexual
development
Ambiguous genitalia
True hermaphroditism
• 46XX mosaic or 46XY
• Both testes and ovaries are present in the
fetus
• Rare

Ch-05.indd 252 8/12/2010 12:18:47 PM


Paediatric respiratory
5.4 radiology

Overview Neonatal chest conditions


Neonatal chest conditions Hyaline membrane disease
Diffuse conditions • Surfactant deficiency leading to collapse of
• Low lung volumes with streaky perihilar the alveoli
densities • Respiratory distress within 2 hours of life;
–– Hyaline membrane disease distress beginning after 8 hours is unlikely
–– Oxygen therapy and complication to be due to hyaline membrane disease
• Large lung volumes with streaky perihilar • Predisposing factors
densities –– Prematurity
–– Meconium aspiration syndrome –– Term infants of diabetic mothers
–– Transient tachypnoea of the newborn –– Second twins
–– Neonatal pneumonia –– Infants delivered by Caesarean section
• Treatment is with ventilation and
Focal conditions exogenous surfactant
• Congenital cystic adenomatoid • Antenatal corticosteroids given to the
malformation mother have been shown to reduce the
• Congenital lobar emphysema incidence
• Pulmonary interstitial emphysema • Postnatal exogenous surfactant via
• Congenital diaphragmatic hernia endotracheal tube has been shown to
• Pulmonary hypoplasia reduce mortality (one of its complications,
• Sequestration however, is pulmonary haemorrhage)
• Bronchogenic cyst
Imaging features
Upper airway problems in • Any opacity on plain X-ray in this age
children group should be considered as hyaline
membrane disease unless proven
• Laryngomalacia
otherwise
• Tracheomalacia
• Small-volume chest
• Tracheal stenosis
• Ground-glass appearance in the lungs with
• Epiglottitis
fine reticulonodular markings
• Laryngeal papillomatosis
• Bell-shaped thorax in the unintubated
• Croup
baby
• Foreign body aspiration
• Air bronchograms may be present and
• Juvenile angiofibroma
may extend to the periphery
Childhood chest conditions • Consolidation and pleural effusion are
• Pneumonia very rarely seen
• Chest in drowning Complications ‘due to oxygen
• Tuberculosis
• Cystic fibrosis
therapy’
• HIV infenction • Pulmonary interstitial emphysema occurs
• Immunodeficiency syndromes after 2–3 days
• Chronic granulomatous disease –– Presence of air in the lymphatic
parenchymal tissues causing
overdistension and subsequent rupture

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254 Paediatrics

–– Usually seen as bilateral curvilinear • Main differential diagnoses are group B


lucencies on plain X-ray streptococcal sepsis and total anomalous
–– However, it may occur unilaterally if the pulmonary venous drainage
endotracheal tube selectively enters • May lead to persistent fetal circulation
one bronchus during intubation • Predisposing factors
• Acquired lobar emphysema –– Elective Caesarean section (transient
• Interstitial fibrosis occurs after 28 days; tachypnoea seen in 9% of cases)
also known as bronchopulmonary –– Maternal diabetes
dysplasia –– Maternal sedation
–– Hypoproteinaemia
Evolution of bronchopulmonary –– Hypervolaemia
dysplasia –– Male sex
Imaging features
• < 4 days: mucosal necrosis
• 1 week: oedema with exudates CXR
• 2 weeks: bronchial metaplasia • Interstitial oedema (caused by fluid
• 1 month: fibrosis (mortality 40–50%) overload)
• In infants who survive, improvement • Lungs clear in 48–72 hours as a rule
in the appearance of the chest
radiographs is seen, even progression
Neonatal pneumonia
to a normal radiograph at 3–5 years in • 1 in 200 live births
about 10% of cases • Risk factors include prolonged labour,
premature rupture of membranes and
placental infection
• Group B streptococcus is the most
Meconium aspiration common causative organism (acquired in
syndrome the birth canal)
• Meconium-filled amniotic fluid breathed • Others causative organisms include
into the infant’s lungs during delivery Pseudomonas, Enterobacter,
• Significant only if aspirated to below the Staphylococcus and Klebsiella species
level of vocal cords • Empyema can develop as a complication,
• Occurs in 1% of all full-term neonates mostly due to staphylococcus or Klebsiella,
• Usually clears in 3–5 days rarely streptococci
• Complications include persistent fetal Imaging features
circulation and pulmonary hypertension • Patchy, asymmetric bilateral infiltrates
Imaging features • Pleural effusions
CXR
• Hyperinflated lungs Infants
• Atelectasis with patchy bilateral opacities
• Pneumothorax or pneumomediastinum in
Congenital cystic adenomatoid
25% of cases malformation
• CXR returns to normal by 1 year of age • Anomalous development of terminal
respiratory structures resulting in polypoid
Transient tachypnoea of the glandular lung tissue without normal
newborn alveolar differentiation
• Delayed clearance of intrauterine • Communicates with the bronchial tree
pulmonary liquids (‘wet lungs’) • Respiratory distress within first few days of
• Occurs in 5% of full-term infants life, but may present up to 1 year of age

Ch-05.indd 254 8/12/2010 12:18:47 PM


Paediatric respiratory radiology 255

• Usually confined to one lobe; equally • Sites: Left upper lobe in 40% of cases, right
common in all lobes middle lobe in 30%, right lower lobe in
• Rarely undergoes sarcomatous 20%; bilateral in 5%
degeneration • Congenital heart defects are seen in 10% of
• Can cause pulmonary hypoplasia if large cases, e.g. patent ductus arteriosus, ventral
• Associations septal defect
–– Pectus excavatum (in 25% of cases) • Can cause pulmonary hypoplasia
–– Cardiac malformations
–– Renal agenesis
Imaging features
–– Prune belly syndrome CXR
–– Jejunal atresia • Expanded hyperlucent lobe
–– Bronchopulmonary sequestration • Variable degrees of atelectasis of the
adjacent lung
Imaging features • Contralateral mediastinal shift
Antenatal ultrasound CT
• Echo-bright cysts • Hyperlucent, expanded lobe with reduced
• Fetal ascites vascularity and midline substernal lobar
CXR herniation
• Unilateral cystic mass • Usually the mediastinum is shifter to the
• Multiple fluid levels (even in the absence opposite side
of infection)
• Contralateral mediastinal shift Congenital diaphragmatic
• Spontaneous pneumothorax hernia
CT • Failure of closure of the pleuroperitoneal
• Complex cystic structure with surrounding membrane
air trapping • Males more commonly affected than
females
Causes of an echo-bright • One in 2500 live births
intrathoracic mass on antenatal • The left side is more commonly than the
ultrasound right; bilateral in 5% of cases
• Present later than 1 year of age in 10% of
cases
• Congenital diaphragmatic hernia
• Delayed onset of this condition is related
• Congenital cystic adenomatoid
to group B streptococcal infection
malformation
• Complications include pulmonary
• Intralobar pulmonary sequestration
hypoplasia and persistent fetal circulation
• Neonatal death occurs in 35% of cases, still
birth in 35–50%
Congenital lobar emphysema • Multiple associations
• Progressive lobar over-distension, caused –– CNS abnormalities: spina bifida (with
by narrowing of a bronchial segment neural tube defect in 30% of cases),
• Bronchial cartilage dysplasia or encephalocele and anencephaly
immaturity is the usual culprit –– Pulmonary hypoplasia
• Vascular compression or extrinsic mass –– Intestinal malrotation (in 95% of cases)
lesions can also lead to lobar emphysema –– Trisomy 13, trisomy 18
• Presents in the first 6 months of life –– Infections: cytomegalovirus and rubella
• Males more commonly affected than –– Cardiovascular anomalies (in 20% of
females cases)
• Idiopathic in 50% of cases

Ch-05.indd 255 8/12/2010 12:18:47 PM


256 Paediatrics

Bochdalek’s hernia versus Types of pulmonary hypoplasia:


Morgagni’s hernia primary and secondary

• Bochdalek’s hernia accounts for 90% of • Primary idiopathic pulmonary


cases; Morgagni’s hernia accounts for hypoplasia
10% of cases • Secondary bilateral or unilateral
• Bochdalek’s hernia is located pulmonary hypoplasia
posteriorly; Morgagni’s hernia is • Bilateral pulmonary hypoplasia
located anteriorly secondary to oligohydramnios or a
• Bochdalek’s hernia is commoner on the restricted chest cage (resulting from
left; Morgagni’s hernia is commoner on skeletal dysplasias)
the right • Unilateral pulmonary hypoplasia
secondary to congenital diaphragmatic
hernia, hydrothorax or Swyer–James
(McLeod) syndrome
Imaging features
• Acquired pulmonary hypoplasia
Antenatal ultrasound secondary to infection
• Echo-bright solid or cystic mass positioned
behind the left atrium and ventricle in the
thorax
• Contralateral mediastinal shift Imaging features
• Polyhydramnios Antenatal ultrasound
• Absent stomach in the abdomen • Small thorax
CXR • Oligohydramnios
• Bowel loops in the chest CXR
Oral contrast study • Small hyperlucent lung
• Used to confirm the anatomy • Diffuse pattern of scarring
Fluoroscopy
Pulmonary hypoplasia • Little change in volume with respiration
• Underdevelopment of lung parenchyma
CT
and vasculature
• Pruned appearance with mosaic pattern of
• Hypoplastic lung is supplied by the aorta
air trapping
and drained by the IVC or portal vein
• Almost always right-sided (commonest site Scimitar syndrome
is the right middle lobe) (pulmonary venolobar
• Associations
–– Diaphragmatic hernia or accessory
syndrome)
diaphragm • Aplasia or hypoplasia of one or more lobes
–– Congenital tracheal stenosis of the right lung with partial or complete
–– Bronchiectasis anomalous pulmonary venous return
–– Congenital heart disease, e.g. atrial • Absent or small pulmonary artery
septal defect, ventricular septal defect, • Males more commonly affected than
patent ductus arteriosus, tetralogy of females
Fallot • Associations include atrial septal defect,
–– Skeletal problems, e.g. osteogenesis tetralogy of Fallot, hemivertebra and rib
imperfecta, hemivertebra, hypoplastic anomalies
ribs with or without notching, early-
onset scoliosis

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Paediatric respiratory radiology 257

Imaging features Imaging features


CXR • Antenatal US: Echo-bright solid/cystic
• Classic finding is of a ‘scimitar’ vein mass within chest cavity. Colour Doppler
(shaped like a Turkish sword), seen medial can be used to identify anomalous vessels
and inferior to the IVC • CXR: Solid/cystic opacity near diaphragm.
Air fluid levels with air bronchograms if
Pulmonary sequestration infected
• Congenital mass of aberrant pulmonary • CT: Bronchial mucus plugs
tissue that has no connection with the • Solid/cystic mass
bronchial tree or the pulmonary arterial • Hyperinflated lung
system • Air-fluid levels if infected
• Typically in posteromedial segments of the
lower lobes
Bronchogenic cyst
• Two types: extralobar and intralobar • Developmental cyst lined by respiratory
• Extralobar pulmonary sequestration epithelium caused by ectopic bronchial
–– Presents with respiratory distress in budding during embryogenesis
neonates • Site: 85% occur in mediastinum when
–– Aberrant lung tissue with its own early in development. Posterior > middle >
pleural lining anterior
–– Usually airless, unless it communicates • Typically lower lobes when occurs late in
with the bowel development. Right> left
–– Males more commonly affected than Imaging features
females
–– More commonly on the left side than CXR
the right, usually adjacent to the • Oval mass projecting from the
diaphragm mediastinum
–– Arterial blood is supplied by the aorta CT
(or, rarely, by the coeliac artery) • Well-defined, fluid-filled or air-filled
–– Venous drainage is by the IVC, the cyst in the medial third of the lung or
azygos system or the portal veins mediastinum
–– Associations are common • Cysts rarely calcify
• Intralobar pulmonary sequestration
MRI
–– Presents with chest infections in older
• Fluid-filled cyst (low signal on T1WI, high
children
signal on T2WI)
–– No separate pleural lining
–– Airless or cystic lung mass
–– Arterial blood is supplied by the aorta Upper airway pathologies
–– Venous drainage is by the pulmonary
veins into the left atrium
Choanal atresia
–– Associations are uncommon (however, • Neonatal nasal obstruction due to failure
skeletal associations are more of perforation of the oronasal membrane
common) • One in 5000 live births
• Associations • Bilateral disease is commoner than
–– Pulmonary hypoplasia unilateral
–– Diaphragmatic defects • May be bony (in 90% of cases) or
–– Bronchogenic cysts membranous (10%)
–– Anomalous pulmonary venous • Causes severe respiratory distress in the
circulation neonate (immediately after birth)
–– Tracheo-oesphageal fistula

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258 Paediatrics

• Associations (75% are associated with to the hilum, simulating collapse of the
other abnormalities) left upper lobe (corresponding to the
–– Craniosynostosis mucocele)
–– Congenital heart disease
–– Tracheo-oesophageal fistula Laryngomalacia (supraglottic
–– Intestinal malrotation hypermobility syndrome)
–– Polydactyly • Laryngeal collapse with inspiration
–– Treacher–Collins syndrome, fetal secondary to infolding of the aryepiglottic
alcohol syndrome, DiGeorge’s folds
syndrome • Presents with inspiratory stridor in the first
year of life
Imaging features
• Self-limiting condition
CT • Stridor improves with activity, prone
• Axial CT is the investigation of choice positioning and neck extension
• Enlarged vomer • Usually assessed by laryngoscopy
• Fusion of the bony aspects of the pterygoid
process and the palatine bone Tracheomalacia (soft trachea)
• Narrowing of the posterior choanae to • Characterised by collapse of the trachea
< 3.4 mm with expiration
• Can be focal or diffuse
Congenital bronchial atresia • Focal disease is seen secondary to
• Characterised by obliteration of the congenital abnormalities, e.g. a vascular
proximal lumen of a segmental bronchus ring
with preservation of distal structures • Can be extrinsic (the commoner type)
• Pathogenesis unknown but proposed to be or intrinsic (caused by weakness of
secondary to vascular insult supporting structures)
• Air enters the affected segment via • Can be primary (associated with
collateral channels, producing over- chondromalacia, relapsing polychondritis
inflation and air trapping and prematurity) or secondary (associated
• Usually asymptomatic with vascular rings and mediastinal
• May present in older children with chest causes)
infections • Clinically produces expiratory or biphasic
• Commonest site is in apicoposterior stridor
aspect of the left upper lobe • Congenital diffuse tracheomalacia
• Associations improves by age 6–12 months as the
–– Congenital cystic adenomatoid structural integrity of the trachea is
malformation gradually restored
–– Lobar emphysema (however, there is no
mucus plug in this case) Imaging features
• Fluoroscopy and oesophagography are
Imaging features diagnostic
Antenatal ultrasound • Fluoroscopy shows an exaggerated
• Echo-bright mass with dilated bronchi decrease in the calibre of the trachea
during expiration
Plain X-ray
• Trapped air during expiration
accompanied by a central ‘mass’ next

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Paediatric respiratory radiology 259

• Subglottic narrowing secondary to oedema


Associations of tracheal • Distended hypopharynx and pyriform
compression sinuses
• Caution: complete obstruction of the
• Aberrant origin of the innominate epiglottis can be precipitated by handling
artery (left of the trachea in 30% of the neck of the child during radiographic
cases) positioning
• Double aortic arch (posterior
indentation from the right-sided arch Croup
more prominent) (laryngotracheobronchitis)
• Right aortic arch • Subglottic location
• Pulmonary ‘sling’ (left pulmonary • Caused by infection with parainfluenza
artery from the right pulmonary artery virus, respiratory syncytial virus or bacteria
crossing between the trachea and such as staphylococci and Clostridium
oesophagus) diphtheriae
• High cervical aortic arch • Affects children aged 6 months to 3 years
Imaging features
Tracheal stenosis CXR
• Subglottic narrowing on anteroposterior
• Can be primary or secondary view (‘steeple’ sign)
• Primary tracheal stenosis • Loss of subglottic shoulder
–– Intact tracheal rings • Anteroposterior film cannot distinguish
–– Associated with vascular ring, between croup and epiglottitis, and
pulmonary sling and H-type tracheo- a lateral view is required to exclude
oesophageal fistulae epiglottitis
• Secondary tracheal stenosis is seen with a
subglottic haemangioma (soft tissue mass Fluoroscopy
causing compression) • Shows that the narrowing is most obvious
• Present during the first year of life in 90% on inspiration
of cases, often with biphasic stridor Foreign body
• Frontal and lateral high-voltage, filtered
radiographs are used to give a clear • Typical age of presentation is 6 months to
visualisation of the stenosed segment 4 years
• Most commonly lodged in the right
Acute epiglottitis bronchus, followed by the left bronchus
• Life-threatening condition and then the larynx and trachea
• Presents with expiratory stridor and Imaging features
dysphagia • Unilateral air trapping, which indicates
• Caused by infection with Haemophilus a bronchial foreign body unless proven
influenzae type B otherwise
• Commonest age is 3–6 years • Only 10% of foreign bodies are radio-
Imaging features opaque
CXR Juvenile angiofibroma
• Lateral film shows thickened aryepiglottic • Benign vascular, locally invasive tumour
folds • Occurs almost exclusively in adolescent
• Thickened epiglottis (‘thumb’ sign) boys

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260 Paediatrics

• Treatment is with embolisation followed Childhood tuberculosis


by resection
• In children most tuberculosis is primary
• Recurrence occurs in up to 30% of cases
• Miliary tuberculosis is due to
Imaging features haematogenous spread and is more
common in post-primary tuberculosis
Plain X-ray
• Anterior bowing of the posterior wall of the Imaging features
maxillary sinus • Primary tuberculosis
• Large mass in the nasopharynx with or –– Ill-defined consolidation, which may
without bony erosion be subsegmental or lobar, involving any
CT lobe
• Best for showing bony involvement –– Typically unilateral hilar or
paratracheal lymphadenopathy
MRI
–– Pleural effusions are uncommon
• Best for showing sinus extension
• Miliary tuberculosis
–– Multiple ‘millet’-like nodules
Childhood chest conditions (1–2 mm in diameter) diffusely spread
throughout both lungs
Childhood pneumonia
• Mycoplasmal in 30% of cases, Chest in drowning
with segmental, subsegmental or • Pulmonary oedema with a normal-sized
reticulonodular interstitial infiltrates, heart
lobar involvement in the lower lobes and • Changes resolve over 5–10 days
effusions in 20% • Admission X-ray may be normal, and a
• Viral in 65% of cases, with peribronchial repeat X-ray after 24 hours is mandatory
thickening, hyperinflation and scattered • Can lead to acute respiratory distress
atelectasis syndrome (ARDS) in severe cases
• Bacterial in 5% of cases, caused by • Pneumomediastinum and pneumothorax
infection with the pneumococcus, are recognised complications
Staphylococcus aureus or Haemophilus
influenzae Cystic fibrosis
• Fungal pneumonia is associated with • Autosomal-recessive inheritance
mycetoma or allergic bronchopulmonary • Abnormality on chromosome 7
aspergillosis (seen in asthma and cystic • Commoner in Caucasians
fibrosis, with mucus plugs in the bronchi, • Affects exocrine glands (in the skin,
outlined by collateral air drift, which pancreas, chest and paranasal sinuses)
appears like a gloved finger) • In the lungs, the upper lobes are most
• Round pneumonias respond rapidly to severely affected
treatment • Early pathogens are Staphylococcus aureus
and Haemophilus influenzae
Swyer–James (MacLeod) syndrome • Late pathogens are Pseudomonas
(bronchiolitis obliterans) aeruginosa and Burkholderia cepacia
• The earliest presentation is with a
• Acquired pulmonary hypoplasia bronchiolitis-like illness, reflecting small
• Seen as a small hyperlucent lung with airway obstruction
diminished vessels (focal emphysema)
Imaging features
following a viral respiratory tract
infection CXR
• Overinflated lungs with or without air
space shadowing

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Paediatric respiratory radiology 261

• Used for severity scoring Thymus and mediastinal


• Portacath tip should be in the superior
vena cava (SVC) masses
AIDs in children Thymus
• Pneumocystis carinii, cytomegalovirus • Usual extent is from the level of left
and Aspergillus are the commonest chest brachiocephalic veins up to the level of the
pathogens pulmonary arteries
• CXR in Pneumocystis carinii pneumonia • However, in young infants it may extend
shows perihilar haze, which is progressive into the neck and down to the level of the
with air bronchograms and hyperinflation; diaphragm
pneumatocoeles, pneumothoraces may • Starts involuting at 3–4 years of age
develop later • Temporary involution seen during acute
• Cytomegalovirus causes a more prominent illnesses
reticulonodular pattern in the outer third Imaging features
of the lung
• Lymphocytic interstitial pneumonia CXR
may be the presentation of AIDS, with • Thymus is normally prominent on CXR
ground-glass attenuation, poorly defined during the first few years of life
centrilobular nodules, and thickening • Normal gland is not radio-opaque, and
of the interstitium along the lymphatic bronchial markings can be seen through it
vessels with lymphadenopathy • In the presence of a pneumomediastinum,
• Biopsy is ultimately required to establish thymic lobes may be elevated and well
the diagnosis demarcated, owing to air outlining their
inferior margins
Chronic granulomatous • A normal thymus never compresses the
disease trachea
• X-linked or autosomal-recessive condition Ultrasound
in which phagocytes can engulf but not kill • Echotexture similar to that of the liver:
organisms generally hypoechoic with linear or
• Boys are affected punctuate echoes
• Pathogens include Staphylococcus aureus, • In children < 5 years of age, seen as
Staphylococcus epidermidis, Escherichia quadrilateral with convex lateral borders
coli, Klebsiella species, Salmonella species • In children > 5 years of age, seen as
and fungi triangular with concave or straight margins
• Normal antibodies, normal cell-mediated • Nodularity is not a normal finding at any
immunity age
• Heterogeneity seen only in children
Imaging features > 10 years of age, when fatty tissue starts
• Lymphadenopathy, abscesses, fibrosis, replacing the normal gland
effusions and parenchymal or nodal • Heterogeneity, calcification and cystic
calcification changes are abnormal in children
DiGeorge’s syndrome MRI
• Congenital thymic aplasia • In children < 10 years of age, thymus seen
• Absent parathyroid glands as an intermediate signal on T1WI (similar
• Associated with congenital heart defects, to muscle and liver)
oesophageal atresia and mandibular • Seen as homogeneous mild hyperintensity
hypoplasia on T2WI
• Patients have deficient T-cell function

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262 Paediatrics

–– Enteric cyst
Thymomas –– Oesophageal tumour
–– Bronchogenic tumour
• Rare in childhood (< 5% of mediastinal –– Bronchogenic cyst
tumours)
• Usually occur in isolation, i.e. not with
myasthenia gravis
Miscellaneous useful tables
• Calcification is uncommon
Common causes of lung ‘white out’
and opaque hemithorax
Mediastinal masses • Congenital diaphragmatic hernia
• Causes of anterosuperior mediastinal • Agenesis of the lung
masses • Pleural effusion
–– Goitre • Empyema
–– Aneurysm • Atelectasis
–– Parathyroid tumour • Consolidation and large tumours (rare)
–– Oesophageal tumour
–– Angiomatous tumour
–– Teratoma Common causes of bubbly chest in
–– Thymoma infants
–– Pericardial cyst
–– Lymphoma
• Congenital cystic adenomatoid
–– Morgagni’s hernia
malformation
–– Lipoma
• Congenital diaphragmatic hernia
–– Bronchogenic cyst
• Pneumatoceles
• Causes of middle mediastinal masses
• Pulmonary interstitial emphysema
–– Lymphoma
(often bilateral)
–– Lymph node hyperplasia
• Congenital lung cysts
–– Bronchogenic tumour
• Abscesses
–– Bronchogenic cyst
• Bronchopulmonary dysplasia (often
• Causes of posterior mediastinal masses
bilateral)
–– Neurogenic tumour
–– Aneurysm

Ch-05.indd 262 8/12/2010 12:18:49 PM


Paediatric cardiovascular
5.5 radiology

Fetal circulation –– Ebstein’s anomaly


–– Tricuspid atresia
• Oxygenated blood leaves the placenta via a
solitary umbilical vein Tetralogy of Fallot
• It reaches the umbilicus in the umbilical • Four features of TOF
cord and then runs in the falciform –– Pulmonary stenosis
ligament to the porta hepatis –– Right ventricular hypertrophy
• Here it joins the intrahepatic left portal –– Large ventricular septal defect
vein and most blood passes via the ductus –– Aortic valve overrides ventricular
venosus into the left hepatic vein and the septum
IVC • Pulmonary stenosis is usually progressive,
• Blood entering the right atrium is directed with cyanosis developing at about 3
by a valve towards the foramen ovale, months of age
through which it enters the left atrium • Patients often adopt a squatting posture,
• Some blood enters the pulmonary artery, which forces blood through the pulmonary
but resistance diverts most of it via the stenosis
ductus arteriosus to the descending aorta • Multiple associations, including coronary
• Each internal iliac artery gives an umbilical artery and valvular anomalies
artery, which passes deoxygenated blood • Treatment
back to the placenta in the umbilical cord –– Blalock–Taussig shunt (from the
subclavian artery to the ipsilateral
Pulmonary plethora without pulmonary artery)
–– Surgical closure of the VSD and relief of
cyanosis the pulmonary stenosis
• Causes Imaging feature
–– Atrial septal defect (ASD)
–– Ventricular septal defect (VSD) CXR
–– Patent ductus arteriosus (PDA) • Boot-shaped heart
• Left-to-right shunts increase pulmonary • Elevated apex (caused by right ventricular
blood flow (pulmonary hypertension) if hypertrophy)
untreated • Small pulmonary trunk
• Large high-pressure shunts, e.g. VSD, PDA, • Right aortic arch in 25% of cases
can cause pulmonary hypertension within • Pulmonary oligaemia
the first few years of life, with plethora but
no cyanosis
Ebstein’s anomaly
• Smaller low-pressure shunts, e.g. ASD, • Displacement of the tricuspid valve deep
often do not present until adulthood into right ventricular cavity
• When pulmonary resistance rises above • Proximal right ventricle is atrialised
systemic resistance, shunt reversal occurs but contracts synchronously with the
with cyanosis (Eisenmenger’s syndrome) remainder of the ventricle
• Impaired ventricular function, with
cyanosis and oligaemia
Cyanosis without • Severe tricuspid regurgitation and massive
pulmonary plethora right atrial dilatation
• Associated with other structural and
• Causes
conduction defects
–– Tetralogy of Fallot (TOF)

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264 Paediatrics

• Wolf–Parkinson–White syndrome is a closure of the ductus arteriosus


common cause of death • 90% mortality in first year of without
• Mortality is 50% in the first year of life surgical intervention
Imaging features Imaging features
CXR CXR
• Box-shaped heart • ‘Egg-on-a-string’ sign
• Dilated azygous vein • Right ventricular hypertrophy
• Narrow vascular pedicle—pulmonary
Tricuspid atresia trunk lies posteriorly
• Complete agenesis of the tricuspid valve
• Fibrous tissue occupies the cleft between Congenitally corrected
the right atrium and the right ventricle transposition of the great
• Obligatory right-to-left ASD or patent arteries
foramen ovale to sustain life • Ventricles are transposed
• Most also have a small VSD allowing some • The right ventricle is on the left side; it
left-to-right flow through a hypoplastic receives blood from the left atrium and
right ventricle to the pulmonary artery empties into the pulmonary trunk
• Severe cyanosis at birth • The left ventricle is on the right side; it
• Rarely there can be pulmonary plethora (if receives blood from the right atrium and
the VSD is large) empties into the aorta
• Surgical shunt procedures • No cyanosis
–– Glenn’s anastomosis (from the SVC to
the pulmonary artery) Total anomalous pulmonary
–– Fontaine’s operation (from the right venous connection
atrium to the pulmonary artery)
• Pulmonary veins all drain to the systemic
Imaging features veins or the right atrium
• The SVC is the commonest site for the
MRI and echocardiogram
connection
• Large right atrium
• Complete left-to-right shunt: shunt-
• Hypoplastic ventricle
dependant (ASD or patent foramen ovale)
• Pulmonary vein may be obstructed,
Cyanosis with pulmonary causing low cardiac output
plethora Imaging features
• Causes CXR
–– Transposition of the great arteries • ‘Figure-of-eight’-shaped heart
–– Total anomalous pulmonary venous
connection Scimitar syndrome
–– Truncus arteriosus
–– Tricuspid atresia with a large VSD
• Partial anomalous pulmonary venous
Transposition of the great connection
arteries • Associated with a hypogenetic right
lung
• Systemic and pulmonary circulations are • Anomalous vein seen adjacent to the
in parallel right side of the heart
• Aorta arises from the right ventricle, the • Most commonly drains to
pulmonary artery from the left ventricle subdiaphragmatic IVC
• Cyanosis at birth with pulmonary plethora • Occurs almost exclusively on the right
• Shunt-dependent, i.e. relies on ASD, VSD side
or PDA to sustain life
• Treated with prostaglandin E1 to stop

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Paediatric cardiovascular radiology 265

Truncus arteriosus Imaging features


• Failure of septation of the embryonic CXR
truncus arteriosus • Right paratracheal opacity
• A single vessel drains both ventricles, • Opacity posterior to the trachea on lateral
supplying the systemic, pulmonary and views
coronary circulations CT
• Moderate cyanosis and pulmonary • Seen as the last and most posterior vessel
plethora to come off the arch
Imaging features
CXR Persistence of the primitive right arch is
• Truncus is larger than a normal ascending referred to as Kommerell’s diverticulum
aorta

Aortic arch anomalies Double aortic arch


• Only 70% of the population have the • Forms a vascular ring around the trachea
classic aortic arch branch pattern and oesophagus
• Common variations • Presents in neonates and children with
–– A ‘bovine arch’ (the commonest arch respiratory distress and/or stridor
anomaly, seen in 22% of people), in • The descending aorta is left-sided in 75%
which the left common carotid artery of cases
arises from the right brachiocephalic • The ascending aorta divides into separate
artery right and left arches, which pass on either
–– Left arch with aberrant right subclavian side of trachea; the right arch is higher
artery than the left
–– Left vertebral artery arising directly • Each arch gives a single subclavian and
from arch (seen in 5% of people) common carotid artery
–– Common origin of left common carotid • The subclavian arteries arise posterior to
and subclavian arteries (seen in 2% of the carotid arteries
people) • The right arch crosses behind the
–– Separate origin of the right common oesophagus to join the left as the
carotid and subclavian arteries (rare) descending thoracic aorta

Aberrant right subclavian Imaging features


artery Axial CT and MRI
• Four vessels evenly spaced around trachea
• The right subclavian artery arises as the
(‘four artery’ sign)
last branch of the aortic arch and ascends
obliquely towards the right side of the Contrast swallow
body • Lateral view shows posterior indentation
• It traverses the mediastinum posterior to • Frontal view shows bilateral indentations
oesophagus in 80% of cases, between the (‘reverse S-shape’)
oesophagus and the trachea in 15%, and
anterior to the trachea in 5%
Right-sided aortic arch
• Associated with congenital heart defects in • Incidence is 1%
10–15% of cases • ‘Mirror’ branching in two thirds of
• Usually asymptomatic; rarely causes cases; in order of appearance, the left
‘dysphagia lusoria’ brachiocephalic artery, the right common
carotid artery and the right subclavian
artery; associated cyanotic congenital
heart disease in 99% of cases

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266 Paediatrics

• Aberrant left subclavian artery in one third • Unilateral right-sided rib notching if
of cases; in order of appearance, the left the coarctation is proximal to the left
common carotid artery, the right common subclavian artery
carotid artery, the right subclavian artery • Unilateral left-sided rib notching if the
and an aberrant left subclavian artery; coarctation is proximal to an aberrant right
commonly associated with slings and rings subclavian artery

Coarctation of the aorta MRI


• Modality of choice
• Eccentric luminal narrowing caused by
• Clearly depicts extent of the coarctation
infolding of the aortic wall
and collateral vessels
• Most commonly a focal narrowing distal to
• T1 spin echo imaging (black blood
the origin of the left subclavian artery
imaging) in a left anterior oblique position
• Blood flow to the descending aorta is via
is suitable for measuring the aortic calibre
intercostal collateral vessels
before and after repair of the coarctation
• Rarely, a long segment of tubular
hypoplasia presenting in neonates with Pseudocoarctation
heart failure and congenital cardiac • Elongation of the ascending aorta (‘high
anomalies aortic arch’)
• Associations • Arch buckles at the insertion of the
–– Bicuspid aortic valve ligamentum arteriosum
–– Turner syndrome (45XO) • No pressure gradient across buckle
–– Berry aneurysms • Associated with trauma, hypertension,
Imaging features bicuspid aortic valve, PDA, VSD, aortic
stenosis, single ventricle and ASD
CXR
• ‘Figure three’ sign of pre- and post-stenotic Imaging features
dilatation • No rib notching
• Inferior rib notching (on third to ninth • Anteromedial deviation of the aorta and
ribs), caused by dilated intercostal oesophagus on contrast studies
collateral vessels

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Paediatric
5.6 musculoskeletal radiology

Injuries –– Birth trauma (in the middle one third of


the clavicle)
Non-accidental injury –– Generalised osteoporosis and over-
tubulation
Skull fractures seen in non- –– Osteogenesis imperfecta
accidental injury –– Rickets in an older child
–– Neuropathy
• Complex patterns with fracture Periosteal reaction in children
crossing sutures
• Physiological
• Non-parietal fracture in infants
–– Most commonly occurs in infants
• Bilateral fractures
–– Seen in long bones as smooth lines
• Delayed presentation
running parallel to the cortex. It
remains confined to the diaphysis
• Osteomyelitis, which typically involves the
Imaging features metaphyses in infants
CT • Scurvy
• Hyperacute blood may be hypodense –– Rare in children < 6 months of age
• Recent clotted blood is hyperdense, –– Osteoporosis and abnormal bleeding
evolving to isodense in about 10 days and tendency follow
becoming hypodense 3–4 weeks after the • Neuroblastoma or leukaemia
trauma • Caffey’s disease
• Extradural haematoma is infrequently –– Characteristically involves the
associated with non-accidental injury; a mandible
subdural haematoma is typical –– Cause unknown cause
–– Clinically presents with spontaneous
Limb and skeletal injuries limb pain
• Commonest fracture is diaphyseal Osteogenesis imperfecta
• Most specific fracture is metaphyseal
• High specificity injuries • Autosomal-dominant condition
–– Posterior rib • Normal mineralization but inadequate
–– Multiple ribs osteoid formation
–– Acromion of the scapula • Thin osteoporotic fragile bones
–– Pelvis • One in 40,000
• Low specificity injuries: • Males and females are equally often
–– Clavicle affected
–– Shaft fractures outside infancy • Associated with deafness and otosclerosis
–– Parietal skull fractures outside infancy • Predisposes to intracranial bleeds
• Typical abdominal injuries (reduced platelet count)
–– Traumatic pancreatic pseudocyst • Types of osteogenesis imperfecta
–– Liver laceration –– Type I: ages 2–6 years
–– Retroperitoneal haemorrhage –– Type II: congenital disease, and lethal
–– Splenic and renal injuries (though rare) –– Type III: progressive limb and spine
• Mimics of non-accidental injury deformities
–– Normal variants, e.g. nutrient foramina –– Type IV: mildest form with best
prognosis

Ch-05.indd 267 8/12/2010 12:18:49 PM


268 Paediatrics

Imaging features • Painful irregularity of the tibial tuberosity


• Bowing secondary to trauma
• Coxa valga or coxa vara • Typically bilateral
• Kyphoscoliosis • Thickened patellar tendon
• Cortical thinning • Obliteration of the infrapatellar fat pad
• Fractures with extensive callus
• Wormian bones in the skull
Cervical spine
• Vertebral scalloping in the spine • Anterior subluxation of C2 on C3
• Protrusio acetabuli in the pelvis • 25% of children under 8 years of age have
up to 3 mm of C3 on C4
Salter-Harris injuries • Anterior wedging of C3 is also a normal
• Salter-Harris described five types of growth variant
plate fractures, and this classification • Crying may increase soft tissue thickness
should be used in describing fractures that anteriorly
affect the growth plates
• Salter-Harris type I The limping child
–– May be difficult to detect radiologically
without stress views Perthes’ disease
• Salter-Harris type II • Avascular necrosis of the femoral head in
–– The fracture passes through much of children
the growth plate but includes a piece of • Peak age is 4–8 years
metaphyseal bone on one side • Bilateral in up to 10% of cases
–– The periosteum is intact on the side • Males are five times more commonly
with the metaphyseal fragment, but is affected than females
torn on the opposite side • Usually idiopathic, but may be secondary
• Salter-Harris type III to trauma
–– Includes a piece of the epiphysis
• Salter-Harris type IV Imaging features
–– Includes the metaphysis and the Plain X-ray
epiphysis • Smaller femoral epiphysis on the affected
–– Usually intra-articular, and the side, typically with sclerosis
commonest fracture of the lateral • Widened joint space (secondary to
condyle thickened intra-articular cartilage, joint
• Salter-Harris type V fluid or joint laxity)
–– Crush injury of growth plate from axial • Later, a lucent subchondral fracture line
loading appears, followed by fragmentation of the
–– Exceedingly rare femoral head
–– May lead to progressive angular
CT
deformity if part of the growth plate is
• Loss of asterisk in the centre of the femoral
damaged
head (‘asterisk’ refers to the stellate pattern
–– Growth cessation at the end of the limb
of crossing trabecula in the centre of a
may occur if the entire growth plate is
normal femoral head)
involved
–– Injuries may not be recognised until MRI
cessation of growth is noticed • Marrow signal from femoral epiphysis is
low on T1WI and high on T2WI
Osgood–Schlatter disease • Double-line sign: sclerotic rim between
• Males are more commonly affected than viable and non-viable bone is edged by a
females high-signal rim of granulation tissue
• Incidence 25%

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Paediatric musculoskeletal radiology 269

Slipped upper femoral • Pseudomonas is more typical of


penetrating trauma and Salmonella is seen
epiphysis in patients with sickle cell anaemia
• Atraumatic fracture through the physeal
plate Imaging features
• Usually one-sided Bone scan
• Associations • Positive in 24 hours of symptoms
–– Trauma (Salter-Harris type I injury) developing
–– Growth spurt (commonly seen in boys
aged 8–17 years) Plain X-ray
–– Renal osteodystrophy and rickets • Soft tissue swelling is seen initially
–– Childhood irradiation • Fat plane obliteration is seen after 3 days
–– Growth hormone or steroid treatment • Periosteal reaction is seen after 5–7 days
–– Developmental dysplasia of the hip • Involucrum formation is seen after 20 days
–– Perthes’ disease • Sequestrum is seen after 30 days; this is a
–– Endocrine problems, e.g. specific sign of active infection in chronic
hypothyroidism, hypoestrogenism, osteomyelitis
acromegaly, cryptorchidism, pituitary • In chronic osteomyelitis, a Brodie’s abscess
adenoma, parathyroid adenoma may be seen as an elongated, radiolucent
• Complications lesion in the long bone, with marked
–– Avascular necrosis (seen in 10–15% of surrounding bony sclerosis
cases) • Brodie’s abscess is typically seen in the
–– Coxa vara metaphysis of the tibia
–– Osteoarthritis (in 90% of cases) MRI
–– Discrepancy in limb length • In the early stages, infected marrow is
low in signal intensity on T1WI, with
Imaging features correlating high signals on T2WI and short
• Widened growth plate (prior to the slip) inversion time inversion recovery (STIR)
• Posteromedial displacement of head sequences
during the slip • Post-contrast enhancement is seen
• Decrease in the neck–shaft angle • In chronic cases, the signal drops on T1WI
• If long-standing, then one sees sclerosis and T2WI secondary to fibrosis, and no
and irregularity with a widened physis enhancement is seen after contrast

Childhood osteomyelitis Nutritional diseases


• Infection of the bone and bone marrow
• May occur from haematogenous spread
Rickets
(commonest cause in children), direct • Osteomalacia in the skeletally immature
inoculation from trauma or spread from patient
infection in adjacent soft tissue Imaging features
• Usually affects a single bone
• Widened and irregularly shaped
• Commonest sites are metaphyses of the
metaphyses with flaring
proximal tibia, the distal femur, the wrist,
• Cupping and fraying of the metaphyses is
the distal radius, the shoulder and the
typical
proximal humerus
• Metaphyseal spurs are also a feature
• Staphylococcus aureus is the most
• Poorly mineralised epiphyseal centres with
commonly identified causative organism
delayed appearance
• Others organisms include group B
• Periosteal reaction may be present
streptococci, other streptococci,
• Coarse trabeculation noted (a ground-
Escherichia coli and Kingella kingae
glass appearance is not a feature)

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270 Paediatrics

• Soft bones with deformities, e.g. bowing –– Early arthritis


• Risk of Salter–Harris type I fractures in –– Avascular necrosis, though this is rare
weight-bearing areas (commoner in treated cases)

Scurvy Imaging features


• Caused by a long-term deficiency of Ultrasound
vitamin C • At least 50% of the normal femoral head is
covered by acetabulum; in developmental
Imaging features dysplasia of the hip, less than 50% is
• Ground-glass osteoporosis covered
• Increased density and widening of the • The acetabular cartilage is deformed
zone of provisional calcification (white line • The alpha angle is < 60°
of Frankl)
• Metaphyseal spurs or marginal fractures Plain X-ray
(Pelkan’s sign) • Puttis triad
• Transverse radiolucent band at the –– Superolateral displacement of the
metaphysis subjacent to the zone of proximal femur
provisional calcification (scurvy line or –– Increased acetabular angle (normal
Trummerfield’s zone, which is a site of angle is 15–30°)
fractures and infarction) –– Anteversion of the femoral neck
• Parke corner sign: subepiphyseal
infarction leading to cupping of the Juvenile rheumatoid
epiphysis
• Ring of increased density surrounding the
arthritis and juvenile
epiphysis (Wimberger’s sign) chronic arthritis
• Periosteal elevation • Arthritis of unknown cause persisting for
• Sites of abnormalities include the distal at least 6 weeks in a child under 16 years of
end of femur, the proximal and distal ends age
of the tibia and fibula, the distal end of the
radius and ulna, the proximal humerus, Imaging features
and the distal ends of the ribs Plain X-ray
• Periarticular soft tissue swelling
Developmental dysplasia of • Juxta-articular osteopenia
• Periosteal reaction is commonly seen in
the hip the metacarpals and metatarsals
• One in 400 • Advanced skeletal maturity, secondary to
• Bilateral in 30% of cases local hyperaemia
• Rare in Asians and Africans • Joint ankylosis in advanced cases
• The left side is affected in 70% of cases
• Predisposing factors
–– Female sex
Down’s syndrome
–– Breech delivery (trisomy 21)
–– First-born infants • Characteristic skeletal abnormalities
–– Oligohydramnios –– Short stature
–– Positive family history –– Reduced bone mass
• Associations include sternomastoid –– Atlantoaxial instability
tumour, torticollis, talipes, scoliosis and –– 11 pairs of ribs
Down’s syndrome –– Ossification of the posterior
• Complications in untreated cases longitudinal ligament
–– Permanent dislocation or subluxation –– Two ossification centres in the
–– Pseudoarthrosis manubrium
–– Shortening of the limb –– Flared iliac wings

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Paediatric musculoskeletal radiology 271

Achondroplasia Imaging features


• Progressive narrowing of disc spaces
• Autosomal-dominant, rhizomelic, short-
• Anterior wedging
limb dwarfism
• More than three vertebral bodies affected
• Normal intelligence and life span
• Multiple Schmorl’s nodes
Imaging features
• Lumbar kyphosis in infancy, progressing
Blount’s disease
to exaggerated lordosis in adulthood • Congenital tibia vara
• Enlarged skull with narrowed cranial • Abnormal enchondral ossification
foramina and narrow foramen secondary to stress or compression
magnum leading to a communicating • Obese Afro–Carribean children presenting
hydrocephalus with painful bowed legs is a typical
• Bullet-shaped vertebral bodies in infancy, presentation
vertebra plana in adulthood Imaging features
• Squared iliac wings in the pelvis with • Fragmented medial tibial epiphysis
‘champagne glass’ inlet • Irregular medial physeal line or bridging
• Long bones are short and wide • Tibial–femoral angle > 15°
• Spinal and cranial stenosis, which are the • Metaphyseal–diaphyseal angle > 11°
major causes of morbidity
Freiberg’s disease
Congenital scoliosis • Osteonecrosis of distal end of the second
and third metatarsals
• Spinal curvature with vertebral anomalies
• Female preponderance
• Males and females are equally commonly
• Associated with hallux valgus and a short
affected
first metacarpal
• Most commonly located in the thoracic
spine Club foot (talipes equino
• Surgery is recommended for scoliotic varus)
curves that are progressing > 10° per year
• One in 1000
• Anteroposterior talocalcaneal angle < 20°
Vertebral anomalies
(normal angle is 20–40°)
• Lateral talocalcaneal angle > 35° (normal
• Hemivertebra: unilateral or anterior angle is 35–50°)
vertebral hypoplasia • Adduction and varus deformity of the
• Butterfly vertebra: central vertebral forefoot
cleft due to failure of central vertebral • Talonavicular subluxation is an associated
body development finding
• Fused or block vertebra:
embryological failure of segmentation Congenital vertical talus
• Kippel–Feil syndrome: multiple (rocker bottom feet)
cervical segmentation anomalies
• Dorsal dislocation of the navicular bone
on the talus
• Associations include meningomyelocele,
arthrogryposis and trisomies 13–18
Miscellaneous pathologies
Accelerated skeletal maturity
Scheuermann’s disease • Causes
• Vertebral osteochondrosis –– Hyperthyroidism
• Kyphosis > 35° –– Pseudohypoparathyroidism
• Thoracic spine is affected in 75% of cases –– Hypothalamic mass lesions

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272 Paediatrics

–– McCune–Albright syndrome Coxa valga


–– Idiopathic precocity
• Neck shaft angle > 135°
–– Adrenal and gonadal tumours
• May be a normal variant
–– Acrodysostosis
• Common causes of acquired form:
Wormian bones (sutural –– Juvenile rheumatoid arthritis
bones) –– Poliomyelitis
–– Diaphyseal aclasis
• Causes
–– Cleidocranial dysostosis Coxa vara
–– Pyknodysostosis • Neck shaft angle < 120°
–– Osteogenesis imperfecta • Can be congenital (noted at birth and
–– Hypothyroidism differentiable from congenital dislocation
–– Down’s syndrome of the hips by MRI), developmental
Caffey’s disease (infantile (autosomal dominant, progressive), or
acquired (traumatic)
cortical hyperostosis)
• Seen in infants aged < 6 months Imaging features
• Possible viral aetiology • Deformity in the subtrochanteric region
• Multifocal, self-limited and benign • An angle < 45° usually corrects itself,
condition whereas an angle of > 60° (or a neck shaft
angle < 110°) usually requires surgery
Imaging features
• New bone formation along the mandibles,
clavicles and ulna bones

Ch-05.indd 272 8/12/2010 12:18:50 PM


Chapter 6
Central nervous system,
and head and neck
6.1 Adult brain tumours
6.2 Paediatric brain tumours
6.3 Congenital CNS disorders
6.4 CNS infections
6.5 Stroke
6.6 Pituitary gland
6.7 Brain and spinal trauma
6.8 Vascular malformations
6.9 Demyelinating diseases
6.10 Orbit and lacrimal glands
6.11 Spine
6.12 Temporal bone
6.13 Nose and paranasal sinuses
6.14 Salivary glands
6.15 Pharynx
6.16 Larynx
6.17 Mandible
6.18 Neck
6.19 Thyroid gland
6.20 Miscellaneous topics

Ch-06.indd 273 8/12/2010 12:19:04 PM


6.1 Adult brain tumours

Supratentorial intra-axial Imaging features


tumours CT
• Low-attenuation mass
Gliomas • 90% contain calcifications
Astrocytoma Ependymoma
• 80% of adult gliomas
• 5% of adult gliomas, but much commoner
• Primarily involve white matter
in children
• Grades I and II: low grade, slow growing,
• Tumour arises from ependymal cells,
younger patients
which line the ventricles
• Grades III and IV: high grade, aggressive,
• Slow growing (grade II) tumour
older patients
• 70% occur in the brain: commoner in
• 20% calcify, making astrocytoma the
children
commonest overall cause of a calcified
• 30% occur in the spine: commoner in
brain tumour
adults
Glioblastoma multiforme (grade IV • Commonest locations in the brain:
astrocytoma) –– Children: within fourth ventricle
• Commonest primary brain tumour –– Adults: within lateral ventricles
• Worst prognosis (mean survival 12 –– Within parenchyma adjacent to
months) ventricles (cell rests)
• Peak age: sixth decade
• Associated with neurofibromatosis Supratentorial metastases
type 1, Turcot’s syndrome, Li–Fraumeni
• Two thirds of all brain metastases
syndrome
• Two thirds are multiple
Imaging features • Two thirds of patients are symptomatic
• Commonest primaries: small cell
CT and MRI
carcinoma of the lung, breast carcinoma,
• Large, usually solitary mass within cerebral
melanoma
white matter
• Imaging cannot specify tissue of origin
• Tendency to cross midline via corpus
callosum (‘butterfly’ glioma) Haemorrhagic metastases:
• Extensive oedema and mass effect
• Thick irregular rim enhancement
common primary tumours
• Small cell carcinoma of the lung
Main differential diagnoses • Melanoma
• Cerebral abscess: typically thinner, • Thyroid carcinoma
uniform rim • Renal carcinoma
• Metastases: typically multifocal • Choriocarcinoma
• CNS lymphoma: typically multifocal
Calcified metastases (rare):
Oligodendroglioma common primary tumours
• 15% of adult gliomas • Gastrointestinal tumour (mucinous
• Slow growing, solid tumour adenocarcinoma)
• Commonest location is frontal lobe white • Breast carcinoma
matter • Sarcoma
• Typically presents with seizures • After chemotherapy or radiotherapy, e.g.
lymphoma
• Squamous or adenocarcinoma of lung

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Adult brain tumours 275

High-attenuation metastases • Most are supratentorial


• Commonest site is periventricular,
on non-contrast CT: common contacting the ependymal lining
primary tumours • Symptoms are caused by mass effect:
• Lymphoma headaches, confusion, seizures
• Haemorrhagic metastases • Prognosis is very poor, with mean survival
• Calcified metastases of only a few months
Imaging features Imaging features if
CT immunocompromised
• Small spherical lesions at grey– Non-contrast CT
white matter junction with extensive • Multiple high-attenuating lesions of
(disproportionate) surrounding oedema variable size
• Most are of low attenuation on non-
contrast scans MRI
• Double-dose contrast with delayed • Lesions isointense on T1WI
imaging is most sensitive modality • Central high-signal necrotic areas on T2WI
• Small lesions display homogeneous • Extensive surrounding oedema and rim
enhancement enhancement
• Large lesions display rim enhancement • Ependymal enhancement along adjacent
(because of central necrosis) ventricular wall

MRI Imaging features if


• Most sensitive modality (especially for immunocompetent
posterior fossa lesions)
CT and MRI
• Small foci of high T2 signal with extensive
• Unifocal lesion
surrounding oedema
• Minimal surrounding oedema
• Display homogeneous or rim
• Absence of central necrosis
enhancement
• Homogeneous enhancement
• Delayed scans do not improve sensitivity

Melanoma metastases Lymphoma versus


toxoplasmosis
Imaging features • Lymphoma more likely if lesion is solitary
MRI • Lymphoma shows subependymal
• High signal on T1-weighted image (TIWI) enhancement
• Low signal on T1-weighted image (T2WI) • Lymphoma shows more uptake of
• Paramagnetic effects shorten T1 and T2 thallium-201
relaxation times • Lymphoma shows meningeal
enhancement (a late feature)
CNS lymphoma
Supratentorial extra-axial
Primary tumours tumours
• 90% of CNS lymphomas
• Most are non-Hodgkin’s B-cell lymphoma Meningioma
• Most patients are immunocompromised, • Commonest extra-axial tumour
although it can occur in • Second commonest primary brain tumour
immunocompetent patients (the commonest being glioma)
• 20% of all brain tumours, more common in
Secondary tumours females than in males
• 10% of CNS lymphomas • Arise from arachnoid cell rests (‘cap cells’)
• Occurs as part of a systemic non-Hodgkin’s • Risk factors are radiation (up to 30 years
lymphoma lag) and neurofibromatosis type 2

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276 Central nervous system, and head and neck

• Vast majority are benign and exert • 20% are isoattenuating to brain
symptoms via pressure effects parenchyma
• Parasagittal meningiomas often cause • 20% show calcifications
contralateral leg weakness • 20% show focal hyperostosis, which is
• 2% are malignant, with parenchymal reactive and not a sign of invasion
invasion and distant metastases • Variable degree of adjacent parenchymal
• Skull-base meningiomas are often brain oedema
unresectable • Display uniform and intense enhancement
Types MRI
• Globular (the majority): flat, dural-based • Isointense on T1WI and mildly
lesion with a lobulated outer margin hyperintense on T2WI
• En-plaque: sheet-like, poorly vascular • ‘Cleft sign’: thin CSF space between lesion
lesion and underlying brain
• Perilesional flow voids
Common sites • Avid, intense uniform enhancement
• 90% are supratentorial • 70% display dural tail enhancement
• Parasagittal: often occlude the superior
sagittal sinus Causes of dural tail sign
• Cerebral convexities
• Sphenoid wing • Meningioma
Uncommon sites • Schwannoma
• Dural metastases
• Suprasellar, cerebellopontine angle, optic
• Lymphoma
nerve sheath, spinal, intraventricular,
• Tuberculoma
intraosseous
• Sarcoidosis
• 1% arise outside CNS, e.g. in the paranasal
• Glioma
sinuses, mediastinum, lung
Imaging features
Plain skull X-ray MR spectroscopy
• Characteristic alanine peak
• Usually normal
• May see a focal area of hyperostosis or Catheter angiography
calcifications • Highly vascular lesion (except en-plaque
• May see an enlarged foramen spinosum, type)
which represents an enlarged middle • Supplied by meningeal branches of the
meningeal artery supplying these external carotid artery
hypervascular tumours • Early tumour blush and delayed washout

Causes of focal skull vault Recognised atypical imaging


hyperostosis features
• Intralesional cysts
• Meningioma • Ring enhancement
• Paget’s disease • Focal areas of non-enhancement
• Fibrous dysplasia
Leptomeningeal metastases
• Metastasises to pia and arachnoid mater
CT • Seen in late-stage, disseminated
• Well-circumscribed, high-attenuating, malignancy
broad-based extra-axial mass • Commoner than dural metastases

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Adult brain tumours 277

• Commonest primary tumours are breast, Imaging features


lung and melanoma
MRI
Imaging features • Contrast-enhanced MRI is most sensitive
technique
MRI
• Low signal intensity on T1WI
• Best sequences are FLAIR and T1 post-
• Variable signal intensity on T2WI
contrast
• Extensive surrounding oedema
• Nodular surface enhancement that follows
the gyral contours Cerebellar
• Commonest locations are cerebral
convexities and basal cisterns
haemangioblastoma
• Main differentials are bacterial or fungal • Benign vascular tumour of young adults
meningitis and sarcoidosis • 20% associated with von Hippel–Lindau
disease
Detection rates for leptomeningeal • Cerebellar signs are common
metastases • 40% are associated with polycythaemia
because of erythropoietin secretion
• Surgical resection is of nodule alone as
• 25% with MRI
cyst is non-neoplastic
• 50% with single lumbar puncture
• Juvenile pilocytic astrocytoma has similar
• 95% with serial lumbar punctures
imaging features
Imaging features
CT and MRI
Diploic space metastases • Large cystic mass with an enhancing mural
nodule
• Common: seen in 10% of patients with
• Prominent flow voids from vessels feeding
brain metastases
the nodule
Imaging features • No calcifications
CT Haemangioblastoma versus
• Focal destructive bone lesion
pilocytic astrocytoma
MRI • Haemangioblastomas occur in an older
• Low signal tumour within high signal fatty age group (30–40 years versus 5–15 years)
marrow on TIWI • Haemangioblastoma nodules are
hypervascular whereas pilocytic
Infratentorial intra-axial astrocytomas are hypovascular
tumours • History of von Hippel–Lindau disease
suggests haemangioblastoma
Infratentorial metastases
• One third of all brain metastases Radiation- or
• In two thirds of cases there are multiple
metastases
chemotherapy-induced
• Commonest infratentorial tumour in brain damage
adults
• Commonest location is the cerebellar
Transient white matter oedema
hemispheres • Occurs early, often within weeks of
• Most common primary tumours are small treatment
cell carcinoma of the lung, carcinoma of • Common asymptomatic incidental finding
the breast, melanoma • Resolves spontaneously
• Diffuse white-matter high signal on T2WI

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278 Central nervous system, and head and neck

Focal radiation necrosis • Increased uptake with F-18-DG PET


scanning in tumour recurrence
• Occurs late, > 6 months after treatment
• Coagulative destruction of white matter Necrotising
• Occurs adjacent to resected tumour bed leukoencephalopathy
• Mimics tumour recurrence
• Severe diffuse form of radiation injury
Imaging features • Complication of chemotherapy
MRI (intrathecal or intravenous)
• Conventional sequences are non-specific • Increased risk if combined with
• Focal high signal area on T2WI with radiotherapy
central or ring enhancement • Diffuse bilateral white-matter high signal
on T2WI
Radiation necrosis versus
tumour recurrence Other late effects of radiation
• Cerebral and cerebellar atrophy
• Choline:creatine ratio > 3 with MR
• Radiation induced tumours, e.g.
spectroscopy in tumour recurrence
meningioma, gliomas, sarcomas

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6.2 Paediatric brain tumours

Medulloblastoma Juvenile pilocytic


• Commonest posterior fossa tumour in astrocytoma
children • Second commonest posterior fossa
• A primitive neuroectodermal tumour tumour in children
• Fast growing, aggressive tumour • Peak age 10 years, associated with
• Two age peaks: neurofibromatosis type 1
–– – First decade (75%): midline (vermis, • Commonest location is in cerebellar
roof of fourth ventricle) hemispheres
–– – Third decade (25%): parasagittal • Slow growing
(cerebellar hemispheres) • Present with progressive headache, ataxia
• 40% have leptomeningeal ‘drop’ • Rarely occur in optic nerves or
metastases at time of diagnosis, which hypothalamus
can seed anywhere along the neuroaxis
but are most common over the cerebral Imaging features
convexities and lumbosacral region CT
• 5% have bone metastases (osteoblastic) at • Well-defined cystic mass with an avidly
time of diagnosis enhancing mural nodule
• Treatment is with surgical resection and • Very similar appearance to that of
full neuroaxis irradiation cerebellar haemangioblastoma
Imaging features • 10% calcify

CT MRI
• Large hyperdense midline mass with • Cyst is of low T1 and high T2 signal
homogeneous enhancement • Mural nodule displays prominent
• 90% have hydrocephalus secondary to enhancement
ventricular obstruction
• Atypical features: Ependymoma
–– – Calcified (10%)
–– – Haemorrhagic components (10%) • Slow growing tumour arising from
–– – Cystic components (10%) ependymal lining of ventricles
–– – Non-enhancing • 70% occur in the brain: commoner in
children
MRI • 30% occur in the spine: commoner in
• Typically low T1 and high T2 signal adults
• FLAIR and T1 post-contrast are best • Commonest locations in children is within
sequences for drop metastases the fourth ventricle
• Seen as nodular surface enhancement • Fourth ventricle tumours typically
following gyral contours extend through foramina of Luschka and
Magendie, and do not invade through
Other paediatric causes of ‘drop’ ventricular walls
metastases
Imaging features
• Medulloblastima CT
• Ependymoma • Large low-attenuation midline tumour
• Germinoma obliterating the fourth ventricle
• Pineoblastoma • Non-communicating hydrocephalus
• 50% contain calcifications, 50% contain
cystic elements

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280 Central nervous system, and head and neck

CT features: ependymoma versus Brainstem glioma


medulloblastoma • High-grade, aggressive tumour with a poor
prognosis
• Ependymoma is a high-attenuation • Commonest location is the pons
lesion, rarely calcified and rarely cystic • Present early with cranial nerve palsies
• Medulloblastoma is a low-attenuation • Hydrocephalus uncommon at time of
lesion, commonly calcified and diagnosis
commonly cystic
Imaging features
CT and MRI
• Asymmetric expansion of pons and
MRI
medulla
• Heterogeneous signal on T1WI and T2WI
• Effacement of pre-pontine cistern
• Solid components enhance
• Posterior displacement of fourth ventricle

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6.3 Congenital CNS disorders

Arnold–Chiari • Supratentorial anomalies


–– Thinning of tentorium, wide incisura
malformation –– Upward herniation of cerebellum
Arnold–Chiari malformation –– Dysgenesis of corpus callosum
–– Large massa intermedia with small
type 1 third ventricle
• Presents in adulthood –– Small gyri
Imaging features
• Small posterior fossa Dandy–Walker
• Normal-sized foramen magnum malformation
• Tonsillar herniation > 5 mm
• Elongated fourth ventricle in a normal • Sporadic condition
position • 50% mortality in first year of life
• Obliterated cisterna magna • Dysembryogenesis of the fourth ventricle
• Associations: • Risk in subsequent pregnancies < 5%
–– Syringomyelia (25%) • Associated anomalies (in 90% of cases):
–– Hydrocephalus (25%) –– Lipoma or agenesis of corpus callosum
–– Fused cervical vertebrae (Klippel–Feil –– Holoprosencephaly
syndrome) –– Gyral malformations
–– Fused atlanto-occipital junction –– Klippel–Feil syndrome
–– Platybasia Imaging features
• No myelomeningocele, no supratentorial
abnormalities CT and MRI
• Large posterior fossa
Arnold–Chiari malformation • Large posterior fossa cyst connected to
type 2 fourth ventricle
• Elevated tentorium cerebelli
• Presents with respiratory disease in
• Key feature is agenesis of cerebellar vermis
newborns
• Small and widely separated cerebellar
Imaging features hemispheres
• Small posterior fossa • 75% have non-communicating
• Wide foramen magnum with variable hydrocephalus
herniation of tonsils, vermis, medulla or
pons Corpus callosum anomalies
• Scalloped clivus and petrous ridges
• Dysplasia of membranous skull, which Lipoma of the corpus callosum
disappears by 6 months of age • Congenital pericallosal malformation (not
• Elongated or obliterated low-lying fourth a tumour)
ventricle • Caused by abnormal resorption of the
• Obliterated cisterna magna primitive meninges
• Associations: • Always associated with hypoplasia or
–– Myelomeningocele (95%) agenesis of corpus callosum
–– Non-communicating hydrocephalus • Asymptomatic incidental finding in 50%
(95%) • 50% have seizures, mental retardation
–– Syringomyelia • Other associations are midline frontal
–– Tethered cord lipoma and encephalocele

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282 Central nervous system, and head and neck

Imaging features –– Ophthalmic distribution: occipital lobe


angioma
CT
–– Mandibular distribution: frontal lobe
• Midline area of low attenuation (fat)
angioma
• Often contains areas of calcification
• Leptomeningeal angioma:
MRI –– Underlying cortical hemiatrophy
• Sagittal T1 is best sequence –– Age-related gyral ‘tramtrack’
• Complete or partial absence of corpus calcifications
callosum –– Overlying skull vault thickening
• High signal intensity –– Ipsilateral choroid plexus hypertrophy
• Chemical shift artefact seen at lipoma– –– Ipsilateral dilated subependymal veins
tissue interface in the frequency encoding –– Bleeding (rare)
direction
Causes of gyral calcification
Hypoplasia or agenesis of the
corpus callosum • Old infarct
• Normal sequence of formation is genu, • Meningoencephalitis
anterior body, posterior body, splenium, • Intrathecal chemotherapy
rostrum • Sturge–Weber syndrome
• Hypoplasia (partial absence)
–– Typically the last three sections to form
are absent
–– Associated with lipoma of the corpus
Tuberous sclerosis
callosum, fetal alcohol syndrome • Autosomal-dominant (70% are new
• Agenesis (complete absence) mutations)
–– Multiple associations, including • Epilepsy and mental retardation
Dandy–Walker malformation, • Skin abnormalities:
holoprosencephaly and lipoma of the –– Adenoma sebaceum
corpus callosum –– Shagreen patches
–– Ash leaf macules
Imaging features –– Periungual fibromas
MRI and cranial ultrasound –– Café au lait spots
• High riding third ventricle (between lateral • Renal abnormalities
ventricles) –– Angiomyolipoma
• Parallel lateral ventricles –– Renal cysts
• Colpocephaly (dilated occipital horns) –– Renal cell carcinoma
• Absent corpus callosum • CNS abnormalities
• Medial sulci have a radial configuration –– Multiple cortical hamartoma (90%)
towards the midline –– Multiple subependymal hamartoma
(90%)
–– Heterotopic grey-matter islands (90%)
Phakomatoses –– Giant cell astrocytoma (15%)
Sturge–Weber syndrome –– Subependymal tumour located near
foramen of Munro
• Rare sporadic disease (1 in 50,000)
–– Enlargement, causing obstructive
• Contralateral hemiparesis, hemiatrophy,
hydrocephalus
hemianopia, seizures
–– Transformation into high-grade
• Ipsilateral leptomeningeal angioma
astrocytoma
• Ipsilateral choroidal haemangioma
• Other abnormalities
• Ipsilateral facial port wine stain:
–– Cardiac rhabdomyoma
–– Maxillary distribution: parietal lobe
–– Bone cyst
angioma (commonest)
–– Retinal hamartoma, which can calcify

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Congenital CNS disorders 283

Neurofibromatosis von Hippel–Lindau disease


Neurofibromatosis type 1 • Autosomal dominant, chromosome 3
• One per 40,000 births
• Autosomal dominant, chromosome 17
• Absence of cutaneous signs
• One case per 3000 births
• Haemangioblastomas develop in 50%
• Intracranial abnormalities
–– 50% of patients with von Hippel–Lindau
–– Optic nerve glioma (20%)
disease develop them
–– Cerebral gliomas
–– 20% of all haemangioblastomas are
–– CNS hamartomas (pons, globus
associated with von Hippel–Lindau
pallidus, thalamus)
disease
–– Cranial nerve neurofibromas (VIII and
–– Benign slow growing vascular tumours
V commonest)
–– Cerebellum is commonest site followed
–– Hydrocephalus (aqueductal stenosis)
by spinal cord
–– Craniofacial plexiform neurofibroma
–– Occasionally they secrete EPO causing
–– Not associated with meningioma or
polycythaemia
acoustic schwannomas
–– Large cystic lesion with an avidly
• Bony abnormalities
enhancing mural nodule
–– Multiple non-ossifying fibromas
• Retinal angiomas develop in 70% and are
–– Bowing of tibia and pseudarthrosis
commonly multiple and bilateral
–– Ribbon ribs
• Multiple visceral organ neoplasms include:
–– Scoliosis (with a sharp, angular, high
–– Renal cell carcinoma (and cysts)
thoracic curve) in 40%
–– Phaeochromocytoma
–– Dural ectasia (posterior scalloping)
–– Pancreatic cystadenocarcinoma and
–– Widened neural foramina
islet cell tumours
• Vascular abnormalities
–– Endolymphatic sac tumour (causing
–– Coarctation of the aorta
sensorineural deafness)
–– Stenoses of major vessels, e.g. the
–– Cystadenocarcinoma of epididymis
carotid arteries
–– Hypertension (renal artery stenosis and
phaeochromocytomas)
Diagnostic criteria for
• Lung abnormalities
von Hippel–Lindau disease
–– Lower zone fibrosis
–– Intercostal neurofibromas • More than one CNS
• Gastrointestinal haemangioblastoma or
–– Intussusception (submucosal • One CNS haemangioblastoma and
neurofibromas) visceral manifestations or
–– Increased incidence of carcinoid and • Any manifestation and family history of
gastrointestinal stromal tumours VHL

Neurofibromatosis type 2
• Autosomal dominant, chromosome 22
• One case per 50,000 births Arachnoid cyst
• Benign congenital intra-arachnoid cyst
Neuroibromatosis type 2 versus
filled with cerebrospinal fluid (CSF)
neurofibromatosis type 1
• Caused by a focal splitting of the arachnoid
membrane
• Cutaneous signs are much less
• Commonest location is the anterior aspect
common
of middle cranial fossa
• Not associated with learning difficulties
• 10% occur at the cerebellopontine angle
• Not associated with optic nerve glioma
• Often an asymptomatic incidental
• Associated with meningiomas and
finding but slow expansion with pressure
acoustic schwannomas

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284 Central nervous system, and head and neck

effects can cause headaches, seizures or • Spinal contents are exposed


hydrocephalus • Commonest subtype is myelomeningocele
• Commonest location is lumbosacral
Recognised complications • Risk factors:
• Intracystic haemorrhage (neurosurgical –– mother aged > 35 years
emergency) –– Lack of folic acid
• Adjacent subdural haematoma –– Positive family history
• 95% have a neurological deficit
Imaging features • Common associations:
CT –– Scoliosis
• Well-defined, low-attenuating lesion –– Hydrocephalus
• No calcifications, no enhancement –– Arnold–Chiari malformation type 2
• Thinning of overlying calvarium, which –– Tethered cord
displaces adjacent structures –– Diastematomyelia
MRI –– Syringomyelia
• Isointense to CSF on all sequences
including FLAIR Other associations of
• Hypogenesis of adjacent structures, e.g. myelomeningocele
temporal lobe
• Rockerbottom and club foot
Arachnoid cyst versus epidermoid • Valgus deformity of hip
• Exuberant callus formation
• Cyst displaces structures; epidermoid • Metaphyseal fractures
encases structures
• Cyst is dark on FLAIR; epidermoid is
bright
• Cyst is dark on diffusion-weighted Diastematomyelia
imaging (DWI); epidermoid is bright
• Congenital sagittal cleft through cord
splitting it into two ‘hemicords’
• Each hemicord has its own central canal
• The hemicords often reunite distally
Spinal dysraphism • Subtypes
• Incomplete midline fusion of the bony –– Type I: both hemicords contained
spine within a single dural sheath with no
bony or fibrous septum between them
Spina bifida occulta –– Type II : both hemicords have their own
• 15% of spinal dysraphisms dural sheath and are separated by a
• Bony defect is covered by skin bony or fibrous septum
• Usually at the L5 or S1 levels • Commonest location is thoracolumbar
• Common incidental plain film finding junction region
• Majority have no neurological deficit • Site of the split is often marked by an
• Majority have a midline cutaneous lesion overlying cutaneous lesion
marking level of defect • Presents in teenage years with progressive
• Can be associated with other cord lesions, lower limb neurological problems
e.g. diastematomyelia • Association:
–– Tethered cord (>50%), s
Spina bifida aperta –– Scoliosis (>50%)
• 85% of spinal dsyraphisms –– Dysraphism
• Bony defect not covered by skin –– Syringomyelia

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Congenital CNS disorders 285

Tethered cord • Lower cervical cord


• Associations
• Abnormal dorsal fixation of the filum –– Arnold–Chiari malformation type 1
terminale (>50%)
• Caused by a fibrous band, lipoma or –– Dysraphism
intrathecal dermoid –– Diastematomyelia
• Cord unable to ascend during growth spurt –– Scoliosis
becomes ‘stretched’ –– Tethered cord
• Progressive lower limb weakness and • Clinical features
bowel and bladder dysfunction –– Horner‘s syndrome
–– Loss of pain and temperature sensation
Imaging features
over the shoulder (leading to a Charcot
MRI joint)
• Thickened filum (> 2 mm) –– Upper motor neurone signs in legs,
• Low-lying conus (below L2 level) lower motor neurone signs in arms
• Dorsal fixation of cord,
• Lipoma (in the filum or cutaneous) Acquired syringomeylia
• Dysraphism • Fluid-filled cavity within cord parenchyma
• Thoracic cord
Syringomyelia • Associations
–– Cord tumours
• Fluid filled cavity within the spinal cord, –– Trauma
which slowly expands, compressing nerve –– Infection
fibres
Imaging features
Congenital syringomelia MRI
• Commonest type • Cord expansion with low T1 and high T2
• Dilatation of central canal signal in cavity

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6.4 CNS infections

Bacterial meningitis • Commonest over cerebral convexities


(especially frontal lobe)
• Inflammation of the leptomeninges (pia • Proteinaceous fluid is of intermediate
and arachnoid) with development of a signal on T1WI and T2WI
subarachnoid exudate • Peripheral enhancement around
• Unenhanced CT and MRI usually normal collection
• Post contrast may see leptomeningeal • Underlying sulcal effacement and
enhancement cerebritis
• Lumbar puncture is needed to establish
the diagnosis Brain abscess
• Streptococcus milleri is the commonest
Complications
organism
• Arterial thrombosis with infarction
• Begins as an area of cerebritis which
• Venous sinus thrombosis with infarction
develops a capsule
• Cerebritis leading to abscess formation
• Fever, raised WCC, seizures, focal
• Ventriculitis (ependymal enhancement)
neurology
• Hydrocephalus caused by exudative
• Neurosurgical emergency requiring
adhesions
drainage and antibiotics
• Subdural empyema
Causes
Causes of leptomeningeal
enhancement
Direct spread
• Sinusitis (frontal lobe abscess)
• Bacterial meningitis
• Mastoiditis (temporal lobe abscess)
• Tuberculous or fungal meningitis
• Meningitis
• Carcinomatosis
Haematogenous spread
• Lymphoma
• Immunocompromise
• Subarachnoid haemorrhage
• Endocarditis
• Sarcoidosis
• Intravenous drug use
• Right-to-left cardiac shunts
• Pulmonary arteriovenous malformation
Subdural empyema
• Purulent fluid collection in the subdural
space Imaging features
• Neurosurgical emergency with mortality CT and MRI
around 20% • Typically a solitary lesion in a subcortical
• Rapid neurological deterioration (seizures, location
focal signs, coma) • Extensive surrounding oedema and ‘mass
• Causes effect’
–– Sinusitis (frontal sinuses most • Classically displays a uniform thin rim of
commonly involved) enhancement
–– Mastoiditis • Steroids reduce the degree of
–– Meningitis enhancement
–– Trauma (penetrating injury) • Restricted diffusion on DWI (unlike a
Imaging features tumour, which displays normal diffusion)

MRI
• Much more sensitive than CT

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CNS infections 287

• Key features
Solitary rim-enhancing lesion –– Sparing of lentiform nucleus
–– Streaky contrast enhancement around
• High grade glioma sylvian fissure
• Metastasis –– Petechial haemorrhages
• Cerebral infarct • Diffuse brain involvement is seen in AIDS
• Resolving haematoma patients
• MS plaque in acute stage
• Lymphoma (in immunocompromised)
• Tuberculoma
Tuberculosis
• Radiation necrosis • Immunocompromised are most at risk
• Arteriovenous malformation
• Thrombosed aneurysm
Tuberculous meningitis
• Commonest manifestation of cranial
tuberculosis
• Leptomeningeal enhancement
Multiple rim-enhancing lesions • Hydrocephalus caused by exudative
adhesions
• Metastases Tuberculoma
• Septic emboli
• Develops in 25% of patients
• Bacterial endocarditis
• Usually solitary lesion in the posterior
• Intravenous drug use
fossa
• Toxoplasmosis
• CNS lymphoma Imaging features
• Fungal infection CT
• Neurocysticerosis • Isoattenuating lesion with surrounding
oedema
• Central focus of calcification in 25%
Herpes simplex encephalitis MRI
• Low T1, high T2 signal and ring
• In adults caused by herpes simplex virus enhancement
(HSV)-1
• In neonates caused by HSV-2
• Virus lies dormant in cranial nerve ganglia AIDS-related CNS infections
• Rapid deterioration, with behavioural • MRI is the modality of choice in all these
changes, seizures and coma conditions
• Mortality approximately 50%
• Empirical antiviral treatment should be Incidence of AIDS-related CNS
given if there is a clinical suspicion infections
Imaging features
• HIV encephalitis: 60%
CT • Toxoplasmosis (opportunistic
• Findings often normal in first 5 days
infection): 30%
MRI • Cryptococcus (opportunistic infection):
• Findings usually apparent within first 2 5%
days • Progressive
• FLAIR is the best sequence multifocalleukoencephalopathy: 3%
• Unilateral diffuse high T2 signal in frontal • Cytomegalovirus encephalitis,
and temporal lobes tuberculoisis: 2%
• Progresses to bilateral disease and parietal
lobe involvement*

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288 Central nervous system, and head and neck

HIV encephalitis • Lumbar puncture needed to establish


diagnosis (India ink stain)
• Caused by HIV virus directly
• 25% will develop cryptococcomas
• Dementia-like picture
• Slowly progressive, diffuse high T2 signal Cryptococcoma
in centrum semiovale and periventricular • Pseudocysts fill dilated Virchow–Robin
white matter spaces
• No enhancement or mass effect • Common in basal ganglia, thalamus and
• Diffuse cerebral, cerebellar and brainstem brainstem
atrophy • Multiple well-defined high T2 signal
lesions
Toxoplasmosis • No surrounding oedema or enhancement
• Commonest opportunistic CNS infection
in AIDS Progressive multifocal
• Reactivation of prior infection with leukoencephalopathy
Toxoplasma gondii (from cat faeces) • Progressive, fatal demyelinating disease
• Usually multiple small abscesses with • Reactivation of JC virus, which destroys
surrounding oedema oligodendrocytes
• Main differential diagnosis is CNS • Focal neurological signs, e.g. limb
lymphoma weakness
Imaging features Imaging features
MRI MRI
• Predilection for basal ganglia and grey– • Diffuse bilateral white matter high signal
white matter junction (on T2WI and FLAIR)
• Homogeneous or thin rim of enhancement • Commonest site is posterior centrum
• Most lesions resolve within a few weeks of semiovale
treatment • No enhancement or mass effect
• Some remain visible on imaging for years • Extension into the gyri, following the
Cryptococcus neoformans outline of the overlying grey matter; this
distinguishes it from HIV encephalitis
infection
• Second commonest opportunistic Cytomegalovirus encephalitis
infection of the CNS • Imaging features indistinguishable from
• Causes an insidious onset of meningitis HIV encephalitis
• Imaging features are usually normal, • Diagnosis via PCR analysis of CSF
though may see subtle meningeal • Tendency to cause post-inflammatory
enhancement on post-contrast MRI parenchymal calcifications

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6.5 Stroke

Relevant vascular anatomy callosum giving off the orbitofrontal,


frontopolar, callosomarginal and
Arterial supply of the central nervous system pericallosal branches. These branches
is via the paired internal carotid arteries and supply the frontal lobe and the medial
the paired vertebral arteries. aspect of the parietal lobe
MCA
Internal carotid artery (ICA) • M1 segment (pre-MCA bifurcation):
Course lenticulostriate perforating arteries, which
supply the basal ganglia and adjacent deep
The ICA enters the skull base through the structures
carotid canal (in the petrous temporal bone). • M2 segment (post-MCA bifurcation):
It exits through the foramen lacerum to enter insular and cortical branches, which
the middle cranial fossa. It then enters the supply the insula and lateral surface of the
posterior part of the cavernous sinus and cerebral hemispheres
runs forward in the sinus before turning
upwards to pierce its roof medial to the Vertebral artery (VA)
anterior clinoid process. Here it gives off the
ophthalmic artery (which enters the orbit Course
through the optic canal with the optic nerve). The VA is the first branch of the subclavian
The ICA then turns backwards, curving over artery. It enters the C6 foramen
the roof of the sinus. It terminates by dividing transversarium (the vertebral vein exits
into the anterior cerebral artery (ACA), the at C7). It exits at the C1 foramen, running
middle cerebral artery (MCA), which runs posterior to the lateral mass. It then enters
in the sylvian fissure, and the posterior the skull through the foramen magnum, and
communicating artery (PCOM). it unites with the other vertebral artery in the
pre-pontine cistern to form the basilar artery.
Circle of Willis
The circle of Willis forms an anastomosis Important branches of the vertebral
between the ICA and the vertebrobasilar arteries
system and lies in the interpeduncular • Paired anterior spinal arteries which unite
fossa on the ventral surface of the brain. It and descend in the anterior sulcus of the
comprises: cord
• The two PCOMs connecting the MCAs • Paired posterior inferior cerebellar arteries
with the posterior cerebral arteries (PICAs) which supply the lateral medulla
(terminal branches of the basilar artery) and posterior inferior cerebellum. The
• The anterior communicating artery PICAs give off the posterior spinal arteries
(ACOM) uniting the two ACAs Important branches of the basilar artery
• Paired anterior inferior cerebellar arteries
Important branches of the ACA and
(AICAs) supply the anterior aspect of the
MCA inferior cerebellum
ACA • Perforator arteries supply the pons
• A1 segment (pre-ACOM): recurrent artery • Paired superior cerebellar arteries supply
of Heubner, which supplies the anterior the superior aspect of the cerebellum
limb of the internal capsule and adjacent • Basilar artery terminates as paired
structures posterior cerebral arteries, which supply
• A2 segment (post-ACOM): this passes the occipital lobes
posterosuperiorly over the corpus

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290 Central nervous system, and head and neck

Venous sinus anatomy Primary cerebral haemorrhage


• Paired internal cerebral veins run (15% of strokes)
posteriorly in the roof of the third ventricle • Hypertensive bleed
• Paired basal veins of Rosenthal run • Amyloid angiopathy
posteriorly around both sides of the
Other causes (15% of strokes)
midbrain (between the posterior cerebral
and superior cerebellar arteries) • Carotid or vertebral artery dissection
• The internal cerebral and basal veins unite • Vasculitis (e.g. systemic lupus
under the splenium of the corpus callosum erythematosus)
to become the great cerebral vein of Galen • Arterial spasm (aneurysm rupture)
• The single inferior sagittal sinus runs • Venous sinus thrombosis
posteriorly in the lower free edge of the falx • Cocaine use
cerebri; it joins the great cerebral vein to • Acute hypotension (watershed infarct)
become the straight sinus • Hypoglycaemia (occipital lobe infarct)
• The straight sinus runs posteriorly in the • Carbon monoxide poisoning (globus
midline along the tentorium cerebelli pallidus infarct)
• The single superior sagittal sinus runs • Periventricular leukomalacia (perinatal
posteriorly in the upper aspect of the falx hypoxic or ischaemic insult)
cerebri, joining the straight sinus at the
venous confluence at the internal occipital Thromboembolic stroke
protuberance
• The confluence divides into two transverse Pathophysiology
sinuses, which are asymmetrical and • 0–2 hours: reduced perfusion
which run laterally • 2–6 hours: intracellular neuronal grey
• The transverse sinuses become the matter swelling (cytotoxic oedema)
sigmoid sinuses before entering the • > 6 hours: vasogenic white matter oedema
jugular foramina to become the internal (blood–brain barrier breakdown)
jugular veins
Territorial involvement
• 50%: MCA
Stroke • 20%: lacunar
Definition • 20%: vertebrobasilar system
• 10%: anterior cranial artery
• An acute neurological deficit of presumed
vascular origin lasting more than 24 hours
Intravenous thrombolysis
Classification
Intravenous thrombolysis can be
Thromboembolic (70% of strokes)
administered within 3 hours of onset
• In situ atherothrombosis
provided intracerebral haemorrhage has
• Embolic
been ruled out and strict clinical criteria
–– Carotid artery plaque
have been met
–– Left heart mural thrombus (AF, LV
aneurysm)
–– Valve vegetations (endocarditis)
–– Paradoxical embolism (patent foramen MCA infarction
ovale) Imaging features in acute infarction
–– Pulmonary arteriovenous malformation
• There will be early signs of MCA territory
thromboembolic stroke in the first 6 hours

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Stroke 291

CT (non-contrast) • Two phenomena, resulting from reduced


• Hyperdense MCA sign (intraluminal cortical neuronal input, are recognised
thrombus) –– Wallerian degeneration, with atrophy of
• Loss of definition of lentiform nucleus ipsilateral corticospinal tracts
• Loss of definition of insular cortex –– Crossed cerebellar diaschisis, with
• Loss of grey-white matter differentiation atrophy of the contralateral cerebellar
hemisphere
MRI (T1WI and T2WI)
• Normal or non-specific changes in the Lacunar infarction
early stages.
• Caused by occlusion of small
• Absent flow void in a major occluded
deep penetrating end arteries, e.g.
vessel is sometimes seen
lenticulostriate branches of MCA or
MRI (DWI) pontine perforators of the basilar artery
• Most accurate test for ischaemia in the first • Small (< 1 cm2) areas of infarction in deep
6 hours structures, e.g. basal ganglia, internal
• Produces an MR image sensitised to the capsule, thalamus, pons, deep white
microscopic Brownian motion of water matter
molecules • Common in patients with diabetes or
• Acute stroke appears bright on DWI hypertension
(restricted diffusion of water) • In the pons the infarcts never cross the
• Acute stroke appears dark on the midline, except following occlusion of
corresponding apparent diffusion a rare anatomical variant, the artery of
coefficients (ADC) map image Percheron
• High DWI signal persists for around 5 days
Imaging features
MR spectroscopy
• Low N-acetyl aspartate CT
• High lactate peak • Small, well-defined areas of low
attenuation
Imaging features in established
MRI
infarction • Small areas of high signal on T2WI
CT • Small bright areas in the acute stages on
• Vasogenic white matter oedema develops DWI
• Infarct becomes better defined and exerts
mass effect ACA infarction
• Haemorrhagic transformation occurs in • Uncommon pattern of thromboembolic
around 10% of cases infarction
• Most often occurs secondary to vasospasm
MRI
following subarachnoid haemorrhage
• High signal on T2WI
• Small petechial haemorrhages are Imaging features
commonly seen
CT and MRI
• Contrast enhancement is typical beyond
• Infarct involving medial portion of the
48 hours (owing to opening of collateral
frontal lobe
vessels) in the cortex and overlying
leptomeninges Vertebrobasilar infarction
• Enhancement may persist for up to 12
• Clinically presents with cerebellar signs,
months
cranial nerve palsies, vertigo, lateral
Imaging features in chronic stroke medullary syndrome (occlusion of PICA)
• Well-defined area of brain atrophy with
enlargement of adjacent CSF spaces

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292 Central nervous system, and head and neck

–– Rupture of Charcot–Bouchard
Cerebellar signs microaneurysms.
–– Solitary deep bleed in basal ganglia,
• Nystagmus thalami, pons
• Slurred speech • Amyloid angiopathy
• Intention tremor –– Normotensive elderly patients
• Past pointing –– Multifocal peripheral bleeds
• Rebound
• Hypotonia Other causes
• Truncal ataxia • Vascular malformations
• Dysdiachokinesia • Cocaine use
• Pendular knee jerk • Brain tumours
• Drunken gait • Coagulopathy
• Vasculitis
Hint: Not Romberg’s sign, which is caused • Venous infarction
by loss of joint position sense • Trauma
Imaging features
Imaging features CT
• Focal area of high attenuation (60–80 HU)
CT and MRI in the acute stage, which slowly decreases
• Infarcts correspond to the vascular in size over several weeks becoming
territory supplied by the occluded vessel isodense with brain parenchyma
• Specific imaging features are the same as • Typically displays rim enhancement
for the anterior circulation during this phase, eventually becoming a
low-density area of encephalomalacia
Basilar artery thrombosis
• A rare but important variant of MRI
vertebrobasilar infarction • T2 gradient echo is the most sensitive
• Bilateral occipital lobe and thalamic sequence for blood breakdown products
infarcts • 0–2 days: low T1, low T2,
• Clinically causes occulomotor palsies, deoxyhaemoglobin
locked-in syndrome • 3–7 days: high T1, low T2,
• 90% mortality if untreated methaemoglobin (intracellular)
• Treated with systemic thrombolysis • 7–14 days: high T1, high T2,
(within 3 hours) methaemoglobin (extracellular)
• Intra-arterial catheter-directed • Chronic: low T1, low T2, haemosiderin
thrombolysis can improve the dismal
prognosis in this condition Special causes of stroke
Imaging features Carotid dissection
CT (non-contrast) • Accounts for 10–25% of strokes in young
• May show hyperdense clot in the basilar adults (< 45 years of age)
artery • Increased risk with fibromuscular
dysplasia, Ehlers–Danlos syndrome,
Primary cerebral Marfan’s syndrome or homocystinuria
• Most commonly occurs spontaneously or
haemorrhage following minor, unrecalled trauma. Less
Main causes commonly caused by major trauma or
penetrating injuries
• Hypertensive bleed • Presents with headache, stroke or painful
–– Elderly patients Horner’s syndrome (in 50% of cases)

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Stroke 293

Imaging features • Oral contraceptive pill


• Malignancy
MRI
• Vasculitis (lupus anticoagulant)
• Axial T1WI is the best sequence)
• Sagittal meningioma
• Intimal flap (only seen in one third of
• Paroxysmal nocturnal haemoglobinuria
cases).
• Congenital coagulation defects (deficiency
• Narrowed true lumen.
of protein S, protein C or antithrombin III)
• False lumen containing high signal
thrombus. Imaging features
MR angiography, CT angiography or Non-contrast CT
catheter angiography • Hyperdense thrombus in a dural sinus:
• Commonest location is cervical ICA at C1– ‘delta’ sign, seen in about 25% of cases
C2 level • Haemorrhagic infarction at underlying
• Tapered narrowing of vessel over a few grey–white matter junction
centimetres • Extensive thrombosis, which typically
• Lumen typically reconstitutes within the causes multifocal areas of haemorrhagic
bony carotid canal infarction that do not correspond to an
arterial distribution and often occur some
Watershed infarct distance from occluded sinus
• Caused by an episode of profound
hypotension, e.g. from cardiac arrest ‘Pseudodelta’ sign: false positives
• Affects regions of the brain located
between major vascular territories, • Raised haematocrit (neonates)
e.g. between ACA and MCA (anterior • Subdural blood
watershed) or between MCA and PCA • Subarachnoid blood
(posterior watershed)
• Causes symmetrical deep cerebral white
matter infarcts
Contrast-enhanced CT or CT venography
Venous infarction • Modality of choice
• Accounts for 1–2% of all strokes • Collateral venous channels enhance
• Commonest in children and young adults peripherally around a non-enhancing
• Non-specific symptoms (headache) thrombosed sinus: ‘empty delta’ sign, seen
evolving over several days in about 30% of cases
• Thrombotic occlusion of a dural venous • In chronic cases the falx and tentorium
sinus appear thickened, owing to the
• The bridging and cortical veins become development of prominent collateral
occluded, causing regional ischaemia, venous channels
cortical venous infarction and petechial MR venography
haemorrhages • Loss of normal flow void in the occluded
• Superior sagittal sinus is commonest sinus sinus
affected, followed by transverse sinus and • Direct visualisation of clot within the sinus
sigmoid sinus • Signal characteristics of clot vary with
time, e.g. subacute thrombus is high signal
Causes on T1WI
• Idiopathic in 25% • Adjacent subcortical cerebral oedema or
• Dehydration (in neonates) infarction appears high signal on T2WI
• Adjacent infection, e.g. mastoiditis, • Low signal areas within this represent
subdural empyema haemorrhage
• Pregnancy

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294 Central nervous system, and head and neck

Magnetic resonance venography (MRV) • Methods of assessing ICA stenosis include


• Provides complimentary information Doppler ultrasound, MR angiography and
• Sinus thrombosis appears as a segment of catheter angiography
signal loss
Doppler ultrasound of the
Pitfalls of MRI carotids
Features of the normal ECA
• Slow flowing blood gives high signal waveform
on T1WI and can mimic thrombus
• ECA arises anteromedial to the ICA
• Thrombus in the early stages gives low
• Side branches in the neck
signal and can mimic a normal flow
• Demonstrates the ‘temporal tapping’
void
phenomenon
• Has a high resistance waveform, i.e. low
end-diastolic flow
Deep cerebral venous • Waveform has a characteristic notch and
thrombosis may dip below the baseline
• Devastating clinical event Features of the normal ICA
• Occlusion of straight sinus, vein of Galen waveform
or internal cerebral veins
• No side branches in the neck
• Bilateral infarction of thalamus, basal
• No ‘temporal tapping’ phenomenon
ganglia and brainstem
• Has a low resistance waveform, i.e. high
end-diastolic flow
Carotid artery imaging Features of the normal CCA
Anatomy waveform
• The common carotid artery (CCA) • Intima-media thickness, measured just
bifurcates at the C4 level into the ICA and proximal to carotid bulb is normally
ECA < 0.8 mm
• The ICA has no branches in the neck • Thickening is recognised as an
independent risk factor for cardiovascular
Branches of the ECA events
• CCA waveform is higher resistance than
• Superior thyroid artery ICA but lower than ECA
• Ascending pharyngeal artery • Can normally dip below the baseline
• Lingual artery Criteria for diagnosing ICA stenosis
• Facial artery • Direct signs
• Occipital artery –– Peak systolic velocity in the ICA
• Posterior auricular artery > 230 cm/second
• Temporal artery –– ICA and CCA peak systolic velocity
• Maxillary artery (the first branch is the gradient > 4
middle meningeal artery; the terminal • Indirect signs
branch is the sphenopalatine artery) –– Spectral broadening of ICA waveform -
‘filling in’ under curve
–– ECA waveform shows increased
ICA stenosis diastolic flow - ‘ICA like’
• Atherosclerotic plaques
• ICA stenosis of 70–99% has been shown
–– Echo-poor: unstable with a high risk of
to benefit from endarterectomy surgery
rupture
(NASCET study)
–– Calcified: stable with low risk of rupture

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Stroke 295

Limitations of ultrasound Carotid stenting


• Calcified plaques interfere with Doppler
• Endovascular procedure as an alternative
interrogation
to endarterectomy
• Inability to visualise ICA lesions near the
• Especially suited for patients with
skull base
significant medical co-morbidities who are
• Inability to image the origins of the neck
considered too high an anaesthetic risk
vessels
• Shortened hospitalisation and
• Difficult to distinguish high-grade stenosis
convalescence times
from occlusion
• Similar 30-day stroke risk to
MR angiography endarterectomy (approximately 5%)
• Contrast enhanced MR angiography is the • Distal embolic protection devices (filters)
technique of choice have been developed to reduce further the
• The most accurate non-invasive technique risk of stroke
for evaluating the extracranial carotid • Can also be used to treat carotid dissection
vessels
• Can image the origins of the carotid Subclavian steal syndrome
arteries at the aortic arch
• Narrowing (partial steal) or occlusion
• Has a tendency to overestimate the degree
(complete steal) of the proximal subclavian
of stenoses
(or brachiocephalic) artery. Blood flows
in a retrograde direction down the same
Residual ‘trickle’ flow in high grade
side vertebral artery to supply the distal
stenosis
subclavian artery
• This diverts blood flow away from the
• Residual flow: suitable for surgery vertebrobasilar circulation causing
• Absent flow: unsuitable for surgery syncopal episodes, vertigo and ataxia,
classically induced by exercising the arm
on the affected side.
Catheter angiography Imaging features
• Definitive imaging test for distinguishing
Ultrasound
between high-grade stenosis and
• Partial steal: antegrade flow during
occlusion of the ICA
diastole and retrograde flow during systole
• Carries an approximate 1% risk of stroke
• Complete steal: retrograde flow during
diastole and systole

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6.6 Pituitary gland

Anatomy and physiology Components of the hypothalamus


• The pituitary fossa (sella turcica) houses
the pituitary gland, which is connected to • Optic chiasma
the hypothalamus via the infundibulum • Tuber cinerum
• Composed of a large anterior lobe and • Infundibulum
smaller posterior lobe • Mamillary bodies
• Anterior lobe produces prolactin, growth • Posterior perforated substance
hormone (GH), thyroid stimulating
hormone (TSH), adrenocorticotropic
hormone (ACTH), luteinising hormone
(LH) and follicle stimulating hormone Normal MRI appearances of
(FSH) the pituitary
• These hormones are secreted in response • T1WI: posterior lobe is high signal,
to releasing factors, which are carried by anterior lobe is isointense
hypophyseal veins in the infundibulum • T2WI: anterior and posterior lobes are
(the exception being prolactin, which is isointense to white matter
inhibited by dopamine release) • Post-contrast: homogenous enhancement
• Posterior lobe produces antidiuretic of the whole gland; adjacent cavernous
hormone (ADH) and oxytocin sinuses enhance more brightly than the
• These hormones are secreted in response pituitary
to neuronal impulses travelling down the
infundibulum
Pituitary disorders
Relations of the pituitary gland
Hypopituitarism
• Superior: diaphragm sella and suprasellar
cistern • Presents with lethargy, hypotension and
• Inferior: lamina dura and sphenoid sinus impotence
• Lateral: cavernous sinuses
• Anterior: tuberculum sella Causes of hypopituitarism
• Posterior: dorsum sella
The pituitary fossa is bordered by two anterior • Macroadenoma
and two posterior bony projections, the • Craniopharyngioma
clinoid processes. • Radiation therapy
• Suprasellar meningioma
Hypothalamus • Lymphocytic hypophysitis
• Trauma
• Forms roof of the suprasellar cistern and
• Haemochromatosis
floor of the third ventricle
• Sheehan’s syndrome
• Infundibulum arises from the tuber
cinereum and projects down through the
suprasellar cistern. It passes through a
hole in the diaphragma sella to attach to Lymphocytic hypophysitis
the pituitary gland • Autoimmune disease: lymphocytic
infiltration of pituitary

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Pituitary gland 297

• 90% of cases occur in women Imaging features


• Often arises in late pregnancy
Plain X-ray
• Causes headaches, visual symptoms and
• Enlarged sella turcica
hypopituitarism
CT and MRI
Imaging features • Pituitary tissue is pushed posteroinferiorly
MRI within the sella
• Swollen gland and infundibulum, which • Remainder of sella is filled with CSF
are of high T2 signal • Infundibulum extends to sella floor

Hyperprolactinaemia Causes of a large sella turcica


• Presents with galactorrhoea,
amenorrhoea, loss of libido and impotence Tumours
• Macroadenoma, e.g. acromegaly
Causes of hyperprolactinaemia • Craniopharyngioma
• Meningioma
• Prolactinoma Others
• Stalk compression by any suprasellar • Pituitary hyperplasia
mass (inhibiting dopamine release) • Hypothyroidism
• Anti-dopaminergic drugs, e.g. • Nelson’s syndrome following
phenothiazines adrenalectomy
• Lactation and pregnancy • Raised intracranial pressure
• Hypothyroidism (TRH stimulates • Benign intracranial hypertension
release) • Empty sella syndrome

Empty sella syndrome Causes of a small sella turcica


• Herniation of CSF-filled subarachnoid
space through the diaphragma sella • Normal variant
• Primary empty sella syndrome is the • Low intracranial pressure
commonest type • Fibrous dysplasia
–– Usually an incidental imaging finding • Childhood irradiation
–– Caused by a congenital defect in the • Dystrophica myotonia
diaphragma sella • Hypoptuitarism
–– More common in females
–– Associated with hypertension, obesity
and benign intracranial hypertension
–– Majority have normal endocrine Pituitary tumours
function
–– Associated with spontaneous CSF Microadenoma
rhinorrhoea • Very common in autopsy series
• Secondary empty sella syndrome • Accounts for one third of pituitary
–– Occurs following destruction of adenomas presenting clinically
pituitary gland • Benign tumours < 1 cm in diameter
–– Causes include Sheehan’s syndrome • Arise from the anterior lobe
and radiotherapy • 75% secrete hormones (the other 25% are
–– More marked degree of CSF herniation ‘incidentalomas’)
than in the primary type • Commonest hormone secreted is prolactin
–– Headaches and visual disturbance are • Less commonly GH is secreted (causing
common acromegaly)
• Rarely ACTH, TSH or FSH are secreted

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298 Central nervous system, and head and neck

Imaging features • Erosion of sella floor (‘double floor’ if


asymmetrical erosion)
MRI (modality of choice)
• Erosion of anterior and posterior clinoids
• Modality of choice
• Subtle low signal area on T1WI, subtle high CT
signal area on T2WI • Mass of soft-tissue density, which
• Commonly off midline enhances homogeneously
• Adenoma is non-enhancing post contrast MRI
• Non-specific signs • Solid, enhancing mass, which may contain
–– Depression of sella floor cystic areas
–– Contralateral deviation of infundibulum • Lateral displacement of ICA flow void
–– Convex diaphragma sella (normally • Macroadenomas typically do not occlude
seen with gland hypertrophy caused by the ICA
pregnancy or hypothyroidism)
Craniopharyngioma
Differential diagnoses • Benign tumour arising from cell remnants
of Rathke‘s cleft
Rathke cleft cyst • Two thirds occur in children
• Arises from Rathke pouch remnants • Majority are suprasellar with an intrasellar
• Small non-enhancing benign component
intrapituitary cyst • Typically large lesions, which exert
• Majority are incidental findings symptoms via mass effects:
• If large can extend into the suprasellar –– Bitemporal hemianopia
region –– Hydrocephalus
Pituitary metastases –– Cranial nerve palsies
• Very rare –– Diabetes insipidus
Imaging features
Plain film
Macroadenoma • Majority have enlarged sella turcica
• Accounts for two thirds of pituitary • Erosion of sella floor and clinoids
adenomas presenting clinically • Visible calcifications within sella (in 90% of
• Benign tumours > 1 cm in diameter paediatric cases and 30% of adult cases)
• Arise from the anterior lobe
CT
• Majority are non-secreting; prolactin is the
• Large cystic or solid mass with
commonest hormone secreted, followed
calcifications
by GH
• Avid enhancement of solid components
• Exert symptoms via mass effects:
–– Bitemporal hemianopia MRI
–– Hydrocephalus • Heterogeneous appearance
–– Cranial nerve palsies • Cystic areas are high signal on T2WI
–– Diabetes insipidus • Calcifications are areas of signal void
• Apoplexy is a rare complication:
–– Massive haemorrhage into pituitary Rule of threes for adult
gland craniopharyngioma
–– Severe headache, visual loss, obtunded
• One third occur in adults
Imaging features • 30% contain calcifications
Plain X-ray • Third ventricle floor is an ectopic site
• Enlarged sella turcica

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Pituitary gland 299

Suprasellar mass lesions Pineal germinoma


Common causes in adults
• Macroadenoma (superior extension) • 80% arise in the pineal gland
• Meningioma • Commonest pineal gland tumour
• ICA aneurysm • Occur in children and young adults
• Cause precocious puberty
Common causes in children • Parinaud’s syndrome (upward gaze
• Craniopharyngioma palsy)
• Hypothalamic hamartoma • Infiltrating soft tissue mass
• Optic glioma of chiasma • Enhances avidly
• Hydrocephalus (aqueductal
Less common causes obstruction)
• CSF ‘drop metastases’
• Lymphoma
• Germinoma
• Histiocytosis X Histiocytosis X
• Sarcoidosis
• Tuberculosis • Disseminated form (Hand–Christian–
• Metastases Schüller disease) can cause pituitary
• Dermoid or epidermoid infiltration
• Arachnoid cyst • Typically a disease of childhood causing
diabetes insipidus
• Loss of normal high signal from posterior
lobe on T1WI
Hypothalamic hamartoma • Pituitary and infundibulum display avid
• Well-defined mass projecting down from enhancement
the tuber cinereum
• Causes precocious puberty
Pituitary metastases
• Isointense to grey matter on T1WI and • Breast is the commonest primary site
T2WI • Hypothalamic and infundibular
• Does not enhance (other non-enhancing metastases are much commoner than
suprasellar masses are dermoids, pituitary metastases
epidermoids and arachnoid cysts)
Suprasellar meningioma
Lymphoma • 10% of all meningiomas
• Non-Hodgkin’s type • Most commonly arise from tuberculum
• Infiltration of hypothalamus, sella or planum sphenoidale
infundibulum and pituitary, which appear • Can invade the cavernous sinus and
enlarged and of high attenuation on non- occlude the ICA
contrast CT • Can cause pneumosinus dilatans of the
• Display avid enhancement sphenoid sinus
• Blood supply is via posterior ethmoidal
Suprasellar germinoma branches of ICA
• Malignant germ cell tumour • CT and MR features are the same as for
• 20% arise in the infundibulum meningiomas elsewhere
• Infiltrating mass
• Display avid enhancement

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6.7 Brain and spinal trauma

NICE head injury guidelines Diastatic fracture


• Fracture extension into a suture
Main indications for head CT within 1 hour of
• Mainly occurs in children
assessment:
• Lamboid suture is the most commonly
• Glasgow coma scale < 13 at any time since involved
the injury • Suture widened > 2 mm
• Any signs of skull base fracture
• Any bleeding tendency with loss of
consciousness at time of injury
Skull base fractures
• Any convulsions following the injury Clinical features
• Age > 65 years with loss of consciousness • Associated with vascular injuries, cranial
at time of injury nerve damage and meningitis
• Any problems with speaking, reading or • Battle sign (mastoid bruising)
writing • Raccoon eyes (periorbital bruising)
• Any problems with balance, walking or • Haemotympanum (blood behind an intact
changes in eyesight tympanic membrane)
• CSF rhinorrhoea or otorrhoea, usually
Skull vault fractures apparent at time of injury

Linear fracture Skull base anatomy


• Commonest type of skull fracture (> 70%)
• Commonest in squamous temporal bone Formed by several bones:
• Fine lucent line on plain film versus • Orbital plate of frontal bone
vascular groove (corticated margins, • Cribriform plate of ethmoid bone
branching) • Sphenoid bone
• Become less lucent as they heal, but may • Temporal bone (squamous and petrous
remain visible for years parts)
Depressed fracture • Occipital bone
• Clivus
• Second commonest fracture type
• Caused by a direct blow
• Commonest in frontoparietal region
• Usually comminuted Imaging features
• Focal area of increased density on plain • Indirect radiological signs
film –– Sphenoid sinus air–fluid level
• CT is mandatory to assess for underlying –– Pneumocephalus
brain damage
• High risk of brain injury if depressed
Orbital plate fracture
> 5 mm • Associated with frontal sinus fractures
• Fracture fragment can ‘blow in’ to the orbit
Growing fracture • Entrapment diplopia of superior rectus or
• Fracture causing a tear in the underlying superior oblique muscles
dura
• Herniation of CSF or brain parenchyma
Temporal bone fractures
into fracture • Longitudinal (80% of cases)
• Fracture slowly widens, ‘growing’ over –– Fracture line runs vertically through
several months middle ear cavity
• Associated with brain injury and seizures –– Ruptures tegmen tympani and
tympanic membrane

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Brain and spinal trauma 301

–– CSF otorrhoea and bleeding from • Does not cross a suture line unless fracture
external auditory canal runs through suture
–– Ossicular dislocation with conductive • Can cross the midline (mainly seen in
deafness venous bleeds)
–– Loss of malleus–incus articulation (‘ice • Exerts considerable ‘mass’ effect because
cream cone’) on axial CT of rapid pressure rise
–– Sensorineural deafness, vertigo and MRI
seventh nerve palsy may occur but are • Signal characteristics depend on age of the
uncommon blood products
• Transverse (20% of cases) • Thin, low-signal line of dura separated
–– Fracture line runs horizontally through from the inner skull table
middle ear cavity
–– Tympanic membrane intact Subdural haematoma
–– Haemotympanum and CSF rhinorrhoea • Accumulation of blood between the
–– Commonly involves bony labyrinth leptomeninges and dura
–– Sensorineural deafness, vertigo and • Tearing of bridging veins which cross
seventh nerve palsy are common through the subdural space (connecting
cortical veins to dural venous sinuses)
Traumatic intracranial • Elderly people and alcoholics are at risk,
haemorrhage owing to cerebral atrophy and ‘stretching’
of bridging veins
Extradural haematoma • Caused by an acceleration–deceleration
• Accumulation of blood between the dura injury or minor trauma
and periosteum • Initial event is often subclinical
• 90% caused by a temporal bone fracture • Clot slowly expands, with gradual
with laceration of underlying middle development of pressure effects
meningeal artery • Rarely caused by rupture of a vascular
• 10% caused by tearing of a dural venous malformation
sinus • Around 15% are associated with a skull
• High-pressure bleeding, which ‘strips’ dura fracture
away from the skull • Commonest location is over the cerebral
• Haematoma confined by strong dural convexities
attachments at suture lines • Posterior interhemispheric subdural is
• Initial loss of consciousness followed by a associated with non-accidental injury
lucent interval • Bilateral in 20% of adult cases and 80% of
• Sudden rapid deterioration caused by paediatric cases
rising intracranial pressure • Underlying brain damage is common, e.g.
• Majority present to hospital within 3 days haemorrhagic contusion
of the injury Imaging features of acute subdural
• Mortality is approximately 15% but higher haematoma (0–7 days old)
if age > 65 years or if Glasgow coma scale
< 8 CT
• Requires urgent neurosurgical evacuation • Crescent-shaped, high-attenuation extra-
• Outcome is usually good because there is axial collection
minimal underlying brain injury • Can appear isodense in anaemic patients
• Can spread over entire surface of cerebral
Imaging features hemisphere
CT • Can track along the tentorium or into
• Biconvex, high-attenuation extra-axial interhemispheric fissure
collection • Does not cross the midline
• Overlying fracture line

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302 Central nervous system, and head and neck

Imaging features of subacute • 3–7 days: high T1 low T2, methaemoglobin


subdural haematoma (7–21 days) (intracellular)
• 7–14 day: high T1 high T2, met
CT deoxyhaemoglobin (extracellular)
• Isodense extra-axial collection which • Chronic: low T1, low T2, haemosiderin
exerts ‘mass’ effect
• Medial displacement of grey–white matter Traumatic subarachnoid
interface haemorrhage
MRI • Commonest cause of subarachnoid blood
• High signal on T1WI (methaemoglobin) • Associated with other injuries e.g.
intracerebral contusion, fracture
Imaging features of chronic
subdural haematoma (>21 days) Imaging features
CT CT
• Low-attenuation extra-axial collection, • Focal area of high-attenuation blood
which exerts ‘mass’ effect within the superficial sulci, which may
• High-attenuation areas within lesion extend into the rest of the subarachnoid
(caused by acute-on-chronic bleeding) space
• Layering of hyperdense material
posteriorly; 2% calcify
Diffuse axonal injury
• Multiple small traumatic lesions at grey–
Cerebral contusion white matter junction
• Caused by direct trauma or acceleration– • Caused by rotational acceleration–
deceleration injuries in which the brain deceleration shearing forces
parenchyma strikes bony ridges of the • Commonest type of injury in severe closed
inner skull head trauma
• Haemorrhagic focus primarily involves the • Usually associated with severe impairment
superficial grey matter of consciousness
• Often bilateral, occurring in a ‘coup– • Poor long-term prognosis
contrecoup’ pattern
• Commonest sites are the temporal and Location (with increasing severity
frontal lobes of injury)
• Seizures are common
• 20% expand, requiring neurosurgical • Frontal and temporal lobes
intervention • Posterior aspect of corpus callosum
• Brainstem
Imaging features
CT
• Initial scan may be normal or show a focus
Imaging features
of high attenuation
• Surrounding low-attenuation oedema CT
develops overs days • Usually normal: 80% are non-
• Oedema can cause significant mass effect haemorrhagic
and herniation • May see multiple petechial haemorrhages
• Typically becomes isodense by 2 weeks at grey–white interface
MRI MRI
• Modality of choice, especially for posterior • Modality of choice
fossa contusions • Multiple small elliptical high-signal lesions
• T2 gradient echo is most sensitive on T2WI and FLAIR
sequence for blood products • Haemorrhage seen as low-signal foci
• 0–2 days: low T1 low T2, within these lesions
deoxyhaemoglobin

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Brain and spinal trauma 303

Brain herniations Brachial plexus


• Caused by an expanding mass within the Anatomy
rigid cranial vault
• Brachial plexus is composed of the C5, C6,
Subfalcine herniation C7, C8 and T1 nerves
• Each nerve is formed from a dorsal and
• Commonest type of herniation
ventral nerve root
• Caused by laterally placed mass in a
• Roots accompanied by a dural sleeve as far
cerebral hemisphere
as the exit foramen
• Medial aspect of ipsilateral frontal lobe
is displaced across the midline under the Traumatic injury
falx cerebri with compression of ipsilateral
• Nerve root injuries are usually caused by
lower limb motor areas and ACA
closed traction
Imaging features • Considerable force is required, e.g. high-
speed road traffic accidents
Axial CT
• Associated with multiple life-threatening
• Midline shift (bowing of falx cerebri)
injuries
• Effacement of ipsilateral frontal horn
• Commonest injury pattern is avulsion of
• Dilated contralateral frontal horn, with
all C5–T1 nerve roots, producing a flaccid
compression of the foramen of Munro
monoplegia
• Can cause ACA territory infarction
Imaging features
Transtentorial herniation
MRI
• Caused by a mass in the middle cranial
• Traumatic meningocele (tearing of dural
fossa
sleeve with CSF leak)
• Uncus (medial aspect of temporal lobe) is
• Avulsion of spinal nerve roots from the
displaced down through the transtentorial
cord
hiatus with compression of midbrain,
ipsilateral third cranial nerve and PCA
Spinal cord injury
Imaging features
• Cord most commonly injured in cervical
Axial CT and thoracolumbar junction regions,
• Effacement of ipsilateral lateral ventricle
usually caused by a fracture dislocation
• Dilatation of contralateral lateral ventricle
• May also be caused by a burst fracture
and temporal horn
resulting from an axial loading injury with
• Widened ipsilateral ambient cistern
retropulsed vertebral body fragments
• Can cause PCA territory infarction

Tonsillar herniation Cord oedema


• Long segment of high signal seen within
• Caused by raised intracranial pressure or
cord on T2WI
posterior fossa mass
• Greatest chance of neurological recovery
• Downward displacement of cerebellar
tonsils through the foramen magnum, Cord haemorrhage
with compression of the cardiorespiratory • Central low-signal areas are seen within
centres the high-signal oedema
Imaging features • Prognostically worse than oedema alone
Axial CT Cord transaction
• Tonsils visible at level of the dens • No chance of neurological recovery
Sagittal CT and MRI
• Tonsils > 5 mm below the foramen
magnum

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304 Central nervous system, and head and neck

Cervical spine fractures • Associated with quadriplegia


• Most commonly occurs at C5
Normal intervals Imaging features
• Atlantodens interval < 3 mm in an adult,
< 5 mm in a child Plain X-ray
• 2 mm subluxation allowed at C2–C3 and • Avulsion of anteroinferior vertebral body
C3–C4 in a child < 8 years of age corner
• Posterior ‘fanning’ of spinous processes
Odontoid fractures • Widening of posterior disc space
Type 1 Extension tear-drop fracture
• 4% of odontoid fractures • Stable injury
• Fracture through upper aspect of odontoid • Most commonly occurs at C2 or C3 level
process
Imaging features
Type 2
Plain X-ray
• 66% of odontoid fractures
• Widening of anterior disc space
• Fracture through base of odontoid
• Fracture of articular pillar
• Best seen on open-mouth ‘peg’ view
• Narrowing of interspinous distance
• Unstable injury
• High risk of non-union, and requires Hangman’s fracture
surgical fixation
• Hyperextension injury
Type 3 • Axial loading on posterior elements by
• 30% of odontoid fractures occiput
• Extension of fracture into body of C2 • Oblique fracture through posterior arch of
• Best seen on lateral view: disruption of C2
Harris’ ring • Unstable injury, but spinal cord usually
not injured
Mimics of an odontoid fracture • Fracture may extend into foramen
transversarium
• Os odontoideum Jefferson fracture
• Ossiculum terminale
• Axial loading injury
• Aplasia of dens
• Burst fracture of C1
• Hypoplastic dens
• ‘Peg’ view shows widened lateral spaces

Clay shoveller’s fracture


Flexion tear-drop fracture • C7 spinous process fracture
• Stable injury
• Very unstable injury

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6.8 Vascular malformations

Non-traumatic Mycotic aneurysm


subarachnoid haemorrhage
• Associated with bacterial endocarditis
Berry aneurysms and intravenous drug use
• 2% of the population have an intracranial • Typically small and peripherally located
aneurysm • High risk of rupture
• 90% of these are saccular ‘Berry’
aneurysms
• 20% are multiple
• Majority are asymptomatic Aneurysm rupture:
• Some cause local pressure effects, e.g. subarachnoid haemorrhage
third nerve palsy with PCOM aneurysm • Presents with severe headache (‘hit on the
• 1% risk of rupture per year with aneurysms head with a hammer’), neck stiffness and
>5 mm photophobia
• 20% mortality
Associations of berry aneurysms • 60% complete recovery
• 20% residual deficit
• Adult polycystic kidney disease
• Fibromuscular dysplasy Imaging features in the first
• Marfan’s syndrome 48 hours
• Ehlers–Danlos syndrome CT
• Coarctation of the aorta • Modality of choice: non-contrast CT has
> 95% sensitivity
• High-attenuation blood seen in CSF
Location cisterns
• 90% in the anterior circulation • Blood may also be seen in the ventricles, as
–– ACOM (commonest) a result of reflux
–– PCOM • Location of blood is clue to location of
–– MCA bifurcation aneurysm
–– Ophthalmic artery –– ACOM: anterior interhemispheric
• 10% in the posterior circulation –– MCA: sylvian fissure
–– Basilar tip –– PICA: fourth ventricle
–– PICA Imaging features beyond 48 hours
Giant saccular aneurysm MRI (Modality of choice)
• Modality of choice
• FLAIR is best sequence
• >2.5 cm • Blood seen as areas of high signal
• Located at skull base, e.g. cavernous
part of ICA Imaging features beyond 7 days
• Middle-aged women • By day 7 only 50% of subarachnoid
• Peripheral calcifications haemorrhages are visible with CT
• Usually presents with symptoms of • Any blood seen beyond 2 weeks on CT
mass effect suggests a rebleed
• CSF xanthochromia is positive in all
patients after 12 hours

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306 Central nervous system, and head and neck

• Xanthochromia persists for around CT angiography


2 weeks • Detects 90% of aneurysms of > 5 mm
• Repeated subarachnoid haemorrhage diameter
causes meningeal signal voids from • Aneurysms at the skull base may be
haemosiderin obscured by bone artefacts
Management pathway for Angiogram-negative subarachnoid
subarachnoid haemorrhage haemorrhage
• CT or lumbar puncture demonstrates
subarachnoid blood • 10% of cases
• Angiography to identify and locate the • CT or lumbar puncture shows
aneurysm subarachnoid blood
• Treatment of aneurysm to prevent • Angiogram demonstrates no aneurysm
rebleeding with endovascular coiling or • Blood tends to be localised around the
craniotomy and aneurysm clipping midbrain
Cerebral angiography in • Represents perimesencephalic venous
bleeding
subarachnoid haemorrhage • Typically has an excellent prognosis
Catheter angiography
• Injection of both carotids and vertebrals
(‘four-vessel’ angiogram) Complications of subarachnoid
• Aneurysms are lobulated and arise from haemorrhage
arterial bifurcations
• Best test for showing aneurysm • Rebleeding
morphology and vascular spasm –– Acute clinical deterioration
• Shows only intraluminal portion of –– Most commonly seen within first 48
aneurysm (not wall clot) hours
• Carries an approximately 1% risk of stroke • Arterial vasospasm
–– Can cause cerebral infarction (2% of all
MR angiography strokes)
• Detects 90% of aneurysms of > 3 mm –– Mainly ACA territory (ACOM aneurysm)
diameter • Non-communicating hydrocephalus
–– Can develop within first few hours
Time of flight –– Blood products blocking the CSF
• Repeated excitations with a short TR pathways, e.g. aqueduct of Sylvius
saturate stationary tissue • Communicating hydrocephalus
• Fresh blood entering the slice is –– Occurs beyond first week
unsaturated and appears bright –– Blockage of arachnoid granulations due
• Slow flow and in-plane flow can mimic to meningeal fibrosis
occlusion
• Technique of choice for cerebral
angiography Arteriovenous
Phase contrast
malformation
• Application of a positive gradient to • Abnormal connection between arterial
moving blood followed by a negative and venous systems
gradient produces a detectable ‘phase’ • No intervening capillary bed, just a nidus
shift, which is proportional to flow velocity of thin-walled vessels
and is not affected by slow flow and in- • Vascular stenoses and aneurysms are
plane flow common within the nidus
• Long examination times and lower spatial • 0.1% of the population have an intracranial
resolution than TOF arteriovenous malformation

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Vascular malformations 307

• Commonest symptomatic cerebral • Enlarged supplying arteries and draining


vascular malformation veins
• 80% are supratentorial, and the parietal MRI
lobe is the commonest location • Modality of choice
• 2% are multiple (associated with Osler– • Central nidus vessels are of variable signal
Weber–Rendu syndrome) intensity
Clinical manifestations • Supplying arteries and draining veins
identified in > 50% of cases
• Intracranial haemorrhage is the
• Adjacent parenchymal volume loss and
commonest clinical manifestation
gliosis (high T2 signal)
–– 2% per year risk, 40% lifetime risk
• Variable appearance of associated
–– Usually an intraparenchymal bleed
haemorrhage
–– Rarely ruptures into the subarachnoid
• Gadolinium is of limited value because of
space
inconsistent enhancement
–– 5% risk of rebleed in the year following
a first haemorrhage Catheter angiography
• Seizures • Demonstrates vascular anatomy better
–– The larger the aneurysm the greater the than MR angiography or CT angiography
risk • Majority supplied by pial branches of ICA
• Progressive neurological deficit • Characteristic early filling of draining veins
–– Caused by stealing of blood from caused by shunting
adjacent vascular territories
Cavernous angioma
Increased risk of haemorrhage
• Well-defined collection of dilated
intraparenchymal vascular channels,
• Size < 3 cm
which are prone to recurrent intralesional
• Exclusive central venous drainage
haemorrhage
• Periventricular location
• Commonly multiple, especially in Osler–
Weber–Rendu syndrome
• 50% are an incidental finding
Imaging features • 50% present with seizures
Plain X-ray Imaging features
• Prominent vascular grooves and
CT
calcifications sometimes seen
• Small, round, high-attenuation lesion (20%
CT are calcified)
• Focal area of mixed attenuation • Enhances post contrast
• 30% contain calcifications • Usually in a subcortical location
• Adjacent brain atrophy from ischaemic
• MRI
steal
• T2 is the most sensitive sequence
• 20% not visualised on non-contrast scans
• Well-defined lesion of mixed signal
• Dense enhancement of serpiginous vessels
intensity (blood products)
(‘bag of worms’)
• Low-signal rim of haemosiderin

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6.9 Demyelinating diseases

Multiple sclerosis –– Display mild surrounding oedema and


‘mass effect’
• Idiopathic inflammatory demyelinating –– Enhance for up to 8 weeks (blood–brain
disease of the CNS barrier breakdown)
• Commonest in young adults and the –– Variety of enhancement patterns, e.g.
middle-aged and temperate climates; homogeneous, ring-like
more common in women –– Slowly decrease in size but do not
• Diagnosis requires objective evidence of disappear completely
lesions disseminated in time (new lesions • Chronic plaques
on follow-up scans) and space (multiple –– Number of plaques correlates with
lesions) duration of disease
• Starts as perivenous inflammation –– No surrounding oedema or ‘mass effect’
progressing to demyelination –– No enhancement (blood-brain barrier
• Mainly affects white matter but lesions in intact)
grey matter are also seen –– Associated cerebral atrophy
• Relapsing–remitting clinical course, with
optic neuritis, spastic paraparesis, ataxia, MRI sequences in multiple sclerosis
nystagmus and urinary incontinence
• Lumbar puncture shows CSF
FLAIR
lymphocytosis and oligoclonal IgG bands
• Best sequence for supratentorial lesions
• Visual evoked potentials show delayed but
• Poor for posterior fossa and spinal cord
well-preserved waveform
lesions
Imaging features Proton-density weighted
• More sensitive than T2WI for brain
CT
lesions
• Low-attenuation periventricular lesions
• Best sequence for spinal cord lesions
(low sensitivity)
MRI
• Most sensitive investigation
• Plaques are typically ovoid lesions
McDonald MRI criteria for
5–15 mm in diameter multiple sclerosis
• Plaques are low signal on T1 • Dissemination in space, with at least three
• Plaques are high signal on T2, proton- of the four following features:
density weighted and FLAIR sequences –– One gadolinium-enhancing brain or
• Typical distribution of MS plaques cord lesion or nine T2-hyperintense
–– Perpendicular to ventricles (Dawson brain or cord lesions
fingers) –– One or more brain, infratentorial or
–– Juxtacortical U-fibre cord lesions
–– Corpus callosum –– One or more juxtacortical U-fibre
–– Temporal lobes lesions
–– Brainstem –– Three or more periventricular lesions
–– Cerebellum • Dissemination in time
–– Spinal cord –– A gadolinium-enhancing lesion
• Acute plaques detected more than 3 months after
initial clinical event

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Demyelinating diseases 309

Causes of periventricular high T2 Central pontine


signal lesions myelinolysis
• Caused by rapid correction of
• Small vessel ischaemia hyponatraemia (> 15 mm in 24 hours)
• Virchow–Robin spaces (dark on FLAIR) • Typically occurs in malnourished
• Multiple sclerosis alcoholics
• Migraine • Rapid demyelination with quadriparesis
• Vasculitis (systemic lupus and bulbar palsy
erythematosis, Behçet’s disease, • Commonly fatal
Wegener’s granulomatosis)
• Sarcoidosis Imaging features
• Lyme disease CT
• Acute disseminated encephalomyelitis • Low attenuation in pons
• HIV infection, progressive multifocal
leukoencephalopathy MRI
• Radiotherapy • High T2 signal in pons

Alcohol and the CNS


Wernicke’s encephalopathy
Acute disseminated
• Caused by thiamine (vitamin B1)
encephalomyelitis deficiency
• Monophasic inflammatory demyelinating • May progress to Korsakoff’s dementia
disease • Clinical triad of ataxia, nystagmus and
• 10–14 days following infectious illness or ophthalmoplegia
vaccination Imaging features
• Affects children more than adults
• Imaging triad
• Presents with change in mental state,
–– Symmetrical high T2 signal in medial
seizures and focal neurological signs
thalamus
• Increased risk of subsequently developing
–– Mamillary body atrophy
MS
–– Enlargement of third ventricle
• 10% mortality
Imaging features Marchiafava–Bignami
syndrome
CT
• Usually normal • Alcohol-induced demyelination of corpus
callosum
MRI
• Multifocal high T2 signal lesions Cerebral and cerebellar
• Predilection for white matter, basal ganglia atrophy
and thalamus
• Especially affects the vermis
• Most lesions shrink or disappear on
follow-up scans

Features distinguishing acute


disseminated encephalomyelitis from
multiple sclerosis

• Younger age group


• Monophasic illness
• Larger more ill-defined lesions
• Majority of lesions enhance

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6.10 Orbit and lacrimal glands

Anatomy Orbital foramina and contents


Optic canal
The eyeball (globe) • Lies in lesser wing of sphenoid, close to the
• Positioned anteriorly in the orbit, closer orbital apex
to roof than floor, nearer to lateral than • 5–6 mm in diameter and 8–12 mm long
medial wall • Connects the middle cranial fossa with the
• Around 24 mm in anteroposterior orbital cavity
diameter • Transmits optic nerve sheath complex,
• Lens separates small anterior from large ophthalmic artery and surrounding
posterior segment sympathetic plexus
• Anterior eyeball contains aqueous humour
• Posterior eyeball contains vitreous Superior orbital fissure
humour • Thin cleft between the greater and lesser
wings of sphenoid
Layers of the eyeball • Separates the lateral wall and roof of the
• Outer sclera is fused with dura and orbit posteriorly
arachnoid sheaths of the optic nerve and is • Connects the middle cranial fossa with the
continuous anteriorly with the transparent orbital cavity
cornea • Transmits third, fourth and sixth cranial
• Uveal layer comprises, from back to nerves and the ophthalmic division of the
front, the choroid, ciliary body and iris, fifth cranial nerve as well as the superior
which form a continuous, highly vascular and inferior ophthalmic veins
structure • The superior ophthalmic vein runs from
• Inner retinal layer extends from the optic medial to lateral over the superior aspect
nerve to a point just posterior to the ciliary of the optic nerve
body
Causes of a wide superior orbital
Muscles of ocular motility fissure
• Four rectus muscles arise posteriorly
from the annulus of Zinn, a fibrous band • ICA aneurysm
continuous with periosteum and dura of • Carotid–cavernous sinus fistula
optic canal • Neurofibroma
• From this common origin the muscles pass • Haemangioma
forward as a muscle cone to be inserted
into the sclera of the eyeball
• The optic nerve sheath complex, which Inferior orbital fissure
enters the orbit through the optic canal, • Lies between the greater wing of the
runs within the muscle cone (intraconal) sphenoid and the maxilla
• Superior oblique muscle arises above • Separates the lateral wall and floor of the
the annulus of Zinn and runs forward orbit posteriorly
along the superomedial orbital wall to the • Connects the pterygopalatine fossa and
trochlea, where it turns to insert on the the inferotemporal fossa to the orbital
posterosuperior aspect of eyeball cavity
• Inferior oblique muscle arises anteriorly, • Closed by the periorbita and the muscle of
below and lateral to lacrimal fossa and Müller
inserts on the posteroinferior aspect of • Transmits maxillary division of fifth cranial
eyeball nerve, the zygomatic nerve (a branch of

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Orbit and lacrimal glands 311

the maxillary nerve) and the emissary echo-poor with respect to the retrobulbar
veins fat surrounding them
Bony margins of the orbit • Individual layers of the globe cannot be
• Roof: orbital plate of frontal bone and resolved separately
lesser wing of sphenoid • Most sensitive modality for detecting
• Medial wall: ethmoid bone, lacrimal bone, retinal detachment
frontal process of maxilla and body of CT
sphenoid • Modality of choice for assessing orbital
• Floor: orbital surface of zygomatic bone, trauma
orbital plate of maxillary bone and orbital • Lens is of high attenuation compared with
process of palatine bone adjacent structures
• Lateral wall: zygomatic bone and greater
wing of sphenoid MRI
• Modality of choice for assessing orbital
Nerve supply of the orbital muscles soft tissues, optic nerve sheath complex,
chiasma, cavernous sinus and cranial
• Lateral rectus: sixth cranial nerve nerves
• Superior oblique: fourth cranial nerve • Fat saturation images help delineate the
• All other muscles: third cranial nerve optic nerve and surrounding subarachnoid
• Mnemonic: LR6–SO4–3 CSF from adjacent retrobulbar fat

Papilloedema
• Disc swelling from raised intracranial
Visual field defects: relationship pressure
between structure affected and • Usually bilateral
clinical defect • Causes include a space-occupying
lesion, benign intracranial hypertension,
Optic nerve: monocular blindness hydrocephalus and cerebral oedema
Optic chiasma: bitemporal hemianopia
Optic tracts: incongruous homonymous Papillitis
hemianopia • Refers to disc swelling from other causes
Lateral geniculate bodies: incongruous (not papilloedema)
homonymous hemianopia • Optic neuritis: viral infection, multiple
Optic radiations: incongruous sclerosis, systemic lupus erythematosus
homonymous hemianopia and other vasculitides
Occipital cortex: congruous • Optic nerve infiltration: sarcoidosis,
homonymous hemianopia leukaemia, lymphoma
• Infection: syphilis, cytomegalovirus
infection, lyme disease, toxoplasmosis
Imaging of the eye • Vascular: anterior ischaemic optic
neuropathy, central retinal vein occlusion,
Ultrasound diabetic papillopathy
• Performed using an 8 MHz or a 10 MHz
probe Optic neuritis
• Can be placed on closed lid or
anaesthetised conjunctiva • Sudden onset reduced vision
• Aqueous and vitreous humour are • Painful eye movements
normally echo-free • Swollen optic nerve, which enhances
• Cornea, lens and retrobulbar fat are echo- • Usually resolves spontaneously
bright
• Extraocular muscles and optic nerve are

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312 Central nervous system, and head and neck

Horner’s syndrome • Commonest cause of unilateral and


bilateral proptosis in adults
• Ptosis, meiosis and pseudoenophthalmos
• Rarely can cause optic atrophy from raised
with or without anhydrosis
intraorbital pressure
• Caused by any interruption of sympathetic
• Disease usually ‘burns out’ within 2–3
supply to the orbit
years
• Brainstem causes include multiple
sclerosis, syringomyelia and lateral Imaging features
medullary syndrome
CT
• Peripheral causes include Pancoast’s
• Proptosis: globe protrusion > 21 mm
tumour, cervical sympathectomy
anterior to the lateral orbital wall
operation and carotid dissection (which is
• Swollen muscle bellies with sparing of
painful)
their tendinous insertions
• Hypertrophy and increased density of the
Causes of enophthalmos retrobulbar fat
• Swollen optic nerve sheath complex
• (Eye shrunken in orbit)
• Orbital rim fracture (blow-out)
MRI
• Swollen muscle bellies have a smooth edge
• Parinaud’s syndrome
• Muscles of low signal on T1WI and T2WI
• Dehydration
in chronic stage
• Thin ‘stretched’ optic nerve
Ultrasound
Third nerve palsy • Swollen muscle bellies are echo-bright
• Ptosis, fixed dilated pupil, eye turned • In severe cases there may be flow reversal
down and out in ophthalmic vein
• Medical causes (which are painless)
include any mononeuritis, e.g. diabetes Causes of intraorbital muscle
• Surgical causes (which are painful) include swelling
PICA aneurysm
• Graves ophthalmopathy
Thyroid eye disease • Pseudotumour
• Acromegaly
• Lymphocytic infiltration of orbital contents • Orbital cellulites
that spares the globe • Orbital myositis
• Caused by long-acting thyroid stimulating • Leukaemia
antibodies • Lymphoma
• Particularly affects the muscle cone and • Metastases
orbital fat; inferior rectus muscle followed • Sarcoidosis
by medial rectus muscle are the muscles • Vasculitides
most commonly involved • Carotid–cavernous sinus fistula
• There is no correlation with blood • Cavernous sinus thrombosis
thyroxine level
• 10% of patients are euthyroid; it can be first
presentation of Graves
• Classically occurs following treatment of
hyperthyroidism Orbital pseudotumour
• Females affected four times more than • Idiopathic lymphocytic infiltration of
males orbital contents
• 80% of cases are bilateral • Associated with vasculitis, sarcoidosis and
• Gradual onset of painless bilateral retroperitoneal fibrosis
proptosis and ophthalmoplegia

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Orbit and lacrimal glands 313

• Disease of middle-aged adults: equal Imaging features


incidence in men and women
Ultrasound
• Commonest cause of an intraorbital mass
• Echo-poor collection of vessels
in an adult
• Intralesional venous flow increases with
• Rapid onset of painful proptosis and
the Valsalva manoeuvre
ophthalmoplegia
• Majority of cases are unilateral CT
• Diagnosis of exclusion because it mimics • Ideally performed with jugular vein
infection, lymphoma and sarcoidosis pressure
• Can affect any orbital structure but five • Serpigenous collection of vessels which
main disease patterns exist: enhance avidly
–– Lacrimal (commonest)
–– Diffuse, mainly involving orbital fat and Orbital tumours
muscle cone
–– Periscleritis Intraconal tumours
–– Myositis • Optic glioma
–– Perineuritis • Optic meningioma
Imaging features • Haemangiomas
• Melanoma
• There is a wide spectrum of findings
• Metastases
CT • Retinoblastoma
• Unilateral proptosis
• Swollen lacrimal gland Extraconal tumours
• Swollen muscle bellies and tendons • Dermoid
• Infiltrative mass lesion • Rhabdomyosarcoma
MRI • Lymphoma
• Swollen muscle bellies have a ‘ragged’ • Orbital extension of adjacent tumours
edge
• Muscles of low signal on T1WI and T2WI
Optic glioma
in chronic stage • Commonest tumour of the optic nerve
• Thickened optic nerve sheath complex • Presents with progressive reduction in
• Characteristic avid enhancement of sclera, visual acuity
optic nerve and mass • Majority occur in children and are of low
histological grade
Tolosa–Hunt syndrome • Rare in adults, but typically high grade
with a poor prognosis
• Associated with neurofibromatosis type 1
• Spread of inflammatory process into
(not type 2)
cavernous sinus via superior orbital
• Commonly extends through optic canal
fissure or orbital apex
into the chiasm
• Retro-orbital pain and cranial nerve
palsies Imaging features
MRI (modality of choice)
• Modality of choice
Orbital varix • Lesion central, within optic nerve sheath
complex
• Intraorbital varicose veins that dilate with
• Obliteration of surrounding subarachnoid
Valsalva manoeuvre or coughing and
space
cause intermittent proptosis
• Isointense on T1WI, high signal on T2WI
• One third develop phleboliths within the
• Avid contrast enhancement
varix

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314 Central nervous system, and head and neck

• 90% have a cutaneous haemangioma


Causes of enlarged optic canal • Spontaneously involute in first year of life
(> 5 mm)
Choroidal haemangioma
• Optic glioma • Can cause retinal detachment in young
• Orbital pseudotumour adults
• Graves’ ophthalmopathy • Associated with Sturge–Weber syndrome
• Ophthalmic artery aneurysm • Can involve any part of the choroid
• Neurofibroma Cavernous haemangioma
• Sarcoidosis
• Proptosis in an adult
Not caused by raised intracranial
• Large intraconal vascular channels
pressure, which narrows the canal
• Phleboliths may form within them
• Well-defined lesion appearing as high
signal on T2WI
Optic meningioma
• Majority occur in adults, arising from
Melanoma
arachnoid cap cells • Commonest ocular malignancy of
• Associated with neurofibromatosis type 2 adulthood
(not type 1) • Arises in choroidal layer of the globe
• Progressive reduction in visual acuity • Extends posteriorly, and the majority
• Usually confined to the orbit cause retinal detachment

Imaging features Imaging features


CT Ultrasound
• Modality of choice • Very sensitive test, with 95% accuracy
• Enlarged optic nerve sheath complex • Ill-defined echo-bright mass
• 50% are calcified • Detached retina, seen as an undulating
• Display peripheral enhancement: ‘tram bright line parallel to back of globe
track’ sign in the axial plane and ‘ring’ sign MRI
in the coronal plane • Modality of choice for assessing
extrascleral extension
Causes of enlarged optic nerve • Appears as a ‘contrasting mass’
sheath complex –– T1WI: vitreous humour is dark and
melanoma is bright
• Optic neuritis –– T2WI: vitreous humour is bright and
• Orbital pseudotumour melanoma is dark
• Graves ophthalmopathy
• Optic glioma Metastases
• Optic meningioma • Majority occur in the highly vascular
• Leukaemia choroidal layer
• Lymphoma • Common, but majority remain clinically
• Subarachnoid haemorrhage silent
• Ophthalmic artery aneurysm • In a child, commonest primary tumours
• Sarcoidosis are neuroblastoma are Ewing’s sarcoma
• In an adult, commonest primary tumours
are carcinoma of the breast and lung
Haemangioma Imaging features
Capillary haemangioma Ultrasound
• Proptosis in a newborn • Ill-defined echo-bright mass (cannot be
distinguished from a melanoma)

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Orbit and lacrimal glands 315

MRI
• Key feature distinguishing it from a Poor prognostic signs (>50%
melanoma is isointensity of the metastatic mortality)
lesion on T1WI)
• Invasion of choroidal layer
Retinoblastoma • Lack of calcifications
• Commonest ocular malignancy of • Extension along the optic nerve
childhood • Contrast enhancement
• One third are hereditary and are usually
bilateral
• Two thirds occur sporadically and are
usually unilateral
Dermoid
• Clinically, patients have leukocoria (white • Common benign tumour that presents in
pupil) childhood
• Tumour mass arises from posterior aspect • Contains tissue from all three germ cell
of the globe layers
• Propensity for extension along the optic • Commonest location is superolateral
nerve aspect of the anterior orbit
• Meningeal metastases can occur via Imaging features
subarachnoid space
CT
Causes of leukocoria (white pupil) • Low attenuation
• May contain calcifications
• Retinoblastoma (50% of cases) MRI
• Retinopathy of prematurity • High signal on T1WI and T2WI
• Coats’ disease • May contain calcifications
• Persistent hyperplastic vitreous
• Toxocariasis infection
Rhabdomyosarcoma
• Congenital cataract • Commonest childhood intraorbital
malignancy (mean age 7 years)
• 60% arise in the genitourinary system, 40%
in the head and neck
Imaging features • Arises from the orbital soft tissues (not the
Ultrasound muscles)
• Echobright mass • Orbital disease presents with rapid onset
• Foci of calcification (80%) of proptosis and visual loss
• Retinal detachment (100%) • 80% occur posterior to globe in the
extraconal space
MRI (modality of choice)
• 20% occur within the tissues of the eyelid
• Modality of choice
• Aggressive expansile soft tissue mass
• Appears as a ‘contrasting mass’
• Metastasises to lungs and bone
–– On T1WI, vitreous humour is dark and
• Responds well to radiotherapy
tumour is bright
• Ill-defined enhancing soft tissue mass with
–– On T2WI, vitreous humour is bright and
bone destruction seen on CT
tumour is dark
• Calcifications seen as areas of signal void

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316 Central nervous system, and head and neck

Lymphoma cavernous sinus


• Orbital structures are markedly
• Majority are non-Hodgkin’s B-cell type
oedematous
• Presents with painless proptosis (mean
• Pressure induced optic neuropathy if not
age 60 years)
treated promptly
• Commonest locations are anterior
extraconal space, lacrimal gland and Imaging features
extraocular muscles
MRI
• Expansile soft tissue mass with no
• Dilated cavernous sinus and superior
characteristic imaging features
ophthalmic vein
• Commonly calcifies following
chemotherapy or radiotherapy Catheter angiography
• ICA contrast leak with early filling of
superior ophthalmic vein
Orbital trauma
Orbital rim fractures Lacrimal apparatus
• Direct blow to the orbit causes an acute
pressure rise
Anatomy
• Decompression via a ‘blow-out’ fracture • The oval-shaped lacrimal gland is lodged
• Inferior wall is the commonest location in the lacrimal fossa on the medial side of
followed by medial wall the zygomatic process of the frontal bone
• Divided into a small inferior part and a
Inferior wall large superior part by the aponeurosis of
• Tear-drop sign of inferior rim fracture levator palpebrae superioris
• Air–fluid level in maxillary sinus • Produces tears that drain by 12 or so small
• Orbital emphysema ducts into the lateral extent of the superior
fornix
Medial wall
• Tears accumulate in the medial canthal
• Fracture of lamina papyracea
area
• Ethmoid sinus opacification
• Drainage is via the superior and inferior
• Orbital emphysema
lacrimal puncta, located on the medial
Complications aspect of the upper and lower eyelid
• Entrapment diplopia of inferior rectus, margins
inferior oblique or medial rectus muscles • Puncta are continuous with the superior
• Infraorbital nerve damage causing and inferior canaliculi, which drain into
periorbital paraesthesia the nasolacrimal sac, which is housed
within a bony fossa, formed anteriorly by
Vitreous haemorrhage the frontal process of maxillary bone and
• Ultrasound is modality of choice posteriorly by the lacrimal bone
• Echo-bright debris seen within the • The nasolacrimal sac narrows inferiorly
normally echo-free vitreous to become the nasolacrimal duct
(approximately 20 mm in length) which
Carotid–cavernous sinus fistula runs within the bony nasolacrimal canal
• Base of skull fracture causing laceration of • The nasolacrimal canal is approximately
intracavernous ICA 2 mm in diameter and has a slightly
• Rarely caused by rupture of an ICA posterolateral course; it is formed laterally
aneurysm by medial wall of maxillary sinus and
• ICA communicates with cavernous sinus medially by the lacrimal bone
and arterial blood feeds the ophthalmic • It opens through the valve of Hasner into
veins, causing pulsatile proptosis the inferior meatus, beneath the inferior
• Palsy of all nerves passing through the turbinate

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Orbit and lacrimal glands 317

Enlarged lacrimal gland Causes


• Sarcoidosis
Causes • Sjögren‘s syndrome
• Infection (dacryocystitis) • Lymphoma
• Orbital pseudotumour • Leukaemia
• Sarcoidosis • Tuberculosis
• Sjögren’s syndrome
• Lymphoma Nasolacrimal duct obstruction
• Pleomorphic adenoma (commonest • Presents with excessive eye watering
benign tumour) (epiphora)
• Adenoid cystic carcinoma (commonest
malignant tumour), which arises in Causes
the orbital part of the gland, extends • Congenital: imperforate valve of Hasner
posteriorly and is associated with adjacent • Post-infectious
bony sclerosis • Post-traumatic
• Iatrogenic, e.g. sinus drainage operations
Mikulicz’s syndrome
• Enlarged lacrimal and salivary glands

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6.11 Spine

Anatomy Cross sectional anatomy


• Spinal cord extends from the medulla • Central H-shaped region of grey matter
oblongata to the conus medullaris • Anterior horns contain cell bodies of the
• Conus lies at L3 level at birth; it is normally motor neurones
above L2 in adulthood • Posterior horns contain cell bodies of the
• 31 pairs of spinal nerves originate from the sensory neurones
cord, and each pair has an anterior and • White matter tracts are located
posterior nerve root peripherally
• C1–C7 spinal nerves exit above the –– Dorsal columns sense vibration and
corresponding pedicle proprioception
• C8 nerve exits above the T1 pedicle –– Lateral columns sense pain and
• Thoracic and lumbosacral nerves exit temperature
below the corresponding pedicle Blood supply
• Spinal nerves take a downward course
from the cord to exit foramen; this is most • Single anterior spinal artery
pronounced in the lumbosacral region –– Formed from a branch of each vertebral
• Nerves exiting below the level of the conus artery
(the cauda equina) are contained within –– Descends anteriorly in the midline
an expanded dural sac which ends at S2 sulcus
• The cord expands in two regions: –– Supplies anterior two thirds of the cord
–– Cervical (most pronounced), between • Paired posterolateral spinal arteries
C3 and T2 –– Each arises from PICA (branch of the
–– Thoracic, between T9 and T12 vertebral artery)
–– Descend along posterolateral aspect of
Filum terminale the cord
• Thin (normally < 2 mm) fibrous filament –– Supply posterior third of cord
that projects downwards from the apex of • Radiculomedullary arteries
the conus and attaches to first coccygeal –– Branches of the intercostal arteries
segment –– Enter spinal canal through the vertebral
• Continuous with the pia matter lining the foramina
cord –– Anastomose with the spinal arteries
• Surrounded by descending nerves of the around the cord
cauda equina –– Play a major role in reinforcing cord
blood supply
Central canal of spinal cord –– Largest radicular branch is the artery of
• Continuous with the fourth ventricle Adamkiewicz, which arises from a left
• Has a short fusiform dilatation within the intercostal artery between T9 and T12
conus (the terminal ventricle)
• Extends for a short distance into the filum Arteriovenous
terminale
• Position within the cord changes over its
malformations
course Arteriovenous fistula
–– Anterior in cord in the cervical and
• Account for 80% of spinal arteriovenous
thoracic spine
malformations
–– Central in cord in the lumbar spine
• Acquired lesion, which usually presents in
–– Posterior in cord in the conus
middle age
medullaris

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Spine 319

• Abnormal communication between a


radiculomedullary artery and intradural Cord ischaemia
veins, which leads to cord hypoperfusion
• Thoracic cord is the commonest location Causes
• Patients present with slowly progressive • Trauma
back pain and leg weakness, which mimics • Atherosclerosis
spinal stenosis • Aortic dissection
• A recognised complication is thrombosis • Hypotension, e.g. following repair of
of the draining veins with rapid cord abdominal aortic aneurysm
infarction (the Foix–Alajouanine • Vasculitis
syndrome) • Arteriovenous fistula
• Hypercoagulable states
Imaging features
MRI Evolution of MRI findings in cord
• Flow voids from large dural veins in region ischaemia
of fistula 1. High T2 signal in paired anterior grey
• Signs of associated cord infarction matter horns: earliest sign
–– High T2 signal in paired anterior grey 2. High T2 signal develops in paired
matter horns: earliest sign posterior horns (‘butterfly sign’)
–– High T2 signal develops in paired 3. High T2 signal of entire cord cross-
posterior horns (‘butterfly sign’) sectional area
–– High T2 signal of entire cord cross- 4. Cord enhancement: late sign indicating
sectional area infarction
–– Cord enhancement: late sign indicating
infarction

Intramedullary arteriovenous
malformation Myelitis
• Accounts for 20% of spinal arteriovenous Causes
malformations • Multiple sclerosis (commonest cause)
• Congenital lesion which usually presents • Vasculitis, e.g. systemic lupus
in young adults erythematosus
• Thin-walled vessels within the cord • Acute disseminated encephalomyelitis
parenchyma connect the arterial • Viral infection, e.g. AIDS-related human T
and venous systems and are prone to lymphotropic virus-1 infection
haemorrhage • Sarcoidosis
• Even distribution throughout the cord
• Patients present with sudden onset of Multiple sclerosis
upper or lower limb neurology • Involves spinal cord without brain lesions
in 10% of cases
Imaging features
• Cervical spine is the most commonly
MRI affected region
• Intramedullary blood breakdown products
Imaging features
MRI
• Proton density and T2 short T1 inversion
recovery (STIR) are the most sensitive
sequences

Ch-06.indd 319 8/12/2010 12:19:10 PM


320 Central nervous system, and head and neck

• FLAIR is a poor sequence for spinal cord Imaging features


multiple sclerosis
MRI
• Eccentric high-signal plaques that align
• Gadolinium is used to distinguish
parallel to long axis of corpus callosum
between fibrous tissue and recurrent disc
• Plaques typically occupy less than half the
herniation
cross-sectional area of the cord
• Epidural fibrosis enhances avidly (because
• Plaques typically extend over less than
it is highly vascular)
three vertebral body lengths
• Disc herniation enhances little if at all
• Plaques show enhancement in the acute
phase
Epidural haematoma
Arachnoiditis • Epidural space contains fat and the
epidural venous plexus
• Inflammation of the subarachnoid space
• Haemorrhage is venous, and the thoracic
• Commonest cause is following spinal
spine is the commonest location
surgery
• Most bleeds are posterior because the dura
• Other causes include meningitis and
is fixed to vertebral bodies
subarachnoid haemorrhage myodil
• Patients present with sudden-onset back
contrast agent (used in myelograms);
pain or lower limb paralysis
some are idiopathic
• Causes include lumbar puncture, trauma,
Imaging features anticoagulant medication, haemophilia
and arteriovenous malformation
MRI
• Most cases occur below the level of the Imaging features
conus
MRI
• Nerve roots are clumped centrally or
• Sagittal sequences best demonstrate the
adhere peripherally to dural sac (empty
collection of blood
sac sign)
• Anterior focal displacement of the dural
sac (curtain sign)
Subacute combined • Blood breakdown products have variable
degeneration of the cord signal characteristics

• Caused by pernicious anaemia (atrophic


gastritis) and low levels of vitamin B12 in Epidural abscess
macrocytic anaemia • Spread of infection from adjacent discitis
• Dorsal columns of cord affected first or osteomyelitis
• Rarely caused by haematogenous spread
Imaging features
• Staphylococcus aureus is the commonest
MRI pathogen
• Can be normal • Thoracic spine is the commonest site
• Cervical spine is the most commonly involved
involved • Most abscesses are located anterior to the
• Continuous long segment of high T2 signal dural sac
in dorsal columns • Present with back pain, fever and raised
white cell count or C-reactive protein
Epidural fibrosis • Can cause cord compression with acute
neurological features
• Common cause of failed back surgery
• Fibrous tissue accumulates in the epidural Imaging features
space, causing localised pressure effects MRI
• T2WI show high-signal, ovoid lesion that
extends over several levels

Ch-06.indd 320 8/12/2010 12:19:10 PM


Spine 321

• T1WI post-contrast shows linear Tuberculous discitis


enhancement along posteriorly displaced
• Caused by spread of infection from the
dura and around margins of abscess (pus
basivenous plexus
does not enhance)
• Commonest site is the thoracolumbar
junction region
Vertebral body metastases
Pathophysiology and imaging
• Lung and breast and myeloma are the features
commonest primary tumours in adults
• Infection begins at the endplate
Imaging features (characteristic focal defect)
• The adjacent disc becomes involved but
MRI
destruction here is slow and disc space
• Vertebral body collapse fracture
height is typically preserved in the early
• T1WI shows low signal foci compared with
stages
normal high-signal marrow fat
• Infection tracks beneath the anterior or
• T2 STIR shows high-signal foci compared
posterior longitudinal ligaments to involve
with saturated ‘dark’ fat
multiple vertebral bodies with collapse
• Standard T2 sequences are unreliable for
and kyphosis
detecting metastases
• Complications are paravertebral and
epidural abscess formation
Discitis
Pyogenic discitis Degenerative disc disease
• Usually caused by blood bourne spread Imaging features
• Risk factors include spinal surgery and
intravenous drug use MRI
• Staphylococcus aureus is the commonest • Loss of normal T2 signal of nucleus
pathogen pulposus (caused by dehydration)
• Non-specific symptoms of low back pain • Narrowed intervertebral disc space
and fever • Endplate changes
• Usually confined to one disc space and –– Modic type I: low T1 and high T2 signal,
adjacent vertebral bodies caused by oedema
• Complications are paravertebral and –– Modic type II: high T1 and high T2
epidural abscess formation signal, caused by fat (the commonest
type)
Imaging features –– Modic type III: low T1 and low T2
Plain X-ray signal, caused by sclerosis
• It is several weeks before changes can be • Weakened annulus fibrosis and posterior
detected longitudinal ligaments
• Early features are narrowing of the disc Disc bulge
space and irregularities of the endplate
• Late features are endplate sclerosis and • Weakened annulus fibrosus
bony fusion Disc protrusion
Technetium-99m bone scan • < 3 mm beyond the vertebral margin
• Detects changes much earlier than plain • Ruptured annulus fibrosus, intact nucleus
radiographs pulposus
MRI
• Modality of choice (sensitivity > 90%)
Disc herniation
• Endplates have low T1 and high T2 signal • > 3 mm beyond the vertebral margin
• High T2 signal within the affected disc • Ruptured annulus fibrosus and posterior
• Disc shows marked contrast enhancement longitudinal ligament

Ch-06.indd 321 8/12/2010 12:19:10 PM


322 Central nervous system, and head and neck

• Commonest at C5–C6, C6–C7 and L4–L5, • Discovertebral erosions and disc


L5–S1 levels calcification
• Posterolateral herniation is commonest • Calcification of supraspinous ligament
pattern (dagger sign)
• Ankylosis of apophyseal and
Atlantoaxial subluxation costovertebral joints

• Atlantodens interval > 3 mm in an adult Complications of ankylosing


and > 5 mm in a child spondylitis
Causes • Spinal fractures
• Trauma • Apical fibrosis
• Pharyngitis or retropharyngeal abscess • Aortic regurgitation
• Congenital (trisomy 21, Marfan’s • Amyloidosis
syndrome, Morquio’s syndrome) • Atrioventricular conduction defects
• Rheumatoid arthritis • Atlantoaxial subluxation
• Juvenile rheumatoid arthritis
• Ankylosing spondylitis
• Systemic lupus erythematosus
• Os odontoideum
• Aplasia of the dens Diffuse idiopathic skeletal
• Gout hyperostosis
• Calcium pyrophosphate dihydrate disease
• Typically occurs in males >50 years age
• HLA-B27 positive in 30% of cases
Spinal fusion • Associated with retinoids (vitamin A
analogues)
Causes • Commonest in thoracic spine
• Diffuse idiopathic skeletal hyperostosis • Causes back stiffness and reduced range of
• Ankylosing spondylitis movements
• Juvenile rheumatoid arthritis
• Reiter’s syndrome Imaging features
• Psoriasis Plain X-ray
• Infections (pyogenic infection more • Flowing right-sided paravertebral
commonly than tuberculosis) ossification over four or more vertebral
• Klippel–Feil syndrome levels
• Osteophytes are on anterolateral aspects of
Ankylosing spondylitis vertebral bodies
• Disc spaces well maintained and often
• Seronegative spondyloarthropathy calcified
• Typically occurs in young males • Ligamentous ossification and
• HLA-B27 positive in 95% of cases osteophytosis in pelvis or lower limbs
• Insidious onset of low back pain and
stiffness Diffuse idiopathic skeletal
Imaging features hyperostosis versus ankylosing
spondylitis
Plain X-ray
• Bilateral sacroiliitis • No corner erosions
• Sclerosis and squaring of vertebral body • No sacroilitis
corner (Romanus lesion) • No apophyseal joint ankylosis
• Calcification of the annulus fibrosis
(syndesmophyte)

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Spine 323

Spondylolisthesis • Paget’s disease


• Giant vertebral body haemangioma with
Lytic spondylolisthesis collapse
• Caused by repetitive minor trauma • Vertebral metastases with collapse
• Occurs in young athletes, e.g. fast bowlers • Epidural lipomatosis
• Bilateral stress fractures of pars
interarticularis Plain X-ray signs
• Widened spinal canal and foraminal
stenosis Widened interpedicular
Imaging features
distance
Causes
Plain X-ray
• ‘Scottie dog’ view: lateral spine with • Trauma (burst fracture)
patient turned 45° • Longstanding raised intraspinal pressure
• Most commonly occurs at L5–S1 level –– Ependymoma
• Break in neck of ‘Scottie dog’ –– Communicating hydrocephalus
• L5 vertebral body slips forward but • Dural ectasia
posterior elements stay behind –– Neurofibromatosis type 1
• Posterior step is above level of slip –– Marfan’s syndrome
–– Ehlers–Danlos syndrome
CT • Myelomeningocele and associated
• Continuous facet sign on axial sections conditions
–– Syringomyelia
Degenerative spondylolisthesis –– Diastematomyelia
• Caused by severe apophyseal joint
osteoarthritis Posterior scalloping
• Occurs in the elderly
• No pars interarticularis fractures
Causes
• Narrowed spinal canal and foraminal • Longstanding raised intraspinal pressure
stenosis • Dural ectasia
• Acromegaly
Imaging features • Achondroplasia
Plain X-ray • Mucopolysaccharidoses, e.g. Morquio’s
• Most commonly occurs at L4–L5 level syndrome
• Apophyseal degenerative changes
• L4 vertebral body and posterior elements Achondroplasia
slip forward
• Posterior step is at the level of slip • Autosomal dominant
• Rhizomelic dwarfism (short humerus
and femur)
Spinal stenosis Anteroposterior lumbar X-ray:
• Commonest sites are cervical and lumbar • Progressive narrowing of interpedicular
spine distance
• Rounded iliac bones
Causes • Horizontal acetabular roofs
• Congenital: achondroplasia, trisomy 21, Lateral lumbar X-ray:
Morquio’s syndrome • Posterior scalloping and inferior
• Degenerative: hypertrophy of facet joints beaking
or ligamentum flavum, disc herniation • Short pedicles (spinal stenosis)
• Fracture

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324 Central nervous system, and head and neck

Sclerotic pedicle Causes of chondrocalcinosis


Causes
• Unilateral spondylolysis in chronic stage • Wilson’s disease
• Lysis of contralateral pedicle • Hyperparathyroidism
• Lymphoma • Haemochromatosis
• Osteoid osteoma • Idiopathic
• Pseudogout
Absent pedicle • Acromegaly
Causes • Diabetes
• Lytic metastasis • Onchronosis
• Multiple myeloma • Gout
• Giant cell tumour
• Aneurysmal bone cyst
• Congenital absence
Intramedullary spinal
Narrow intervertebral disc tumours
spaces
Ependymoma
Causes
• Commonest cord tumour in adults
• Degenerative change
• Associated with neurofibromatosis type 2
• Scheuermann’s disease
• Arise from ependymal cells lining the
• Discitis (pyogenic infection more common
central canal
that tuberculosis)
• Commonest location is in the conus
• Ankylosing spondylitis
medullaris
• Ochronosis
• Recognised ectopic sites: presacral, broad
ligament of ovary
Hint • Low-grade slow growing tumour: average
3 years to presentation
• Diffuse idiopathic skeletal hyperostosis:
joint spaces are well maintained Imaging features
• Metastases: do not affect the joint Plain X-ray
space • Posterior scalloping
• Wide interpedicular distance
• Pedicle erosion
Intervertebral disc calcification MRI
• Localised fusiform expansion of cord by
Causes spherical solid mass
• Any cause of chondrocalcinosis • Mildly low signal on T1WI and T2WI
• Degenerative changes • Homogeneous enhancement
• Ankylosing spondylitis • Common findings at upper and lower
• Diffuse idiopathic skeletal hyperostosis poles of tumour are polar cysts, cord syrinx
• Discitis and the ‘cap’ sign (a haemosiderin rim
• Trauma from prior haemorrhage)
• Spinal fusion

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Spine 325

Astrocytoma Imaging features


• Commonest cord tumour in children MRI
• Commonest location is the thoracic spine • Small foci of high T2 signal (best seen on
• Low grade slow growing tumour T2 STIR)
• Extensive surrounding oedema and avid
Imaging features enhancement
Plain X-ray
• Posterior scalloping Extramedullary intradural
• Wide interpedicular distance
• Pedicle erosion
spinal tumours
MRI Leptomeningeal
• Localised fusiform expansion of the cord carcinomatosis
• More cystic ill-defined lesion than • Tumour seeding along the pia and
ependymoma arachnoid layers
• Eccentrically located • Commonest causes in children are
• Heterogeneous enhancement of solid medulloblastoma, ependymoma,
components germinoma and pinealoblastoma
• Common findings at upper and lower • Commonest causes in adults are
poles of tumour are polar cysts and cord metastases from breast, lung, melanoma
syrinx or lymphoma
• Tumour haemorrhage is uncommon
Imaging features
Haemangioblastoma MRI
• Nodular leptomeningeal enhancement
• 20% of all haemangioblastomas occur in • Serial lumbar punctures more sensitive
the spinal cord than MRI
• 20% associated with von Hippel–Lindau
disease
Meningioma
Imaging features
• 10% of all meningiomas are spinal
MRI • Slowly growing dura-based lesion
• Large cystic mass with an enhancing mural • Extrinsic cord compression with
nodule radiculopathy or myelopathy
• Prominent flow voids from vessels feeding • Commonest location is the dorsal thoracic
the nodule spine
• Adjacent cord syrinx
• No calcification Imaging features
MRI
Medullary metastases • Signal characteristics as per other
meningiomas
• Primary tumours include carcinoma of
the lung (commonest), carcinoma of the
breast and melanoma Nerve sheath tumours
• Commonest location is cervical and • Benign, slow growing tumours arising
thoracic cord from spinal nerve roots

Ch-06.indd 325 8/12/2010 12:19:10 PM


326 Central nervous system, and head and neck

• Dumbbell shaped lesions with an intra- • Presents with low back pain
and extradural component • Large presacral mass (average 10 cm)
• Grow out through neural foramina • Extends anteriorly, displacing the bladder
(widened on plain X-ray) and rectum
• Two main types: neurofibroma and
schwannoma Causes of a presacral mass
Neurofibroma • Chordoma
• Associated with neurofibromatosis type 1 • Rectal carcinoma
• Cervical spine is the commonest location • Anterior meningocoele
• No enhancement • Teratoma
Schwannoma • Metastases
• Cervical spine is the commonest location • Ependymoma
• Avid enhancement

Extradural tumours Chordoma of the clivus


Epidural metastases • Large expansile tumour
• Anteriorly invades the sphenoid sinus and
• Commonest extradural spinal tumour nasopharynx
• Commonest location is the dorsal lumbar • Superiorly invades the sella turcica,
spine cavernous sinuses or orbits
• Commonest primary tumours are • Laterally invades the cerebellopontine
carcinoma of the breast, carcinoma of the angle
lung, melanoma and lymphoma • Posteriorly invades the pons
Chordoma Imaging features
• Locally invasive and destructive malignant CT
tumour • Low-attenuation soft tissue mass with
• Arises from embryonic notochord bone destruction
remnants • Tumour matrix contains bone fragments
• Mean age 60 years and calcifications in 50% of cases
• 20% have metastases at time of diagnosis
from the liver or lung MRI
• Location • Low-signal tumour contrasts with clivus
–– Clivus (35%) marrow fat on T1WI
–– Vertebrae (15%) • Heterogeneous lesion containing cystic
–– Sacrum (50%) areas and foci of calcification on T2WI
–– Maxilla, mandible or scapula (rare) • Heterogeneous enhancement
Nuclear imaging
Chordoma of the sacrum • No uptake on Technetium-99m bone scan
• Commonest sacral tumour

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6.12 Temporal bone

Anatomy the pars tensa


• Round head of malleus articulates with the
• The temporal bone is composed of two incus in the epitympanic recess: ‘icecream
parts cone’ on axial section
–– Squamous part, which forms the floor • Incus articulates with the stapes, which
of the middle cranial fossa attaches to the oval window via its
–– Petrous part, which houses middle and footplate
inner ear cavities and mastoids • Tensor tympani muscle (mandibular
nerve) attaches to malleus
Middle ear cavity • Stapedius muscle (supplied by the eighth
Mesotympanum cranial nerve) attaches to body of the
• Central portion of middle ear cavity stapes
• Lateral wall is formed by tympanic
membrane, which attaches superiorly to
Internal auditory canal
the scutum and inferiorly to the limbus • Bony canal running from cerebellopontine
• Medial wall has four components, from angle to the inner ear
top to bottom: • Transmits seventh and eighth cranial
–– Bulge of the lateral semicircular canal nerves
–– Bulge of the facial nerve canal • Seventh cranial nerve lies anterior to the
–– Oval window (where the stapes eighth at the meatus
footplate attaches) • The porus acousticus is the posterior bony
–– Bulge of the cochlea promontory lip of the meatus
• Internal auditory canal divided into
Hypotympanum superior and inferior sections by a bony
• Inferior portion of middle ear cavity septum
• Connects to the Eustachian tube orifice • Superior contains facial nerve and
• A thin plate of bone separates it from the superior vestibular nerve
jugular bulb • Inferior section contains the cochlear and
inferior vestibular nerve
Epitympanic recess • These nerves pierce a bony plate to reach
• Superior portion of middle ear cavity the cochlea and vestibular apparatus
• Contains head of the malleus and body of • The seventh nerve then runs anteriorly
the incus before bending around the cochlea to
• Lateral wall is formed by the scutum enter the facial canal in the medial wall
• Connects to mastoid air cells via a of the middle ear cavity, where it runs
posterior opening, the aditus posteriorly
Prussak’s space CT in temporal bone imaging
• Region between the scutum and the head
• High-resolution images acquired by using
of malleus
1–2 mm slice thickness
Tegmen tympani • Performed via a low-amperage, high-
• Thin plate of bone, which forms the roof of voltage technique
the middle ear cavity • Coronal imaging lowers the orbital dose
• Separates the middle ear from the middle
cranial fossa Pathology
Bony ossicles Otitis media
• Lateral process of malleus attaches to the • Middle ear infection
central portion of the tympanic membrane • Usually a clinical diagnosis

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328 Central nervous system, and head and neck

Imaging features • Failure of normal involution of fetal


epidermoid cell rests
CT and MRI
• Usually arises centrally within middle ear
• Middle ear opacification and fluid levels in
cavity
the mastoids
• Presents in childhood with conductive
Gradenigo’s syndrome deafness
• No history of otitis media
• Apical petrositis secondary to otitis media
• Tympanic membrane is intact
• Triad of features
–– Otitis media Acquired cholesteatoma
–– Lateral rectus palsy (supplied by the
• Accounts for 98% of cholesteatomas
sixth cranial nerve)
• Accumulation of squamous epithelial cells
–– Retro-orbital pain (supplied by the fifth
in the middle ear cavity
cranial nerve)
• Arises in epitympanic recess (Prussak’s
• May also get excessive lacrimation through
space)
involvement of the greater superficial
• Usually presents with conductive deafness
petrosal nerve
• Can cause meningitis, brain abscesses,
Imaging features sigmoid sinus thrombosis, facial nerve
palsy and sensorineural deafness
CT and MRI
• Associated with chronic otitis media
• Mastoid opacification
• Tympanic membrane is perforated
• Bone destruction
(usually a pars flaccida perforation)
• Enhancing inflammatory tissue at petrous
apex Imaging features
Otitis externa CT (early)
• Soft tissue mass between the scutum and
• Infection of the external ear canal
the head of malleus
• Associated with diabetes
• Erosion of the scutum
• Commonest pathogen is Pseudomonas
• Medial displacement or erosion of the
• Presents with otalgia and purulent
ossicles
discharge
• Left untreated can cause osteomyelitis and CT (late)
even meningitis • Non-enhancing soft tissue mass filling the
• CT or MRI are used to assess the extent of middle ear cavity
local invasion • Extension through aditus to involve the
mastoid air cells
Exostosis of the external • Superior erosion through tegmen tympani
auditory canal • Erosion into the seventh cranial nerve
• Common benign tumour canal and bony labyrinth
• Associated with swimming in cold water MRI
• Bony overgrowth that can narrow or • Soft tissue mass is isointense to brain on
obliterate the canal TIWI
• A secondary cholesteatoma may form • Mass is mildly high signal on T2WI
adjacent to the exostosis
Cerebellopontine angle
Cholesteatoma lesions
• Benign, slow growing accumulation of
• Three main types of lesions
epithelial debris in the middle ear cavity
–– Acoustic schwannoma (80%)
Congenital cholesteatoma –– Meningioma (10%)
–– Epidermoid (5%)
• Accounts for 2% of cholesteatomas

Ch-06.indd 328 8/12/2010 12:19:11 PM


Temporal bone 329

• Other causes of lesions Meningioma


–– Arachnoid cyst
• Displays typical features on CT and MRI
–– Trigeminal schwannoma
–– Cholesterol granuloma
Meningioma versus acoustic
–– Dermoid
schwannoma: distinguishing features
–– Metastases
–– Endolymphatic sac tumour
• No widening of the internal auditory
Acoustic schwannoma meatus
• Benign, slow growing tumour of the eighth • No erosion of the porus acousticus
cranial nerve • Dural tail enhancement in 70% of cases
• Associated with neurofibromatosis type 2 • Adjacent temporal bone hyperostosis
(bilateral schwannoma is pathognomonic • Calcified in 20% of cases
of this condition) • High attenuation with non-contrast CT
• Majority arise from the superior vestibular
portion within the internal auditory canal
• Tumour expands within IAC and Epidermoid
exits proximally into the cistern of the
• Benign congenital lesion
cerebellopontine angle, where it usually
• Slow build-up of squamous epithelial cells
grows posteriorly (anterior growth is
(like a cholesteatoma)
limited by the facial nerve)
• Encases the adjacent neurovascular
• Tumour encases adjacent neurovascular
structures
structures
• Presents in middle age with seventh and
• Unusual cause of progressive unilateral
eighth cranial nerve palsies
sensorineural deafness
• More than 50% occur at the
• Rarely presents with facial nerve palsy
cerebellopontine angle
Imaging features • Can also occur in the suprasellar region
CT Imaging features
• Widening of the internal auditory meatus
CT
• Erosion of the porus acousticus
• Low-attenuation (fat-density), lobulated
• Dumbbell shaped soft tissue mass which
lesion
enhances avidly
• 20% are calcified
• No calcifications
• No enhancement
MRI
MRI
• Tumour is high signal on T2WI, and large
• Variable signal depending on fat and water
tumours contain cystic areas
content
• Tumour enhances avidly on post-
• Commonly high signal on T2WI
gadolinium T1WI; this is the best modality
for detection of very small lesions
Epidermoid versus acoustic
schwannoma: distinguishing features
Anterior inferior cerebellar artery
• No widening of the internal auditory
• First branch of the basilar artery meatus
• Runs through the cistern of the • No erosion of the porus acousticus
cerebellopontine angle • Calcified in 20% of cases
• Normally enhances post-contrast
• Can mimic a small acoustic neuroma

Ch-06.indd 329 8/12/2010 12:19:11 PM


330 Central nervous system, and head and neck

• Associated with Pendred’s syndrome


Epidermoid versus arachnoid cyst: • Causes a progressive sensorineural
distinguishing features deafness in childhood
• Onset of deafness is associated with minor
MRI FLAIR head trauma
• Epidermoids retain some signal
• Arachnoid cysts that are filled with CSF Imaging features
appear dark CT
MRI (DWI) • Enlarged vestibular aqueduct (> 1.5 mm)
• Epidermoids appear bright (because of
restricted diffusion) Pendred’s syndrome
• Arachnoid cysts appear dark
Autosomal-recessive condition with:
• Sensorineural deafness
• Mondini malformation
Childhood deafness • Enlarged vestibular aqueduct
• Goitre
Causes
• 50% are acquired, particular through
infection in utero (notably the TORCH
infections: Toxoplasmosis; Other Otosclerosis
infections, e.g. HIV, syphilis; Rubella; • Idiopathic deafness caused by bony
Cytomegalovirus; Herpes) sclerosis
• 50% are hereditary • Usually bilateral, with onset around
–– Mondini malformation puberty
–– Enlarged vestibular aqueduct syndrome • Identical imaging appearances to those
–– Otosclerosis seen in osteogenesis imperfect; stapes–
oval window articulation seen in 90% of
Mondini malformation cases
• Incomplete formation of the cochlea,
which has 1.5 turns instead of the normal Imaging features
2.5 CT
• Causes congenital sensorineural deafness • In conductive deafness, thickened stapes
• Associated with Pendred’s syndrome and excess soft tissue at the oval window;
10% involves cochlea
Imaging features • In sensorineural deafness, cochlea appears
CT and MRI thickened and sclerotic
• Cystic dilatation of the cochlea and
vestibular apparatus Temporal bone sclerosis and
deafness
Enlarged vestibular aqueduct
syndrome Occurs in:
• Aqueduct is a narrow bony canal in the • Paget’s disease
petrous temporal bone • Fibrous dysplasia
• It contains the endolymphatic duct, which • Osteopetrosis
drains endolymph from the inner ear to • Meningioma
the endolymphatic sac in the posterior • Otosclerosis
cranial fossa • Osteogenesis imperfecta
• In this condition the aqueduct is • Syphilis
abnormally dilated

Ch-06.indd 330 8/12/2010 12:19:11 PM


Nose and paranasal
6.13 sinuses

Anatomy • Useful for defining sinus anatomy and


extent of sinus disease
The nose
• Upper one third of the external nose is
Structures vulnerable to injury
bony; the lower two thirds is cartilaginous • Several important structures are
• The septum is composed of the ethmoid vulnerable to injury at FESS and CT
bone, vomer and quadrilateral cartilage provides vital information to the surgeon
• Each lateral nasal wall has three medial regarding these
projections: the upper, middle and lower Anterior ethmoidal artery
turbinates (conchae) • Originates from the ophthalmic artery
• The cavities are lined by ciliated and runs medially between the superior
respiratory epithelium oblique and medial rectus muscles
• Communicates posteriorly with the • Enters the anterior ethmoid sinus via
nasopharynx via the choana the cribroethmoid foramen and courses
• Blood supply is from the anterior and medially along its superior aspect before
posterior ethmoidal arteries (branches piercing the cribriform plate to enter the
of the ophthalmic artery) and the anterior cranial fossa
sphenopalatine artery (the terminal • Marks the superolateral boundary for
branch of the maxillary artery) FESS.
Paranasal sinuses Optic nerve
• Frontal sinus drains into the frontal recess • Related to the posterior ethmoid air cells
• Maxillary sinus drains via the (especially the Odoni cell anatomical
infundibulum and hiatus semilunaris variant) and the superolateral aspect of the
• Anterior ethmoid air cells drain into the sphenoid sinus
ethmoid infundibulum
• These drainage pathways unite as the ICA
osteomeatal complex, which opens into • Intimately related to the sphenoid sinus
the middle meatus (between the middle • A deviated intersphenoid septum may
and inferior turbinates) attach to the carotid canal
• Sphenoid sinus and posterior ethmoid • Rarely the thin bone separating the ICA
air cells together drain via the and the sphenoid sinus is absent.
sphenoethmoidal complex, which opens Ethmoid roof
into the superior meatus (between the
• Thin piece of bone composed of the
middle and superior turbinates)
cribriform plate and fovea ethmoidalis
• Easily damaged, causing a CSF leak
Functional endoscopic sinus • Such leaks may not present clinically for
surgery (FESS) several weeks, in contrast to those caused
by skull base fractures, which are usually
• Minimally invasive technique using a evident immediately
fibreoptic endoscope
• Used to restore mucociliary drainage
in patients with chronic sinus disease Anatomical variants
unresponsive to medical therapy • Concha bullosa: a large pneumatised
• Preoperative CT is mandatory middle turbinate, which can obstruct the

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332 Central nervous system, and head and neck

ethmoid infundibulum or osteomeatal • Chronic sinusitis: mucosal thickening


complex (secretions of soft-tissue attenuation)
• Haller cells: ethmoid air cells extending
beneath the floor of the orbit; they Complications
are intimately related to the maxillary • Orbital cellulitis
infundibulum and, if large, can obstruct it • Intracranial extension (meningitis,
• Odoni cell: a posterior ethmoid air cell that abscesses, subdural empyema)
extends superiorly and laterally to abut the • Mucocele
optic nerve.
Mucormycosis
Nasal septal perforation • Fungal sinusitis affecting
Causes immunocompromised patients and
patients with diabetics
• Cocaine
• Aggressive infection, which can spread to
• Septal surgery
the orbits, cavernous sinus and brain
• Nasal intubation
• Wegener’s granulomatosis and other Imaging features
vasculitides
CT and plain X-ray
• Sarcoidosis
• Sinus opacification without air–fluid level
• Lymphoma
• Adjacent bony destruction
• Tuberculosis
• High attenuation on CT (contains
• Syphilis
manganese)

Bacterial sinusitis Mucocele


• Inflammation of the paranasal sinus
• End stage of a chronically obstructed
mucosa
paranasal sinus ostium
• Begins as a viral infection with secondary
• Causes include chronic sinusitis, nasal
bacterial invasion
polyps and osteomas
• Risk factors
• Sinus fills with secretions
–– Cystic fibrosis
• Commonest expansile lesion of the
–– Kartagener’s syndrome (situs inversus,
paranasal sinuses
bronchiectasis)
• Commonest site is frontal sinus
–– Nasal septal deviation
• Can cause proptosis, diplopia, visual loss
–– Horizontal uncinate process
and CSF rhinorrhoea
• Commonest site is isolated maxillary
• An important complication is secondary
sinusitis, followed by frontal sinusitis
infection (mucopyocele), which behaves
• Least common site is sphenoethmoidal
like a large abscess and is a surgical
sinusitis
emergency
• If acute sinusitis does not resolve
adequately with medical therapy Imaging features
(decongestants, antibiotics), chronic
CT
sinusitis may develop.
• Expanded sinus opacified by low-
• FESS is used in these cases to improve
attenuation material
sinus drainage
• Pressure erosion or sclerosis of sinus wall
Imaging features • May contain peripheral calcifications
• Rim enhancement post-contrast (tumours
CT and plain X-ray
enhance centrally)
• Acute sinusitis: air–fluid level in sinus
(secretions of water attenuation)

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Nose and paranasal sinuses 333

MRI • Well-defined, expansile, lucent lesion with


• Variable signal depending on duration: a ground-glass texture
• Acute: high signal on T2WI (because of • 80% are monostotic (localised to a single
high water content), low T1 bone)
• Chronic: low signal on T2WI (because • Commonest sites are the ribs, the femoral
water has been resorbed), low T1 neck and the craniofacial area

Causes of sinus opacification and Craniofacial fibrous dysplasia


bone destruction • Frontal bone is the commonest site, and it
can obliterate the frontal sinus
• Inverting papilloma • Lesion widens the diploic space but spares
• Lymphoma the inner table
• Fungal sinusitis • Skull base lesions are usually sclerotic
• Squamous cell carcinoma • Can mimic a variety of other lesions, e.g.
• Wegener’s granulomatosis meningioma
• Juvenile angiofibroma • Symptoms include headaches and facial
• Tuberculosis deformity (proptosis)
• 0.5% risk of transformation into
osteosarcoma or fibrosarcoma
Imaging features
Nasal polyps Plain X-ray
• Inflammatory swellings of the sinonasal • Wide range of appearances; can be lucent,
mucosa sclerotic or mixed
• Associated with allergic and infective CT
rhinitis • Expansile lesion with a ground-glass
• Common in adults texture
• Rare in children unless associated with
cystic fibrosis MRI
• Usually bilateral within maxillary or • Low T1, low T2, with intense enhancement
ethmoid sinuses Technetium-99m bone scan
• Well-defined soft tissue masses • Increased uptake
• Frequent cause of sinus obstruction
Wegener’s granulomatosis
Antrochoanal polyp • Necrotizing vasculitis of small and
• Large unilateral polyp arising from the medium-sized arteries
maxillary antrum • Positive for classical antineutrophil
• Typically occurs in young adults cytoplasmic antibodies (cANCA),
• Widens the sinus ostium and extends back specifically proteinase-3 (PR3)
into the middle meatus • 80% have nose and paranasal sinus
• A large polyp can pass through the involvement
posterior choana into the nasopharynx • Causes bloody sinus discharge, epistaxis
and collapse of the nasal septum
Fibrous dysplasia • Other findings
–– Otitis media
• Developmental disease of bone –– Subglottic stenosis
• Normal marrow is replaced by fibrous –– Arthritis
tissue –– Neuropathy (mononeuritis multiplex)

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334 Central nervous system, and head and neck

–– Multiple lung cavitatory lesions (in 90%


of cases) Gardner’s syndrome
–– Glomerulonephritis (in 90% of cases,
and the commonest cause of death) Autosomal-dominant inheritance

TRIAD:
Benign tumours • Multiple osteomas
Inverting papilloma • Colonic polyposis
• Commonest benign tumour of the • Soft tissue tumours, e.g. desmoids, and
paranasal sinuses increased risk of duodenal and other
• Peak incidence in the sixth decade carcinomas
• Possible viral aetiology (human papilloma
virus)
• Commonest location is lateral nasal wall
• Polypoid growth with mass effect, causing Malignant tumours
proptosis and facial deformity
• Requires complete surgical excision
Squamous cell carcinoma
because approximately 10% transform into • Commonest paranasal malignant tumour,
squamous cell carcinoma accounting for 80% of cases
• Can arise from inverting papilloma
Imaging features • Majority arise in the maxillary sinus
CT • Soft tissue mass with bone destruction
• Polypoid soft tissue mass • 20% of patients have cervical
• Adjacent bony deformity or destruction lymphadenopathy at time of diagnosis
MRI Lymphoma
• Intermediate signal on T2WI and high
• Account for 5% of malignant tumours of
signal in adjacent inflamed tissues
the sinuses
Osteoma • Majority are non-Hodgkin’s B-cell
lymphomas
• Slow growing, bone-forming tumour
• Peak incidence in the seventh decade
• Majority occur in the frontal sinuses
• Presents with nasal obstruction
• Usually an incidental plain film finding
• Commonest location is the maxillary
• Can obstruct the frontoethmoidal
sinus, followed by the nasal cavity
complex, causing a mucocele
• Bulky soft tissue mass with bone
• If large can cause facial deformity and
destruction
proptosis
• No specific imaging features to distinguish
• Multiple osteomas are associated with
it from squamous cell carcinoma
Gardner’s syndrome
Imaging features Metastases
• Rare
Plain X-ray
• Commonest primary is renal cell
• Solid, homogeneous, well-defined
carcinoma
radiodensity
• Commonest sinus involved is maxillary
CT sinus
• Modality of choice to assess local pressure • Typically presents with epistaxis (because
effects they are hypervascular)

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Nose and paranasal sinuses 335

Esthesioneuroblastoma • Treatment is surgical resection and


radiotherapy
• Rare
• High rate of recurrence
• Neurogenic tumour arising from the
olfactory sensory epithelium in the roof Imaging features
of the nasal cavity (undersurface of the
cribriform plate)
CT and MRI
• Enhancing mass in the upper nasal cavity
• Bimodal age distribution, with peak
with local bone destruction
incidences among teenagers and in the
sixth decade
• Presents with nasal obstruction, epistaxis
Causes of anosmia
and anosmia
• Locally aggressive with submucosal spread • Old age
in all directions: through the cribriform • Frontal lobe tumours
plate into the frontal lobes, into the orbits • Olfactory groove meningioma
and into the ethmoid sinuses • Esthesioneuroblastoma
• 20% of patients have cervical
lymphadenopathy at time of diagnosis

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6.14 Salivary glands

Anatomy mylohyoid muscle


• Drained by several small ducts into
Parotid gland the floor of the mouth, posterior to the
• Composed of superficial and deep lobes submandibular duct orifice.
• Larger superficial lobe lies anterior to the
mastoid process, ramus of mandible and
Minor salivary glands
masseter muscle • The greatest concentration of these small
• Smaller deep lobe passes between ramus submucosal glands is in the hard and soft
of mandible anteriorly and styloid process palates
posteriorly (stylomandibular tunnel) • They are also found in the side walls of the
• Parotid ‘tail’ is a small inferior extension, oral cavity and on the oropharynx, lips and
which wraps around the angle of the tongue
mandible
• Facial nerve exits from the stylomastoid Infections
foramen and runs through the gland in a
• Can be viral, e.g. mumps, or bacterial
plane between superficial and deep lobes
• Stones predispose to bacterial infections
• Parotid gland normally contains around 20
intraparotid lymph nodes Imaging features
• The retromandibular vein and ECA pass
Ultrasound
upwards through the parotic gland and
• Swollen glands are echo-poor with a
deep to the nerve (with the artery deep to
heterogeneous texture
the vein)
• Parotid duct (Stensen’s duct) runs along
the surface of masseter muscle before Sjögren’s syndrome
turning medially to pierce buccinator
• Clinical triad
muscle
–– Xerostomia (dry mouth), with enlarged
• Parotid duct opens into the mouth above
salivary glands
the second upper molar tooth
–– Keratoconjunctivitis sicca (dry eyes),
Submandibular gland with enlarged lacrimal glands
–– Autoimmune disease, e.g. rheumatoid
• Lies medial to the body of mandible in the
arthritis, systemic lupus erythematosus,
submandibular fossa
scleroderma
• Wedged between the mylohyoid and
• Progressive non-obstructive salivary gland
digastric muscles
duct dilatation
• Separated from parotid by deep cervical
• Associated with an increased risk of
fascia and Kuttner’s node
salivary gland lymphoma
• Continuous with a smaller, deep part,
which passes around the posterior border Imaging features
of mylohyoid to lie deep to this muscle
Ultrasound
• Submandibular duct (Wharton’s duct)
• Enlarged salivary glands
opens into the floor of the mouth beside
• Containing multiple small round
the frenulum of the tongue
echo-poor areas
• Similar ultrasound appearance to the
• Increased vascularity seen on colour
parotid gland
Doppler scans
Sublingual gland Sialogram
• Lies in the floor of the mouth beneath • Multiple small cavities of contrast
the mucus membrane and above the accumulation

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Salivary glands 337

MRI • Occasionally extends below the mylohyoid


• Enlarged glands containing multiple cysts muscle and presents as a neck swelling
of varying sizes (‘plunging ranula’)
• Speckled ‘honeycomb’ appearance on • If large may cause dysphagia and airway
T2WI compromise
• CT and MRI are used to assess the extent
of these large lesions
Sarcoidosis
• Salivary glands involved in 30% of cases
• Painless enlargement
Benign salivary gland
tumours
Imaging features
• 80% of benign tumours occur in the
Ultrasound parotid gland
• Enlarged glands, echo-poor • The smaller the gland involved, the more
CT likely the tumour is to be malignant
• Enlarged glands with multiple small foci of
high attenuation Pleomorphic adenoma
• Commonest benign salivary gland tumour,
Uveoparotid fever (Heerfordt’s accounting for 80%
syndrome) • Majority found in superficial lobe of
parotid
TRIAD • Tend to recur following removal
• Bilateral uveitis • Malignant transformation is recognised
• Bilateral parotitis Imaging features
• Facial nerve palsies
Ultrasound
• Well-defined, echo-poor, oval mass with
post-acoustic enhancement
• May contain areas of dystrophic
Salivary gland stones calcification
• 80% of stones occur in the submandibular • Perilesional blood flow with small
gland branches running centrally
• 20% of stones occur in the parotid MRI
• Majority of stones are radiopaque • Very high signal on T2WI
Imaging features Adenolymphoma (Warthin’s
Ultrasound tumour)
• Echo-bright foci with post-acoustic • Accounts for 10% of benign salivary gland
shadowing tumours
• Commonest location of submandibular • Commonest location is in tail of parotid
stones is within the duct gland (the superficial lobe)
Sialogram • Commoner in men than women
• Filling defects, associated strictures and • 10% are bilateral
proximal duct dilatation • Well-defined mass lesion
• More complex appearance than
pleomorphic adenoma
Ranula
• Mucus-retention cyst causing a swelling
Imaging features
on the floor of the mouth Ultrasound
• Most commonly involves the sublingual • Well-defined, echo-poor lesion with
glands internal echoes

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338 Central nervous system, and head and neck

CT • Present as a hard ‘craggy’ mass and facial


• Heterogeneous cystic or solid lesion nerve palsy

Parotid haemangioma Cylindroma (adenoid cystic


• Commonest parotid mass in young carcinoma)
children • Commonest in females, with an average
• Develops in first few months of life age of 60 years
• Progressively enlarges over subsequent • Commonest malignant tumour of the
months minor salivary glands
• Most spontaneously regress by teenage • Uncommon in the parotid gland
years • Aggressive tumour with a propensity for
early perineural spread
Imaging features
Ultrasound Imaging features
• Echo-poor mass (because it contains Ultrasound
vascular spaces) • Ill-defined lesion of mixed echotexture
• Displays internal blood flow • Infiltration around adjacent vessels
MRI • Cervical lymphadenopathy
• Soft tissue mass containing prominent CT and MRI
flow voids • More accurate than ultrasound at
assessing the degree of local invasion and
Malignant salivary gland perineural involvement

tumours Metastases
Mucoepidermoid tumour • Commonest primary tumours are
melanoma and squamous cell carcinoma
• Commonest malignant salivary gland • Imaging features are non-specific
tumour • On ultrasound, typically appear as small
• Majority occur in the parotid gland echo-poor lesions
• Commonest malignant parotid tumour

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6.15 Pharynx

Anatomy Oropharynx
• Vertical fibromuscular tube extending • Lies posterior to the oral cavity:
from skull base to cricoid cartilage, where circumvallate papillae of the tongue mark
it merges with the oesophagus the transition point
• Lies in front of the cervical prevertebral • Includes posterior one third of the tongue
fascia and behind the nasal cavity, oral and the lingual tonsils
cavity and larynx • Contains two muscular folds in its lateral
• There are three layers walls, the palatoglossal arch (anteriorly)
–– Mucosa, the innermost layer and the palatopharyngeus arch
–– Submucosa (also known as the (posteriorly)
pharyngobasilar fascia) • Between these arches lie the palatine
–– Muscular layer, the outer coat tonsils
composed of superior, middle and
inferior pharyngeal constrictors Waldeyer’s ring
• Constrictor muscles diverge anteriorly but
form a continuous overlapping posterior • Ring of lymphoid tissue that lies around
layer the oropharynx and the nasopharynx
• The pharynx is described in three parts • Includes the adenoids and the lingual
–– Nasopharynx: skull base to level of soft and palatine tonsils
palate
–– Oropharynx: soft palate to hyoid bone
–– Laryngopharynx: hyoid bone to cricoid
cartilage (C6)
Laryngopharynx
• Lies posterior to the larynx
Nasopharynx • Continuous inferiorly with the oesophagus
• Continuous anteriorly with the nasal cavity • Contains two deep lateral recesses (the
through the choana piriform fossae)
• Lined by respiratory epithelium • Lateral walls are supported by the inferior
• Roof is applied to the skull base and slopes pharyngeal constrictor muscle
beneath sphenoid bone and clivus • An anatomical weak point where its lower
• Lateral walls supported by superior fibres diverge is called Killian‘s dehiscence;
pharyngeal constrictor muscle this is the site of a Zenker diverticulum
• Eustachian tube pierces its lateral walls on
either side Thornwald cyst
• Torus tubarius is a ridge posterior to each
tubal orifice, formed by the levator veli • Developmental thin-walled cyst
palatine muscle and the cartilaginous (notochordal remnant)
portion of the eustachian tube • Occurs in 2% of the population
• Behind these ridges are the paired lateral • Located in midline on the posterior wall of
pharyngeal recesses of Rosenmüller nasopharynx
• The submucosal layer contains numerous • Contains proteinaceous fluid
minor salivary glands and lymphoid tissue • Can become infected and rupture
• The adenoids are aggregations of lymphoid Imaging features
tissue in the midline roof; they are
prominent in children and regress from CT
puberty onwards • Low-attenuation and non-enhancing
lesion

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340 Central nervous system, and head and neck

MRI Malignant tumours of the


• High signal on T1WI and T2WI
pharynx
Benign tumours of the Nasopharyngeal carcinoma
pharynx (70%)
• Accounts for 70% of malignant tumours of
Juvenile angiofibroma the pharynx
• Commonest benign tumour of the • Squamous cell carcinoma
nasopharynx • High incidence in Chinese and other Asian
• Very locally aggressive and highly vascular men
• Supplied by maxillary or ascending • Associated with Ebstein–Barr virus
pharyngeal artery • Aggressive tumour that infiltrates into
• Mean age 15 years adjacent tissue spaces
• Occurs almost exclusively in males • Late presentation is typical
• Commonest clinical features are nasal • Most tumours arise in the lateral fossa of
speech (caused by nasal obstruction), Rosenmüller
severe recurrent epistaxis and facial • 90% of patients have lymphadenopathy at
deformity time of diagnosis
• Arises in region of the sphenopalatine • Lateral retropharyngeal nodes of Rouvière
foramen at the junction of the nose and and the cervical chain lymph nodes (most
nasopharynx commonly levels II and V) are commonly
• Biopsy is contraindicated involved
Pathways of tumour invasion Pathways of tumour invasion
• Lateral invasion • Lateral invasion (earliest)
–– Into the pterygopalatine fossa, which is –– First sign is obliteration of fat strip
widened in 90% of cases between tensor palatine and levator
–– Through the pterygomaxillary fissure, palatine muscles
which is widened in 90% of cases –– Grows into parapharyngeal space,
–– Into the infratemporal fossa obliterating the fat there
–– Erosion of the medial pterygoid plate –– Further lateral extension is into the
• Superior invasion masticator space
–– Into the sphenoid sinus (in 70% of • Anterior invasion
cases) and the middle cranial fossa –– Blocks the Eustachian tube orifice,
• Inferior invasion causing otitis media and mastoiditis
–– Into the soft palate and the oropharynx –– Into the pterygopalatine fossa
• Posterior invasion –– Into the posterior aspect of the nasal
–– Filling the nasopharyngeal airway cavity
• Anterior invasion • Posterior invasion
–– Into the nasal cavity –– Into the retrostyloid space, which
contains the carotid sheath and the
Imaging features ninth, tenth and eleventh cranial nerves
CT –– Into the prevertebral muscles
• Soft tissue mass that enhances early and • Superior invasion
avidly –– Into the skull base and sphenoid sinus
• Modality of choice to assess extent of local –– Through the foramen lacerum (which
invasion and bony destruction contains the ICA) and into cavernous
MRI sinus
• Prominent flow voids are seen within the –– Through the foramen ovale into the
lesion (which contains large vessels) middle cranial fossa

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Pharynx 341

Masticator space Pathology of related spaces


Retropharyngeal space
• Lateral to the parapharyngeal space • Between pharyngeal constrictor muscles
• Contains muscles of mastication (the anteriorly and prevertebral muscles
medial and lateral pterygoids, masseter posteriorly
and temporalis) • Contains fat and lymph nodes
• Ramus of mandible • A mass here displaces the prevertebral
• Mandibular nerve muscles posteriorly
Retropharyngeal abscess
Imaging features • Spread of infection from tonsils
CT Nodal metastases
• Soft-tissue attenuation mass
• Common in head and neck squamous cell
MRI cancers
• Modality of choice • Lateral nodes in the retropharyngeal
• Isointense to muscle on T1WI, which is the space are the nodes of Rouvière, which
best sequence to assess for obliteration of are involved early in nasopharyngeal
the fat plane carcinoma
• Intermediate signal on T2WI
• Solid tumour elements enhance avidly Prevertebral space
• Deep to the retropharyngeal space
Non-Hodgkin’s lymphoma • Extends from the skull base to the superior
• Accounts for 20% of malignant tumours of mediastinum
the pharynx • Pathology here displaces the prevertebral
• Arises in lymphoid tissue of Waldeyer’s muscles anteriorly
ring
• Disease of the elderly, presenting with Contents of the prevertebral space
nasal obstruction
• Bulky soft tissue mass, which grows in a • Prevertebral muscles (longus coli and
circumferential pattern capitis)
• Late invasion of parapharyngeal spaces • Vertebral artery and veins
• Presence of large non-necrotic cervical • Phrenic nerve
lymph nodes • Scalene muscles
• Systemic signs, e.g. splenomegaly • Brachial plexus
• Imaging features suggestive of lymphoma

Cystic adenoid carcinoma


• Accounts for 10% of malignant tumours of Pre-vertebral abscess
the pharynx • Anterior spread from vertebral body
• Commonest malignant tumour of the osteomyelitis
minor salivary glands Metastatic invasion
• Soft palate is the commonest site
• Anterior extension of vertebral body
• Aggressive lesion that invades the adjacent
metastases
parapharyngeal spaces
• Early perineural spread into the skull base
and cavernous sinuses

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342 Central nervous system, and head and neck

Parapharyngeal space Imaging features


• Slit-like space located lateral to the Ultrasound
nasopharynx, between the pharyngeal • Oval, well-defined, echo-poor mass in the
constrictor muscles and the masticator lateral neck
space • Splays the (CCA bifurcation
• The styloid process, ICA and jugular vein • Encases the CCA but does not narrow it
lie posteriorly CT
• Bounded superiorly by the skull base • Well-defined soft tissue mass that has
• Contains fat, mandibular nerve branches homogeneous intense enhancement
and the ascending pharyngeal artery
MRI
• Important anatomically in that it can be
• Solid areas of high signal with flow voids
invaded by several tumours
on T2WI
–– Posterolateral invasion by tumours in
• ‘Salt-and-pepper’ appearance
the deep lobe of the parotid gland
–– Medial invasion by nasopharyngeal Angiogram
carcinoma • Intense blush (because it is a highly
–– Posterior invasion by vagus nerve vascular tumour)
paraganglioma • Head and neck paragangliomas are
–– Inferior invasion by carotid bulb supplied by the ascending pharyngeal
paraganglioma artery and its branches
Nuclear scan
Paraganglioma • Indium-111 octreotide is highly sensitive
for these tumours
• Rare, slow growing tumour that arises from
paraganglia cells Vagus nerve paraganglioma
• Amine precursor uptake decarboxylase • Arises from paraganglia cells of the vagus
(APUD)-type tumours nerve perineurium
• Commonest site is the adrenal • Commonest at the nodose ganglion
(phaeochromocytoma) • Slow growing, painless neck mass behind
• Head and neck paragangliomas occur at the angle of the mandible
four main sites • Characteristically displaces the ICA
–– Carotid bulb anteromedially and internal jugular vein
–– Vagus nerve posterolaterally
–– Jugular bulb • Large tumours can invade into the
–– Cochlear promontory parapharyngeal space
• 25% invade the sympathetic ganglia,
Carotid bulb paraganglioma causing a Horner’s syndrome
• Arises from paraganglia cells within the
adventitia of the carotid bulb Jugular bulb paraganglioma
• Commonest paraganglioma of the head (glomus jugulare)
and neck (accounting for 60%)
• Arises from paraganglia cells in the
• Slow growing, pulsatile neck mass at the
adventitia of the jugular bulb
anterior border of sternocleidomastoid,
• Causes pulsatile tinnitus
just lateral to the hyoid bone.
• Expands to erode the jugular foramen
• 2% secrete catecholamines
• Can invade superiorly into the middle ear
• 5% undergo malignant transformation
cavity

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Pharynx 343

• Can invade posterosuperiorly along the Cochlear promontory


jugular vein into the posterior cranial fossa
• Can cause jugular vein thrombosis paraganglioma
• Involvement of adjacent cranial nerves • Arises from paraganglia cells of the
(ninth, tenth and eleventh) is Vernet’s tympanic plexus (ninth and tenth cranial
syndrome nerves) on the cochlear promontory
• Commonest tumour of the middle ear
Jugular foramen • Presents with pulsatile tinnitus and
conductive deafness
• Between petrous temporal and • Red tumour mass seen behind the
occipital bones eardrum
• Posteromedial to the styloid process • Tumour remains confined to the middle
• Separated from hypotympanum above ear cavity
by a thin plate of bone
• Asymmetrical (right is larger than left) Causes of pulsatile tinnitus
• Contains the jugular vein, inferior
petrosal sinus, and the ninth, tenth and • Glomus jugulare (commonest)
eleventh cranial nerves (the twelfth • Glomus tympanicum
cranial nerve exits the skull via the • No cause identified
hypoglossal canal) • Meningioma
• Carotid dissection
• Carotid fibromuscular hyperplasia
• Aberrant ICA

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6.16 Larynx

Anatomy Piriform fossae


• Paired lateral recesses of the
Divisions of the larynx laryngopharynx, which make up the
posterior wall of the paraglottic space on
each side
• Supraglottic region: the epiglottis to
• Help to channel food towards the
the upper border of the true cords
oesophagus during swallowing
• Glottic region: the true cords
• Tumours here can invade into the
• Subglottic region: below the true cords
paraglottic space and reach the vocal cords
to the trachea
• False cords are situated just above the
true cords Benign tumours of the
• A thin cleft between them is called the larynx
ventricle
• A small pouch projects laterally from Squamous papilloma
each ventricle (the saccule) • Commonest benign tumour of the larynx
• The arytenoid cartilages lie posteriorly • Caused by human papilloma virus
on the upper surface of the cricoid • Commonly occur on the vocal cords
cartilage and span the level of the • Presents with hoarseness, stridor,
ventricle haemoptysis and recurrent chest
• Each arytenoid cartilage has a vocal infections
process where the true cords attach • In children, usually multiple (laryngeal
• Thyroarytenoid muscle makes up the papillomatosis)
bulk of the true cords • In adults, usually solitary
• Complications include airway compromise
• Can spread to involve the trachea, bronchi
Paraglottic space or lungs
• Transformation into squamous cell
• Lateral space between inner lining of
carcinoma can occur but is rare
larynx and thyroid cartilage
• Contains fat and lymphatics
• Inferiorly it contains the thyroarytenoid Malignant tumours of the
muscle larynx
• An important site of supraglottic tumour
extension Laryngeal carcinoma
• Vast majority are squamous cell
Pre-epiglottic space carcinomas
• Anterior space between thyroid cartilage • Risk factors are smoking and alcohol
or the thyrohyoid membrane and the
epiglottis Supraglottic tumours
• Continuous inferiorly with the paraglottic • Account for 35% of laryngeal carcinomas
space • Present late
• Contains fat and lymphatics • Metastases common at time of diagnosis
• Another important site of supraglottic • Spread into the paraepiglottic space and
tumour extension the paraglottic space and to the jugular
• A key area radiologically in that extension lymph nodes
into the pre-epiglottic space cannot be • Optimal surgery is partial laryngectomy,
assessed endoscopically preserving the true cords

Ch-06.indd 344 8/12/2010 12:19:13 PM


Larynx 345

• Usually represent inferior extension of a


Contraindications to partial glottic tumour
laryngectomy, i.e. requires total • Metastases common at time of diagnosis
laryngectomy • Paraoesophageal and mediastinal lymph
nodes may be involved
• Thyroid cartilage invasion • Requires total laryngectomy because the
• Cricoid cartilage invasion cricoid cartilage is invariably involved
• Extension into the true cords • Majority are non-operable, owing to
• Extension into the piriform fossae extensive invasion into surrounding
structures, e.g. the trachea
• CT and MRI are complementary to
endoscopic evaluation of these tumours Other pathology
• Imaging provides information regarding
endoscopic ‘blind spots‘, e.g. nodal disease
Laryngocele
• Signs of cartilage invasion (CT is modality • Dilatation of either saccule of the laryngeal
of choice) are cartilaginous erosions and ventricle
soft tissue beyond the outer margins of • Caused by raised intraglottic pressure
cartilage (seen on axial image) • 80% unilateral
• Filled with air or fluid
Glottic tumours • Increases in size with Valsalva manoeuvre
• Account for 50% of laryngeal carcinomas • 50% caused by a laryngeal tumour
• Present early with hoarseness obstructing the saccule
• Typically arise from anterior half of the • Also associated with glass blowing and
cord excessive coughing
• Metastases uncommon at time of • A large tumour can extend through the
diagnosis (the true cords have no thyrohyoid membrane to present as an
lymphatics) anterior neck mass
• Optimal surgery is laser resection if very
localised; if more advanced, surgery is a Subglottic stenosis
vertical hemilaryngectomy (removing the • Worse on inspiration (unlike
true and false cord on one side) tracheomalacia, which is worse on
expiration)
Contraindications to • May be congenital
hemilaryngectomy, i.e. requires total • Other causes include post-tracheostomy,
laryngectomy prolonged endotracheal tube placement,
Wegener’s granulomatosis and relapsing
• Tumour extending across anterior polychondritis
commissure to involve more than one
third of the opposite cord Extramedullary plasmacytoma
• Thyroid cartilage invasion • Rare
• Cricoid cartilage invasion • Discrete mass of monoclonal plasma cells
• Extension superiorly into the false cord within the soft tissues
• Inferior extension > 1 cm • 90% occur in the head and neck
• Commonest sites are the upper airways
and conjunctiva
Subglottic tumours • Lobulated soft tissue mass with local bone
and cartilage destruction
• Rare
• CT or MRI is used to assess extent of local
• Present late
invasion prior to resection

Ch-06.indd 345 8/12/2010 12:19:13 PM


346 Central nervous system, and head and neck

Rheumatoid arthritis • Horizontal fractures are best assessed by


coronal reformats
• Can affect the cricoarytenoid joint, but
there are no specific radiological findings Cricoarytenoid joint dislocation
• Normally the arytenoid cartilages are seen
Trauma symmetrically, perched on the cricoids
Cricoid and thyroid fractures • Asymmetry of cartilage in axial plane
• Vertical fractures are easily identified by images
axial CT

Ch-06.indd 346 8/12/2010 12:19:13 PM


6.17 Mandible

Anatomy syndrome, Treacher–Collins syndrome,


progeria
• Each half of the mandible is composed of • Acquired causes include juvenile
–– Horizontal body rheumatoid (Still’s disease) and fetal
–– Angle alcohol syndrome
–– Vertical ramus
–– Coronoid process anteriorly Mandibular fractures
–– Condylar process posteriorly • Commonest site is the condylar neck,
• The mandible houses the mandibular followed by the ramus, the angle and the
canal, which runs from the inner aspect of body
the ramus to open on the external aspect • Fractures are commonly bilateral (the
of the body (mental foramen) mandible acts like a bony ring)
• The inferior alveolar nerve (a branch of • Occlusal films can help detect subtle
the mandibular nerve) and the inferior fractures
alveolar artery are contained within the
mandibular canal
Cystic lesions of the
Temporomandibular joint mandible
(TMJ) Radicular cyst (apical cyst)
• Commonest jaw cyst (accounting for 60%)
• Synovial joint between condyle and
• Inflammatory cyst at root of a carious
temporal bone
tooth (tooth decay)
• Contains a biconcave fibrocartilagenous
• Caused by proliferation of cells of Malassez
disc
in the periodontal ligament
• Disc lies slightly anteriorly when the
• Well-defined, unilocular cyst
mouth is closed
• Disc moves posteriorly to a neutral Residual cyst
position when the mouth is open
• Occurs following tooth extraction
TMJ subluxation Odontogenic keratocyst
• The disc lies very anterior in both the
• Developmental cyst arising from dental
closed and open positions
lamina cell rests
• T1 MRI is the best sequence to assess
• Most occur in the ramus of the mandible
subluxation
(rarely in the maxilla)
TMJ arthritis • Well-defined, unilocular cyst with
corticated margins
• Rheumatoid arthritis is the commonest
• 50% recur following removal
cause
• Multiple cysts are associated with Gorlin’s
• Erosions mainly occur on the condylar
syndrome
head
• T2 MRI is the best sequence to assess
arthritis
Gorlin’s syndrome

• Multiple basal cell carcinomas


Mandibular pathology • Multiple mandibular keratocysts
Mandibular hypoplasia • Skeletal anomalies
• Extensive soft tissue calcification (of the
(micrognathia) falx cerebri and tentorium)
• Can be part of a congenital syndrome,
e.g. Pierre Robin syndrome, Turner’s

Ch-06.indd 347 8/12/2010 12:19:13 PM


348 Central nervous system, and head and neck

Dentigerous cyst –– Encapsulated expansile benign


neoplasm
• Arises from the crown of an impacted or
–– Composed of fibrous tissue and osteoid
unerupted tooth
–– Slow growing, but cause facial
• Third mandibular molar is the commonest
asymmetry
site
–– Lucent cyst with varying degrees of
• Well-defined, corticated, cystic lesion
calcification
containing the impacted tooth
–– Intense uptake on bone scan
• Rarely develops into an ameloblastoma
• Cementoma
Ameloblastoma • Odontoma
–– Hamartoma
• Benign but locally aggressive tumour,
–– Well-defined, cystic lucency
which expands into adjacent soft tissues
–– Contains calcifications and tooth-like
• Peak age is sixth decade
structures
• Two thirds arise from enamel cells around
• Stafne bone cyst
a tooth
–– Incidental finding
• One third arise from enamel cells in
–– Aberrant lobe of submandibular gland
dentigerous cyst wall
causing a well-defined depression in
• 75% occur in the mandible, 25% in the
the body of mandible
maxilla
• Expansile multilocular cystic lesion
containing daughter cysts Lamina dura
• Commonly recurs following removal
• Cortical bone that lines the tooth socket
• CT and MRI are used to assess extent of
• Seen as a thin white line around the root
local invasion
• Separated from the tooth by the
• Rare unicystic variant, which has a better
radiolucent periodontal ligament
prognosis and is more common in the
maxilla Causes of erosion of the lamina
• Plain X-rays cannot reliable distinguish dura
between ameloblastoma, dentigerous cyst
• Re-modelling disorders
and odontogenic keratocyst
–– Hyperparathyroidism, e.g. renal
osteodystrophy
Other cystic jaw lesions –– Fibrous dysplasia
• Brown tumour –– Paget’s disease
–– Seen in hyperparathyroidism (primary –– Anaemia
> secondary) –– Osteoporosis
–– Commonest sites are the jaw, pelvis and –– Cushing’s syndrome (steroids cause
ribs demineralisation)
–– Bone is resorbed by osteoclasts and • Tumours, cysts and other lesions
replaced by fibrous tissue –– Ameloblastoma
–– Expansile lytic lesion –– Metastases
• Myeloma (common) –– Radicular cyst
• Metastases (uncommon), typically from –– Dentigerous cyst
the breast or lung –– Odontogenic keratocyst
• Haemangioma –– Ossifying fibroma
• Eosinophilic granuloma (‘floating teeth’) –– Osteomyelitis
• Fibrous dysplasia –– Periodontitis
• Simple bone cyst –– Eosinophilic granuloma
• Aneurysmal bone cyst –– Cretinism (not adult-onset
• Ossifying fibroma hypothyroidism)
–– Burkitt’s lymphoma

Ch-06.indd 348 8/12/2010 12:19:13 PM


Mandible 349

• Eruption starts at 6 months of age


Burkitt’s lymphoma • Continues until 2–3 years of age
• These are replaced by permanent teeth
• Highly malignant non-Hodgkin’s between 6 and 12 years of age
lymphoma • No deciduous teeth remain > 12 years of
• Endemic in west African children age
• Malaria acts as a co-factor with • Total of 32 permanent teeth
Ebstein–Barr virus –– four maxillary and four mandibular
• Grossly destructive lesion of mandible molars
with a large soft tissue component –– two maxillary and two mandibular
• Abdominal and ovarian involvement is canines
common –– four maxillary and four mandibular
• Responds well to chemotherapy incisors
• Usually all permanent teeth are present by
18 years of age (but this is variable)

Supernumerary teeth
Teeth • Extra teeth nearly always occur in the
Normal development maxilla
• Associated with Gardner’s syndrome and
• Total of 20 deciduous teeth
idiopathic hypoparathyroidism

Ch-06.indd 349 8/12/2010 12:19:13 PM


6.18 Neck

Anatomy Contents of the posterior triangle


• Divided into two triangles by the
sternocleidomastoid muscle (SCM) • Fat
• Level V lymph nodes
Anterior triangle • Accessory (eleventh) cranial nerve
• Borders • Dorsal scapular nerve
–– SCM posteriorly • Brachial plexus
–– Mandible superiorly • Subclavian artery and vein
–– The midline
• Further subdivided by the hyoid bone into
the suprahyoid triangle and the infrahyoid
triangle
Head and neck lymph nodes
Contents of the suprahyoid triangle Cervical lymph nodes
• Left-sided neck nodes drain into the
• Submandibular and submental nodes – thoracic duct
level I • Right-sided neck nodes drain into the
• Submandibular gland junction of the internal jugular vein and
Note: The floor is the mylohyoid muscle; the subclavian vein
structures deep to this are in the • Nodal level predicts survival in squamous
sublingual space, e.g. the sublingual cell carcinoma of the head and neck
gland –– Level I nodes have a 40% 5-year survival
rate
–– Level V nodes a 4% 5-year survival rate
Contents of the infrahyoid triangle • Standard treatment of head and neck
cancer with positive nodes is neck
dissection with removal of all level I–V
• Carotid arteries and internal jugular nodes, because metastases do not occur in
veins any set order
• Strap muscles of the neck
• Level VI lymph nodes Cervical lymph node classification
• Larynx, trachea and thyroid gland • Level I: submandibular and submental
nodes
• Level II: cervical chain nodes deep to
upper third of SCM (above level of hyoid
Posterior triangle bone on axial images)
• Borders • Level III: cervical chain nodes deep to
–– Trapezius posteriorly middle third of SCM (below hyoid and
–– SCM anteriorly above cricoid cartilage on axial images)
–– Clavicle inferiorly • Level IV: cervical chain nodes deep to
• The floor is formed by four muscles: the lower third of SCM (below level of cricoid
splenius capitis, levator scapulae, scalenus cartilage on axial images)
medius and scalenus anterior • Level V: posterior triangle nodes
• Level VI: nodes around thyroid gland
• Level VII: superior mediastinal nodes

Ch-06.indd 350 8/12/2010 12:19:13 PM


Neck 351

Features of malignant head • Peripheral blood flow pattern with colour


Doppler ultrasound
and neck nodes –– Normal nodes have a central hilar flow
• Enlargement pattern
–– Level I and II nodes are normally • Extracapsular spread
slightly larger than level III–VII nodes –– Non-sharp, ‘spiculated’ margins
–– Normal nodes are larger and more –– Absence of a clean fat plane around
conspicuous in children and young node
adults than in the elderly
–– Short axis measurements are used Ultrasound and fine-needle
aspiration cytology
Size criteria for head and neck
nodes
When used together, ultrasound and fine-
needle aspiration cytology have > 90%
• Levels I and II: > 10 mm by ultrasound, accuracy in the detection of malignant
> 12 mm by CT or MRI lymph nodes
• Levels III–VII: > 8 mm by ultrasound,
> 10 mm by CT or MRI

PET–CT in head and neck


• Rounded shape: ration of maximum short cancer
axis to maximum long axis ratio > 0.6
• PET–CT is developing an increasing role
• Loss of fatty hilum
• Can detect lesions as small as a few
–– Ultrasound shows loss of normal echo-
millimetres in diameter
bright centre
• More sensitive than CT and MRI for nodal
–– MRI shows low signal on T1WI and
metastases
T2WI
• Nodal uptake is size-dependent and is
poor for nodes < 6 mm in diameter
MRI of lymph nodes
Indications
• T1WI and STIR are best sequences • Staging of primary head and neck cancer
• Intravenous contrast not required to • Detection of locally recurrent disease after
tell nodes from vessels treatment
• Intravenous contrast improves • Detection of unknown primary with
detection of necrotic nodes positive neck nodes

Normal FDG-18 uptake in the head


• Central necrosis and neck
–– CT shows low-attenuation centre and
peripheral enhancement
• Extraocular muscles
–– MRI: T1WI post-contrast is the best
• Brain grey matter
sequence and shows peripheral
• Fossae of Rosenmüller
enhancement
• Pterygoid muscles
–– Ultrasound shows an echo-poor central
• Prevertebral muscles
cystic area
• Tonsils
–– Tuberculous adenitis can mimic
• Salivary glands
squamous cell carcinoma metastases
• Uvula
• Microcalcifications
• Tongue and oral mucosa
–– Highly suggestive of papillary thyroid
• Mylohyoid
carcinoma
• Vocal cords if talking
–– Coarse nodal calcification is seen in
• Strap muscles
treated lymphoma and tuberculosis
• Brown fat

Ch-06.indd 351 8/12/2010 12:19:14 PM


352 Central nervous system, and head and neck

Anterior triangle pathology • Caused by incomplete obliteration of the


branchial apparatus
Suprahyoid pathology • Fluid filled cyst with or without a fistulous
• Lymphadenopathy (level I and II nodes) tract
• Submandibular gland lesions • Lined by stratified squamous epithelium
• Vagus nerve paraganglioma • Typically present later in life (unlike a
• Plunging ranula cystic hygroma)
• Prone to secondary infection: internal
Infrahyoid pathology debris seen
• Lymphadenopathy (level VI nodes) • There are four types: first, second, third
• Thyroglossal duct cyst and fourth brachial cleft cysts
• Laryngocele First branchial cleft cyst
• Carotid bulb paraganglioma
• Accounts for 5% of cases
• Neurofibroma
• Fistulous tract from parotid region to
• Jugular vein thrombosis
external auditory canal
• Branchial cleft cyst
• Classically presents in middle age with
Neurofibroma recurrent parotid abscesses and otorrhoea
• Can exert pressure on the seventh cranial
• Nerve sheath tumour
nerve
• Commonest in cervical region
• Arises from spinal nerve roots, Second branchial cleft cyst
sympathetic chain and brachial plexus • Accounts for 95% of cases
• Multiple or plexiform neurofibromas are • Usually a cyst
associated with neurofibromatosis type 1 • Anteromedial to SCM (anywhere in
• Only 10% of people with a solitary anterior triangle)
neurofibroma have neurofibromatosis • Lateral to carotid arteries, and may extend
type 1 medially between them
• Well-defined, lobulated soft tissue mass • Ultrasound shows echo-free lesion
Imaging features (internal echoes seen if infected)
• CT shows low-attenuation lesion with mild
MRI rim enhancement
• Isointense on T1 to the adjacent strap • MRI shows lesion low on T1WI, high on
muscles T2WI
• High signal on T2WI with a low signal
centre Third and fourth branchial cleft
• Does not enhance cysts (very rare)
• Very rare
Jugular vein thrombosis • Fistulous tracts running from the piriform
• Associated with central venous catheters fossae to the border of SCM at the base of
and intravenous drug use neck
• Presents with a swollen neck with a firm • Third brachial cleft cyst lies adjacent to the
palpable mass in the anterior triangle laryngeal ventricle
Imaging features • Fourth brachial cleft cyst lies adjacent to
the recurrent laryngeal nerve
Ultrasound
• Early: echo-poor clot expands the vein
• Late: echo-bright clot in a contracted vein Posterior triangle pathology
CT • Lymphadenopathy (level V nodes)
• Wall enhancement of expanded vein • Cystic hygroma
• Tuberculous adenitis
Branchial cleft cyst • Lipoma
• Developmental anomaly • Haemangioma of the trapezius

Ch-06.indd 352 8/12/2010 12:19:14 PM


Neck 353

Cystic hygroma • In children mainly caused by other


mycobacteria, e.g. Mycobacterium avium-
• Developmental anomaly
intracellulare complex
• Failure of lymphatic sacs to connect with
• Presents as a slow growing, painless neck
draining veins, which results in large cystic
mass
lymphatic spaces
• Left untreated, the caseating nodes can
• 80% occur in the neck, 20% in the axilla
weaken the overlying skin leading to sinus
• 80% are present at birth and associated
formation (‘collar stud abscess’)
with Turner’s syndrome; trisomies 13, 18
• Posterior triangle is the commonest site
or 21, Noonan’s syndrome, or fetal alcohol
• Unilateral neck involvement is typical
syndrome
• Painless compressible and Imaging features
transilluminable mass in the posterior
Plain X-ray
triangle
• Chest X-ray is abnormal in only 25% of
• Extends around muscles and blood
cases
vessels, with no displacement
• 10% extend down into the mediastinum CT
• Important complications are haemorrhage • Enlarged nodes with fluid attenuation
and infection causing enlargement and centres
mass effect with tracheal or vascular • Displays rim enhancement
compression • Can mimic metastases from a squamous
cell carcinoma
Imaging features • Coarse nodal calcifications may be seen
Ultrasound after treatment
• Multiple, thin-walled, cystic spaces
• Fluid–fluid levels seen if there is associated
Lipoma
haemorrhage • Posterior triangle is a common location
• Well-defined, elliptical subcutaneous mass
CT
• Low-attenuation cystic lesion Imaging features
MRI Ultrasound
• Typically low on TIWI, high on T2WI • Echo-bright with echogenic lines
• Variable appearance if there is CT and MRI
intralesional haemorrhage • Fat-containing lesion (high on T1WI and
Tuberculous adenitis T2WI)
• In adults mainly caused by Mycobacterium
tuberculosis

Ch-06.indd 353 8/12/2010 12:19:14 PM


6.19 Thyroid gland

Anatomy Normal imaging features


• Thyroid gland is composed of two lateral • Ultrasound shows homogeneous
lobes and a midline isthmus; each lobe has echotexture, brighter than the adjacent
an upper and lower pole strap muscles
• Isthmus is positioned approximately level • CT shows high attenuation (because of the
with the cricoid cartilage iodine content) and uniform enhancement
• Each lobe lies between the trachea • MRI shows a slightly higher signal than the
medially and the CCA and internal jugular adjacent strap muscles on both T1WI and
vein laterally T2WI with uniform enhancement
• Normal craniocaudal length of each lobe
is < 5 cm
• Normal anteroposterior diameter is < 2 cm
Thyroid scintigraphy
• Posterior to each lobe is the longus coli Agents
muscle • Iodine-123: slightly superior to
• 50% of people have an additional technetium-99m-pertechnetate in all
pyramidal lobe, which extends superiorly aspects of thyroid imaging, but more
from the isthmus expensive and requires longer imaging
• Arterial supply times
–– Inferior thyroid artery: branch of the • Iodine-131: principally used for
thyrocervical trunk assessment and treatment of thyroid
–– Superior thyroid artery: first branch of cancer metastases; it delivers a much
ECA higher radiation dose than I-123
–– 5% of people also have a thyroid ima • Technetium-99m-pertechnetate: most
artery, a branch of the aorta or the widely used thyroid imaging agent in the
brachiocephalic trunk UK. Can be performed without the patient
• Venous drainage having to stop thyroxine or carbimazole,
–– Via paired superior and middle thyroid but poor assessment of retrosternal thyroid
veins to the internal jugular tissue (owing to sternal attenuation)
–– Via multiple inferior thyroid veins to the
brachiocephalic veins Technetium-99m-
pertechnetate
Physiology • Method
–– Given by intravenous injection
• Iodine in food is converted to iodide in the
–– Imaged at 20 minutes
intestine and enters the circulation
–– Pin-hole gamma camera is ideal
• One quarter of the iodine is ‘trapped’
• Pertechnetate is trapped by thyroid gland
by the follicular cells of the thyroid and
but not organified into thyroglobulin and
organified into thyroglobulin, from which
rapidly excreted by kidneys
tri-iodothyronine (T3) and thyroxine (T4)
• Normally there is uniform uptake of
are produced
pertechnetate throughout both lobes of
• Three quarters of the iodine is excreted by
the thyroid
the kidneys
• Uptake also occurs in the salivary glands
• The release of T3 and T4 is controlled
(patient is given a drink prior to imaging),
via TSH as part of a negative feedback
the gastric mucosa and the choroid plexus
mechanism with the hypothalamus and
pituitary gland

Ch-06.indd 354 8/12/2010 12:19:14 PM


Thyroid gland 355

Causes of diffuse high uptake Pathology


Thyroglossal duct cyst
• Graves’ disease
• Early Hashimoto’s thyroiditis Embryology of thyroid gland
• Iodine deficiency
• Stopping antithyroid drugs (rebound
phenomenon) • Arises from the tongue base (foramen
• TSHoma cecum)
• Lithium treatment • Descends in the midline with
• Hydatidiform mole or choriocarcinoma thyroglossal duct
• Passes anterior to the hyoid bone
• Reaches its final position by seventh
week of gestation
Causes of diffuse low uptake

• Subacute thyroiditis (de Quervain’s • Commonest congenital midline neck mass


thyroiditis) • Arises from remnants of thyroglossal duct
• Established Hashimoto‘s thyroiditis • 80% are located in the midline, 20% are
• Recent intravenous iodinated contrast, paramedian
e.g. for an intravenous urogram • Present in young adults as a painless neck
• Amiodarone treatment swelling
• Thyroxine replacement therapy • May become secondarily infected
• Anti-thyroid drugs, e.g. carbimazole • Cyst moves upwards with swallowing and
• Riedel’s thyroiditis tongue protrusion
• Congenital hypothyroidism • 1% risk of developing thyroid carcinoma
• Strumi ovari (majority are papillary)
• Thyroidectomy • Even smaller risk of developing squamous
cell carcinoma
• < 5% contain functioning ectopic thyroid
• Hot nodule: a focal area of increased
tissue
uptake, which indicates that a lesion is
• Mainly a clinical diagnosis; imaging is
hyperfunctioning. The majority are caused
used to confirm diagnosis and assess
by a secreting adenoma, and are almost
extent of lesion prior to surgical removal
never malignant (< 1% of cases)
• Surgical procedure is the Sistrunk
• Cold nodule: a focal area of decreased
operation, whereby the cyst is resected
uptake, which indicates that a lesion is
along with the entire thyroglossal duct
hypofunctioning
(including central portion of hyoid bone)
–– 15% will be malignant if solitary
• Removal of the cyst alone is associated
–– 5% will be malignant if multiple
with a high rate of recurrence
• Preoperative technetium-99m-
Causes of a cold nodule pertechnetate or iodine-123 scans are
sometimes performed to confirm the
• Colloid cyst presence of a normal thyroid gland before
• Non-secreting adenoma resection, i.e. to check that ectopic thyroid
• Focal area of thyroiditis tissue in the cyst wall is not the patient’s
• Granuloma only thyroid tissue
• Abscess
• Parathyroid adenoma Imaging features
• Thyroid carcinoma Ultrasound
• Thyroid metastases • Well-defined, cystic lesion embedded
within the strap muscles

Ch-06.indd 355 8/12/2010 12:19:14 PM


356 Central nervous system, and head and neck

• Majority contain internal echoes as a result • 5% get an initial transient thyrotoxic state
of proteinaceous debris (‘hashitoxicosis’)
CT • Up to one third of patients will develop
• Fluid-attenuation lesion with a thin non-Hodgkin’s lymphoma
enhancing rim Imaging features
MRI Ultrasound
• Typically low T1 high T2, but variable • Diffusely enlarged with a coarse
appearance depending on protein content echotexture
• Echo-poor micronodules
Graves’ disease
• Autoimmune disorder caused by long- Scintigraphy
acting thyroid-stimulating (LATS) • Non-specific appearances
antibodies • Increased uptake in early stage
• Predominantly affects young females • Reduced uptake in established disease
• Presents with smooth, painless goitre Subacute thyroiditis
• Raised T4 and low TSH
(de Quervain’s thyroiditis)
Imaging features • Typically occurs 2 weeks after a viral
Ultrasound respiratory tract infection
• Diffusely enlarged and echo-poor • Inflamed gland releases thyroxine with
• Diffusely hypervascular ‘thyroid inferno’ transient hyperthyroidism
• Vessels have high flow velocities (> 50 cm/ • Causes fever and painful goitre
second) with colour Doppler scans • Majority return to a euthyroid state over
• Velocities return to normal (< 30 cm/ several months
second) after treatment Imaging features
Scintigraphy Ultrasound
• Diffusely increased uptake throughout • Mildly enlarged and echo-poor thyroid
both lobes and the isthmus • Absent colour flow
• Hypertrophied pyramidal lobe
Scintigraphy
Complications • Diffusely decreased uptake
• Thyroid acropachy
–– Usually occurs in first year following Amiodarone-induced
treatment thyroiditis
–– Painless swelling of wrists or hands with • Amiodarone is an iodine-containing anti-
finger clubbing arrhythmic drug with a long half-life of
–– Asymmetrical, thick periosteal reaction 90 days
along metacarpals and phalanges • 10% of patients taking it develop problems
• Graves’ ophthalmopathy with thyroid function
• Causes a destructive thyroiditis or a
Hashimoto’s thyroiditis Graves’-like condition
• Commonest cause of hypothyroidism in
developed countries Multinodular goitre
• Autoimmune disease caused by anti- • Benign nodular (adenomatous)
thyroid antibodies hyperplasia
• Predominantly affects middle-aged • Typically occurs in middle-aged females
females • Causes a swollen neck with palpable
• Diffuse lymphocytic infiltration of thyroid nodules
• Gland is enlarged and non-tender and • Retrosternal extension can cause tracheal
feels ‘rubbery’ to palpation compression

Ch-06.indd 356 8/12/2010 12:19:14 PM


Thyroid gland 357

• Thyroid function is usually normal Scintigraphy


• Occasionally a nodules is hypersecreting • Diffuse low uptake
(Plummer’s disease)
Lingual thyroid
Imaging features • Accounts for 90% of ectopic thyroid tissue
Ultrasound (the mediastinum is another recognised
• Asymmetrically enlarged gland ectopic site)
(anteroposterior diameter of lobe > 2 cm) • Caused by arrest of normal descent from
• Contains multiple nodules of varying sizes the foramen cecum
• Nodules can be purely solid, purely cystic • More common in females
or mixed • 2% risk of developing thyroid carcinoma
Scintigraphy Imaging features
• Heterogeneous uptake by an enlarged
gland CT
• May see discrete hot nodules on a • High-attenuation, enhancing lesion at the
background cold parenchyma base of the tongue
• A solitary cold nodule has a 5% risk of Thyroid nodule
being malignant
• Assessed by a combination of ultrasound,
Congenital hypothyroidism scintigraphy and fine-needle aspiration
• Diagnosed at birth via heel prick test cytology
• Diagnosis confirmed via scintigraphy, Imaging features
which shows absent uptake
Ultrasound
Riedel’s thyroiditis • Features suggestive of a benign nodule:
• Rare idiopathic chronic inflammatory –– Purely cystic lesion
disorder –– Cystic lesion with ‘comet tail’ artefacts
• Dense fibrous tissue replaces normal –– Thin uniform echo-poor halo
thyroid parenchyma –– Peripheral coarse calcifications
• Associated with retroperitoneal and –– Perilesional blood flow
mediastinal fibrosis • Features suggestive of a malignant nodule:
• Fibrosis extends beyond thyroid capsule –– Microcalcifications
into adjacent structures –– Irregular margins
• Can cause stridor or dysphagia from –– Irregular halo around lesion
compressive effects –– Invasion of local structures
• Hard, fixed and painless goiter –– Intralesional blood flow
• One third of patients are hypothyroid –– Lymphadenopathy
• Fine-needle aspiration biopsy cannot Scintigraphy
distinguish Riedel’s thyroiditis from • 15% risk of malignancy if a solitary cold
anaplastic carcinoma nodule
• 5% risk of malignancy if multiple cold
Imaging features nodules
Ultrasound Fine-needle aspiration cytology
• Enlarged, echo-poor gland with ill-defined
• Ultrasound and scintigraphy cannot
margins
reliably distinguish benign from malignant
CT nodules
• Extensive areas of low attenuation • When combined with fine-needle
throughout gland aspiration cytology the overall accuracy is
MRI around 90%
• Low signal on T1WI and T2WI • However, cytology cannot distinguish
follicular adenoma from carcinoma

Ch-06.indd 357 8/12/2010 12:19:15 PM


358 Central nervous system, and head and neck

Thyroid malignancy Multiple endocrine neoplasia


• Head and neck irradiation is the major risk type 2a (MEN-2a)
factor (latency > 5 years)
• Medullary carcinoma thyroid (in 100%)
Papillary carcinoma • Phaeochromocytoma (in < 50%)
• Accounts for 70% of thyroid malignancies • Parathyroid adenoma or hyperplasia (in
• Low-grade malignancy usually affecting < 50%)
young adults
• Spreads early via lymphatics to adjacent
neck nodes
• Irregular echo-poor mass containing
Anaplastic carcinoma
microcalcifications • Accounts for 3% of thyroid malignancies
• Nodes contain punctuate • Very aggressive infiltrative tumour
microcalcifications affecting elderly patients
• Lymph node metastases have no bearing • Invades into adjacent muscles and vessels
on overall prognosis • Necrotic tumour often containing foci of
• Prognosis is generally excellent calcification
• Usually inoperable at time of diagnosis
Follicular carcinoma • Treatment is with radiotherapy
• Accounts for 20% of thyroid malignancies • Mean survival is 6 months
• Affects middle-aged females
• Intermediate-grade malignancy
Lymphoma
• Spreads via bloodstream to lungs and bone • Accounts for 2% of thyroid malignancies
• Fine-needle aspiration biopsy cannot • Non-Hodgkin’s B-cell type tumour
distinguish follicular adenoma from • Affects middle-aged females with
follicular carcinoma coexistent Hashimoto‘s thyroiditis
• Up to one third of patients with
Medullary carcinoma Hashimoto‘s thyroiditis develop it
• Accounts for 5% of thyroid malignancies • Usually a solitary echo-poor lesion
• Associated with multiple endocrine
neoplasia type 2a (MEN-2a) and occurs in
Metastases to thyroid
the teenage years • Rare
• Arises from C cells in the upper two thirds • Usually occurs in the context of
of the gland disseminated malignancy
• Secretes calcitonin but blood calcium • Commonest primary lesion is melanoma
levels are not affected • Usually a solitary echo-poor lesion
• Early spread to lymph nodes, lung and
bone
Treatment and follow-up
• Echo-poor lesion containing calcifications • Primary treatment is thyroidectomy with
• Nodes are hypoechoic with dense or without neck dissection
calcifications • T3 (short-acting) is taken for 4 weeks
• Poor prognosis if there are metastases at • T3 is stopped for 2 weeks (to elevate TSH
time of diagnosis level)
• Whole body iodine-131 scan is performed
Multiple endocrine neoplasia to assess for metastases
type 1 (MEN-1) • False-negative scans are seen in 20%
(caused by non-functioning metastases)
• If serum thyroglobulin is raised and
• Pituitary adenoma
iodine-131 scan is negative, an F-18-DG
• Pancreatic islet cell carcinoma
PET scan is indicated
• Parathyroid adenoma or hyperplasia
• PET is very sensitive for these non-
functioning metastases

Ch-06.indd 358 8/12/2010 12:19:15 PM


Thyroid gland 359

• High doses of iodine-131 are used to treat • Increased PTH and increased serum
metastases calcium
• Technetium-99m-pertechnetate is not • Complications include renal calculi,
suitable for whole-body scans because it is pancreatitis, nephrocalcinosis,
masked by background activity chondrocalcinosis and subperiosteal
• Iodine-131 is contraindicated in pregnancy resorptions
and breast-feeding because it crosses the • Treatment is surgical removal of the
placenta and is excreted in breast milk adenoma
• Iodine-131 is more accurate for
assessing thyroid bone metastases than
Preoperative adenoma localization
conventional bone scans investigations
Ultrasound
Medullary carcinoma • Adenomas most commonly arise from the
inferior glands
Does not accumulate I-131, amd the • Well-defined, oblong-shaped, echo-poor
following can be used: lesion
• Technetium-99m(V) DMSA scan • Usually > 1 cm in diameter, and
• Iodine-131-MIBI scan hypervascular
• Thallium-201 scan • Parathyroid carcinoma can have identical
• F-18-DG PET scan imaging features
• 20% are ectopic and not visible with
ultrasound
• Ectopic sites include thymus, mediastinum
and carotid sheath
Parathyroid glands • False positives may be caused by the
Anatomy and physiology longus coli muscle or a lymph node
• Typically there are four parathyroid glands Scintigraphy
• Situated posterior to the thyroid and • Technetium-99m-sestamibi (MIBI) scan
anterior to longus coli muscle • Dual-phase study: intravenous injection
• The two superior glands are deep to the with imaging at 20 minutes and re-imaging
midpart of each lobe at 2 hours
• The two inferior glands are deep to the • Scan covers cervical and thoracic regions
lower pole of each lobe (ectopic adenomas)
• Up to 25% of people have more than four • MIBI washes quickly out off thyroid but is
parathyroid glands retained in abnormal parathyroids
• Normal glands are not visible by
ultrasound Old method: subtraction study
• Parathyroid hormone (PTH) is secreted by
the chief cells of these glands in response • Technetium-99m-pertechnetate given
to low serum calcium levels first
• PTH acts to elevate calcium levels by • Taken up only by thyroid and washes
increasing osteoclastic bone resorption out quickly
• Thallium-201 given second
Primary hyperparathyroidism • Taken up by thyroid and parathyroid
• 85% of cases caused by a parathyroid glands
adenoma • Images are ‘subtracted’ to view the
• Rarer causes include parathyroid parathyroid glands
hyperplasia, ectopic PTH secretion • Major limitation was that a thyroid
(e.g. from squamous cell lung cancer), nodule could mimic a parathyroid
parathyroid carcinoma adenoma

Ch-06.indd 359 8/12/2010 12:19:15 PM


360 Central nervous system, and head and neck

Secondary • Tetany and convulsions


• Most commonly due to surgical removal of
hyperparathyroidism all four glands
• Increased PTH and increased serum • Rarely autoimmune
calcium
• Usually due to chronic renal failure (lack of Pseudohypoparathyroidism
vitamin D activation) • Increased PTH and increased serum
• Compensatory hyperplasia of all four calcium
parathyroid glands • Caused by end organ resistance to PTH
• Clinical features include short stature,
Tertiary hyperparathyroidism short fourth metacarpals, moon face and
• Increased PTH and increased serum intellectual impairment
calcium
• Long-standing hyperplasia develops into Pseudopseudohypoparathyroidism
autonomous secretion • Normal biochemistry
• Phenotypically the same as
Hypoparathyroidism pseudohypoparathyroidism
• Increased PTH and increased serum
calcium

Ch-06.indd 360 8/12/2010 12:19:15 PM


6.20 Miscellaneous topics

Reversible posterior • TORCH infections


• Cockayne syndrome (demyelinating
leukoencephalopathy disease of childhood)
• Brain capillary leak syndrome with • Down’s syndrome
widespread cerebral oedema • Tuberous sclerosis
• Failure of autoregulation following acute • Mitochondrial diseases
blood pressure elevation • Toxins (carbon monoxide, lead)
• Misnomer as anterior changes are well
recognised Neurocysticercosis
• Causes headaches and cortical visual loss
• Progresses to infarction if blood pressure • Caused by ingestion of pork tapeworm
not promptly controlled larvae
• Faecal–oral spread
Imaging features • Endemic in developing countries
MRI • Commonly involves the CNS causing
• Bilateral confluent areas of white matter meningoencephalitis
high T2 signal • Parenchymal calcified cystic lesions with
• Commonly affects centrum semiovale of homogeneous or rim enhancement
occipital lobes • Intraventricular cysts with hydrocephalus
• Changes typically resolve with treatment
over 1–2 weeks High T2 signal thalamic
• Appears dark on DWI (increased diffusion)
lesions
Basal ganglia •

Wernicke’s encephalopathy
New-variant Creutzfeldt-Jakob disease
Anatomy • Wilson’s disease
• Basal ganglia comprise: • Acute disseminated encephalomyelitis
–– Caudate nucleus (40% have thalamic involvement)
–– Lentiform nucleus (globus pallidus, • Lacunar infarcts
putamen) • Lead poisoning
–– Claustra • Hallervorden–Spatz disease
–– Amygdaloid body • Reye’s syndrome
• Leigh’s disease
Low-attenuation lesions in the • Biliary encephalopathy
basal ganglia
Wilson’s disease
• Hypoxia
• Hypoglycaemia
• Wilson’s disease • Autosomal-recessive disorder
• Toxins – carbon monoxide, methanol, • Low levels of caeruloplasmin
cyanide, barbiturates • Copper deposition in tissues
• Eyes show Kayser–Fleisher ring
Basal ganglia calcifications • Cirrhosis
• Idiopathic (50%) • Basal ganglia show low T1, high T2
• Hyperparathyroidism signal
• Hypoparathyroidism, • Copper has only weak paramagnetic
pseudohypoparathyroidism, effects and therefore appears as low
pseudopseudohypo­para­thyroidism signal on T1WI

Ch-06.indd 361 8/12/2010 12:19:15 PM


362 Central nervous system, and head and neck

Normally enhancing Hair-on-end skull


structures • Hereditary anaemias
–– Thalassaemia
Enhancing structures on both –– Glucose-6-phosphate dehydrogenase
CT and MRI deficiency
• Pituitary gland (anterior and posterior –– Spherocytosis
lobes) –– Sickle cell disease
• Infundibulum • Neuroblastoma metastases
• Nasal turbinates
• Sinonasal mucosa
• Choroid plexus
Epilepsy
• Pineal gland • Idiopathic, or caused by a structural brain
• Cavernous sinuses lesion acting as a focus
• Extraconal eye muscles • Identification of focus via
electroencepholography, MRI or PET
Enhancing structures on CT scanning
• Arterial structures, e.g. circle of Willis • Commonest structural lesion is
• Dural venous sinuses and deep veins hippocampal sclerosis

Enhancing structures on MRI Hippocampal sclerosis


• Thin short linear segments of dural • Highly epileptogenic abnormality
enhancement • Associated with temporal lobe epilepsy
• 80% can be cured with an anterior
Normally calcified temporal lobectomy

structures Imaging features


• Choroids plexus MRI
• Basal ganglia • Hippocampal atrophy and enlarged
• Pineal gland temporal horn on T1WI
• Dura • High signal in atrophic hippocampus on
• Habenula T2WI
• Falx cerebri and cerebellum F-18-DG PET
• Tentorium • Decreased uptake in epileptogenic
• Clinoid ligaments temporal lobe

Skull vault thickening Other structural lesions that can


cause epilepsy
Generalised thickening
• Severe anaemias • Stroke: commonest cause in those
• Acromegaly aged > 50 years
• Hyperparathyroidism • Cavernous angioma
• Osteopetrosis • Primary or secondary brain tumours
• Shunted hydrocephalus • Arteriovenous malformation
• Post infectious, e.g. neurocysticerosis
Focal thickening • Phakomatoses
• Paget’s disease • Neuronal migration anomalies, e.g.
• Meningioma heterotopias (grey matter in abnormal
• Fibrous dysplasia location), pachygyria (paucity of gyri),
• Hyperostosis frontalis agyria (absent gyri), schizencephaly
(CSF-filled transcerebral cleft)

Ch-06.indd 362 8/12/2010 12:19:15 PM


Miscellaneous topics 363

Dementia Normal MRI findings in the


Causes ageing brain
• Multiple patchy T2 white-matter
• Alzheimer’s disease (60% of cases)
hyperintensities
• Multi-infarct dementia (20% of cases)
• Enlargement of sulci and ventricles
• Other causes (20% of cases): Lewy bodies,
• Triangle-shaped frontal horn ‘caps’, best
Pick’s disease, Parkinson’s disease,
seen on T2WI
Korsakoff’s psychosis, Creutzfeldt-Jakob
• Thin, smooth periventricular ‘rims’, best
disease, depressive dementia
seen on T2WI
• Iron deposition in globus pallidus or
Reversible causes of dementia putamen: low signal on T2WI

• Normal pressure hydrocephalus


• Meningioma
Trigeminal nerve (fifth
• Hypothyroidism cranial nerve)
• Supplies sensation to the face, the corneal
reflex and the muscles of mastication
Alzheimer’s disease • Arises from ventral aspect of the pons
• Passes into the middle cranial fossa
• Slowly progressive loss of memory and
through Meckel‘s cave
cognition
• Forms its ganglion and divides into three
Imaging features branches, the ophthalmic nerve, the
maxillary nerve and the mandibular nerve.
CT and MRI
• Normal in early stages of disease Ophthalmic nerve
• Later prominent sulci and atrophy of
• Runs in the lateral wall of the cavernous
medial temporal lobe
sinus (below the trochlear and oculomotor
PET and HMPAO-SPECT nerves and above the maxillary nerve)
• Symmetric perfusion defects in posterior before exiting through the superior orbital
temporal and parietal lobes fissure into the orbit
• Here it divides into lacrimal, frontal and
Pick’s disease nasociliary branches
• Pre-senile onset (< 65 years)
• Enlarged frontal horns Maxillary nerve
• Frontotemporal atrophy and • Runs in the lateral wall of the cavernous
hypoperfusion sinus (being the most inferior nerve
here) before exiting through the foramen
New-variant Creutzfeldt-Jakob rotundum into the pterygopalatine fossa
disease • Its main branch is the infraorbital nerve,
• Rapidly progressive dementia and which enters the orbit through the inferior
myoclonus orbital fissure and exits through the
• Occurs in young adults infraorbital foramen
• Caused by a prion protein
Mandibular nerve
Imaging features • Exits the skull before the cavernous sinus
MRI through the foramen ovale to reach the
• Symmetrical high T2 signal in posterior masticator space
thalami (pulvinar nuclei)

Ch-06.indd 363 8/12/2010 12:19:15 PM


364 Central nervous system, and head and neck

Cerebral sulci Germinoma


• Deep grooves that separate the cerebral • 80% occur in the pineal gland
lobes • Commonest pineal tumour)
• Pia mater dips into all the sulci • 20% occur in the suprasellar region
• Only the lateral sulcus has an arachnoid • Malignant germ-cell tumour
mater lining • Causes precocious puberty and Parinaud’s
syndrome (upward gaze palsy)
Lateral sulcus (Sylvian fissure) • Causes hydrocephalus as a result of
• Separates the frontal and temporal lobes aqueductal obstruction
• Contains branches of the middle cerebral
artery Imaging features
CT
Central sulcus of Rolando • Infiltrating calcified soft tissue mass
• Runs upwards from the lateral sulcus • CSF seeding (‘drop’ metastases)
• Separates the frontal and parietal lobes
• Separates the precentral motor gyrus from Causes of vertebral
postcentral sensory gyrus ossifications
• Paravertebral ossification
Parieto-occipital sulcus –– Diffuse idiopathic skeletal hyperostosis
• Separates the parietal and occipital lobes –– Reiter’s syndrome
–– Psoriasis
Calcarine sulcus • Syndesmophytes
• Separates the temporal and occipital lobes –– Ankylosing spondylitis
–– Ochronosis (alkaptonuria)

Ch-06.indd 364 8/12/2010 12:19:15 PM


Bibliography
Index

Books
Adam A, Dixon A, Grainger RG, Allison DJ (eds). Grainger & Allison’s Diagnostic Radiology, 5th edn.
Edinburgh: Churchill Livingstone, 2009.
Sutton D, Reznek R, Murfit J. Textbook of Radiology and Imaging, 7th edn. Edinburgh: Churchill
Livingstone, 2002.
Haaga JR, Lanzieri CF. CT and MR Imaging of the Whole Body, 4th edn. St Louis: Mosby.
Hansell DM, Armstrong P, Lynch DA, Page McAdams H. Imaging of the Diseases of the Chest, 4th
edn. Philadelphia: Mosby, 2004.
Webb R, Muller NL, Naidich DP. High Resolution CT of the Lung, 4th edn. Philadelphia: Lippincott
Williams & Wilkins, 2008.
Dahnert W. Radiology Review Manual, 6th revised edn. Baltimore: Lippincott Williams & Wilkins,
2007.
Helms CA. Fundamentals of Skeletal Radiology, 3rd edn. London: Elsevier, 2005.
Weissleder R, Wittenberg J, Harisinghani MMGH, Chen JW, Jones SE, Patti JW. Primer of Diagnostic
Imaging, 4th edn. Philadelphia: Mosby, 2007.
Rogers LF. Radiology of Skeletal Trauma. New York: Churchill Livingstone, 2001.
Manaster BJ, May DA, Disler DG. Musculoskeletal Imaging: The Requisites, 3rd edn. Philadephia:
Mosby, 2006
Blickman G, Parker BR, Barnes PD Paediatric Radiology: The Requisites, 3rd edn. Philadelphia:
Mosby, 2009.
Zagoria RJ. Genitourinary Radiology: The Requisites, 3rd edn. Philadelphia: Mosby, 2004 .
Kopans DB. Breast Imaging, 3rd edn. Baltimore: Lippincott Williams & Wilkins Ltd, 2007.
Davies SG (ed.). Chapman & Nakielny: Aids To Radiological Differential Diagnosis, 5th edn.
Edinburgh: Saunders, 2009.

Journals
American Journal of Neuroradiology: www.ajnr.org
American Journal of Roentgenology: www.ajronline.org
Radiographics: http://radiographics.rsna.org
Clinical Radiology: www.rcr.ac.uk, www.sciencedirect.com

On-line resources
e-medicine http://emedicine.medscape.com
Wheeless’ Textbook of Orthopaedics. www.wheelessonline.com
www.radiologyassistant.nl

Biliography.indd 365 8/10/2010 11:17:48 AM


Index
Index

A Amiodarone-induced
Abdominal lung disease 27
aorta 60 thyroiditis 356
aortic aneurysm 64 Amniotic fluid embolism 45
Aberrant right subclavian artery 265 Amoebic liver abscess 137
Absent pedicle 324 Amyloidosis 29, 87, 96
ACA infarction 291 Anaplastic carcinoma 358
Acalculous cholecystitis 145 Aneurysm rupture 305
Accelerated Aneurysmal bone cyst 118
bone maturation 126 Angiomyolipoma 195
skeletal maturity 271 Ankle joint and foot 93
Accessory bones 90 Ankylosing spondylitis 94, 322
Acetabular fracture 109 Annular pancreas 148, 243
Achalasia 157 Anorectal malformation 245
Achilles tendon tear 114 Anterior
Achondroplasia 123, 271 cruciate ligament 111
Acoustic schwannoma 329 ethmoidal artery 331
Acromioclavicular joint injury grades 127 junctional line 16
Acro-osteolysis of terminal phalanx 126 mediastinum 54
Adenocarcinoma 2, 175 talofibular ligament tear 114
Adenoid cystic carcinoma 13, 338 triangle 350, 352
Adenolymphoma 337 Antrochoanal polyp 333
Adenomyosis 220 Aortic
Adrenal arch anomalies 61, 265
adenomas 212 arch branching patterns, left 60
carcinoma 213 dissection 62
collision tumour 215 Aortoenteric fistula 64
cortical failure, primary, causes of 215 Apical cyst 347
cyst 213 Aplasia 9
gland 212 Appendicitis, acute 244
haemorrhage 213 Arachnoid cyst 283
hypofunction 215 Arachnoiditis 320
lymphoma 214 ARDS 18
metastasis 213 Arnold-Chiari malformation 239, 240, 281
myelolipoma 212 Arrhythmogenic right ventricular dysplasia 80
Agenesis of corpus callosum 236, 282 Arterial supply 225
AIDS in children 261 Arteriovenous
AIDS-related fistula 318
CNS infections 287 malformation 8, 306, 318
lymphoma 40 Arthritides and connective tissue diseases 94
Air space Asbestos-related lung disease 31
diseases 18 Aspergilloma 35
parenchymal disease 24 Aspergillosis 35, 39
Airways disease 11 Aspiration pneumonia 35
Alcohol and CNS 309 Associations of slipped femoral epiphysis 129
Allergic bronchopulmonary aspergillosis 36 Assessing initial response to therapy 6
Alveolar Asthma 13
microlithiasis 29 Astrocytoma 274, 325
proteinosis 20 Atherosclerotic ICA stenosis 75
Alzheimer’s disease 363 Atlantoaxial subluxation 322
Ambiguous genitalia 252 Atrial enlargement, left 79
Ameloblastoma 348 Atropine 70

Index.indd 367 8/12/2010 12:19:28 PM


368 Index

Atypical pneumonias 34 Brenner tumour 222


Avascular necrosis of femoral head 124 Bright blood 86
Avulsion fractures of pelvis 110 Bronchial artery embolisation 74
Axial compression 105 Bronchiectasis 14
Azygo-oesophageal line 16 Bronchiolitis obliterans 15
Azygous continuation of IVC 59 Bronchitis, chronic 13
Axonal injury, diffuse 302 Bronchoalveolar cell carcinoma 3
Bronchogenic
B carcinoma 32
Baker’s cyst 124 cyst 56, 257
Barium swallow 162 Bronchospasm 69
Barrett’s oesophagus 162 Bucket-handle fracture 109
Basal ganglia 361 Budd–Chiari syndrome 141
Basilar artery thrombosis 292 Buerger’s disease 71
Behçet’s disease 27, 164
Benign mucinous cystadenoma 222 C
Benign prostatic hyperplasia 210 Caffey’s disease 272
Benign salivary gland tumours 337 Calcarine sulcus 364
Benign serous cystadenoma 222 Calcified metastases 5, 274
Benign thymoma 55 Candida oesophagitis 163
Benign tumours of Capillary haemangioma 314
bone 115 Caplan’s syndrome 26
larynx 344 Carcinoid tumour 8, 174
pharynx 340 Cardiac
Berry aneurysms 305 chamber enlargement 79
Berylliosis 31 CT dose reduction 88
Bile duct imaging 78
anatomy 134 MRI 85
variants 134 myxoma 80
Biliary atresia 246 tamponade 84
Black blood 86 Cardiogenic pulmonary oedema 18
Blount’s disease 271 Cardiomyopathy 80, 87
Blunt trauma to bowel 171 Carney’s complex 155
Blunt traumatic aortic injury 52 Carotid
Boerhaave’s syndrome 158 artery imaging 294
Bone 128 bulb paraganglioma 342
lymphoma 119 cavernous sinus fistula 316
sarcoid 97 dissection 76, 292
ossicles 327 Doppler ultrasound 75
Bowed long bones in children 128 imaging 75
Bowel obstruction, causes 178 stenting 76, 295
Brachial plexus 303 Carpal
Brain injuries 106
abscess 286 tunnel 91
and spinal trauma 300 Castleman’s disease 6
herniations 303 Catheter angiography 76, 295, 306
tumours, adult 274 Cavernous
Brainstem glioma 238, 280 angioma 307
Branchial cleft cyst 352 haemangioma 314
Breast 225 Cavitating metastases 5
abscess 229 Cement disease 123
biopsy 232 Central
calcifications 225 canal of spinal cord 318
cancer, mimics on mammography 232 pontine myelinolysis 309
cysts 226 sulcus of Rolando 364
MRI 226 venous pressure line 48

Index.indd 368 8/12/2010 12:19:28 PM


Index 369

Centrilobular nodules 22 Coeliac disease 175


Cerebellar haemangioblastoma 277 Colloid or mucinous carcinoma 231
Cerebellopontine angle lesions 328 Colonic
Cerebral aphthoid ulceration, causes 183
and cerebellar atrophy 309 polyps 181
angiography in subarachnoid haemorrhage 306 strictures, causes 183
contusion 302 Colonic polyps 181
haemorrhage, primary 290, 292 Colorectal carcinoma 182
sulci 364 Common
Cervical primary tumours 274, 275
carcinoma 221 sites of mandibular fractures 128
lymph nodes 350 Community-acquired pneumonias 34
spine 105, 268 Congenital
spine fractures 304 absence of pericardium and pericardial sac
Charcot’s joint 96 defect 83
Chest anomalies 216
in drowning 260 anomalies of aortic arch 60
trauma 50 bronchial atresia 258
Childhood cholesteatoma 328
chest conditions 253, 260 CNS disorders 281
deafness 330 cystic adenomatoid malformation 254
osteomyelitis 269 diaphragmatic hernia 255
pneumonia 260 heart disease 81
tuberculosis 260 hypothyroidism 357
Choanal atresia 257 lobar emphysema 255
Cholangiocarcinoma 145 lung disease 7, 9
Cholangiolithiasis 144 oesophageal atresia 156
Cholangitis 146 scoliosis 271
Cholecystitis syringomyelia 285
acute 145 vertical talus 271
chronic 145 Conn’s syndrome 214
emphysematous 145 Connective tissue diseases 25
Choledochal cyst 144, 245 Constrictive pericarditis 84
Cholelithiasis 144 Contrast
Cholesteatoma 328 medium extravasation 70
Chondroblastoma 118 reactions and treatment 69
Chondromyxoid fibroma 118 Contusion patterns in knee trauma 112
Chondrosarcoma 119 Conventional technique 16
Chordoma 326 Cord
Chordoma of degeneration, subacute combined 320
clivus 326 haemorrhage 303
sacrum 326 oedema 303
Choriocarcinoma 217 transaction 303
Choroid plexus cyst 235 Coronal views 234
Choroidal haemangioma 314 Coronary
Churg–Strauss syndrome 27 artery anomalies 79
Chylothorax 50 CT angiography 87
Circle of Willis 289 Corpus callosum anomalies 281
Cirrhosis 135 Corrosive gastritis 167
Clay Shoveller’s fracture 304 Cortical necrosis, acute 191
Clear cell carcinoma 222 Coxa
Club foot 271 valga 272
CNS vara 272
infections 286 Craniofacial fibrous dysplasia 333
lymphoma 275 Craniopharyngioma 298
Coarctation of aorta 61, 266 Craniosynostosis 235
Cochlear promontory paraganglioma 343 Crazy-Paving pattern 22

Index.indd 369 8/12/2010 12:19:28 PM


370 Index

Creutzfeldt-Jakob disease 363 Dense metaphyseal bands 128


Cricoarytenoid joint dislocation 346 Dentigerous cyst 348
Cricoid and thyroid fractures 346 Depressed fracture 300
Criteria for diagnosing ICA stenosis 76, 294 Dermoid 315
Crohn’s disease 183 Developmental dysplasia of hip 270
Cross sectional anatomy 318 Diagnostic angiography 66
Croup 259 Diaphragm 51
Cryptococcoma 288 rupture 52
Cryptococcus neoformans infection 288 Diastatic fracture 300
Cryptogenic Diastematomyelia 284
fibrosing alveolitis 25 DiGeorge’s syndrome 261
organising pneumonia 20 Digital subtraction angiography 66
Cryptorchidism 252 Dilated cardiomyopathy 80
CT Diploic space metastases 277
angiography 76, 306 Disc
guided lung biopsy 4 bulge 321
in temporal bone imaging 327 herniation 321
pitfalls in aortic dissection 63 protrusion 321
pulmonary angiography (CTPA) 43 Discitis 321
signs of AAA rupture 64 Discoid meniscus 111
staging 174 Dislocation 123
venography 44 Disorders of sexual developmental 252
CTPA, pitfalls 43 Displaced tears 110
Cushing’s syndrome 214 Diverticular disease 181
Cyanosis Diverticulitis 181
with pulmonary plethora 264 Doppler ultrasound of carotids 294
without pulmonary plethora 263 Double bubble sign 247
Cylindroma 13, 338 Down’s syndrome (trisomy 21) 270
Cystic Drug-induced lung disease 27
adenoid carcinoma 341 Duct ectasia 229
adenomatoid malformation 9 Ductal carcinoma 229, 230
carcinomas of pancreas 152 Duodenal
duct variants 134 atresia or stenosis 243
fibrosis 10, 247, 260 carcinoid 175
hygroma 353 diverticulum 172
jaw lesions 348 folds, thickened, causes 173
kidney diseases, acquired 189 ulcer 168
lesions of mandible 347 Duodenum 172
Cystitis 204 dilated, causes 173
Cystosarcoma phyllodes 227 Duplex kidneys 249
Cytomegalovirus Duplication cyst 164, 242
encephalitis 288 Dysgerminoma 223
oesophagitis 164
E
D Ebstein’s anomaly 81, 263
Dandy-Walker malformation 240, 281 ECG-gated dose modulation 88
De Quervain’s thyroiditis 356 Elbow
Deep dislocation 107
cerebral venous thrombosis 294 joint 91
vein thrombosis 44 Embryology 147, 203, 205
Degenerative Emphysema 13
disc disease 321 Empty sella syndrome 297
spondylolisthesis 323 Encephalomyelitis, acute disseminated 309
Delivery technique 68 Enchondroma 115
Dementia 363 Endobronchial metastases 5
Demyelinating diseases 308 Endocrine diseases 98

Index.indd 370 8/12/2010 12:19:28 PM


Index 371

Endodermal sinus tumour 223 Features of malignant head and neck nodes 351
Endometrial Features of normal
carcinoma 218 CCA waveform 75, 294
hyperplasia 218 ECA waveform 75, 294
polyp 218 ICA waveform 75, 294
Endometrioid carcinoma 222 Female
Endometriosis 203, 219 infertility 216
Endotracheal tube 48 pseudohermaphroditism 252
Endovascular aneurysm repair (EVAR) 65 Femoral artery 71
Enhancing pseudoaneurysm 69
structures on CT 362 Femoropopliteal angioplasty and stenting 68
structures on MRI 362 Fetal circulation 81, 263
Eosinophilic granuloma 115 Fibroadenolipoma 228
Ependymoma 238, 274, 279, 324 Fibroadenoma 227
Epidermoid 329 Fibrodysplasia ossificans progressiva 114
cysts 208 Fibroid uterus 220
Epididymis 205 Fibrolamellar HCC 140
Epididymitis 206 Fibrosarcoma 119
Epidural Fibrosing mediastinitis 58
abscess 320 Fibrothecoma 224
fibrosis 320 Fibrotic stage 24
haematoma 320 Fibrous
metastases 326 cortical defect 115
Epiglottitis, acute 259 dysplasia 115, 333
Epilepsy 362 histiocytoma, malignant 119
Epiploic appendagitis 179 Fibrosis, massive progressive 31
Epitympanic recess 327 Fibular collateral ligament 112
Eponymous sarcoidosis syndromes 23 Fifth cranial nerve 363
Erlenmeyer’s flask deformity 127 Filum terminale 318
Esthesioneuroblastoma 334 First branchial cleft cyst 352
Ethmoid roof 331 Fissures and junctional lines 16
Ewing’s sarcoma 119 Flexion tear-drop fracture 304
Excessive callus 128 Focal
Exostosis of external auditory canal 328 fat necrosis 228
Extension tear-drop fracture 304 nodular hyperplasia 138
External hernia 172 radiation necrosis 278
Extraconal tumours 313 thickening 362
Extradural Follicular carcinoma 358
haematoma 301 Fractures 50, 123
tumours 326 Fractures of
Extrahepatic cholangiocarcinoma 146 distal radius 107
Extralobar sequestrations 10 radius and ulna 107
Extramedullary Freiberg’s disease 271
haemopoiesis 57 Functional
intradural spinal tumours 325 endoscopic sinus surgery 331
plasmacytoma 345 segmental anatomy 134
Extrapulmonary complications 35 Fusion of cervical spine 126
Extravaginal 208
Extravesical insertion of ureter 201 G
Extrinsic allergic alveolitis 23 Galactocoele 228
Eyeball 310 Gallbladder and biliary tree 144
Gastric
F cancer 167
Fat embolism 45 carcinoid 174
Fatty liver disease 140 gastrointestinal stromal tumour 169

Index.indd 371 8/12/2010 12:19:28 PM


372 Index

thickened mucosal folds, causes 169 Heart valve imaging 88


ulcer 168 Hepatic
volvulus 168 artery 134
Gastrinoma 153 haemangioma 137
Gastritis 166 veins 135
Gastrointestinal haemorrhage, acute 73, 74 veno-occlusive disease 142
Gastrointestinal tumours in children 246 Hepatitis 135
Gastro-oesophageal reflux 242 Hepatobiliary system 134
disease 162 Hepatoblastoma 247
Gastroschisis 244 Hepatocellular
Generalised anaphylactoid reaction 70 adenoma 138
Genitourinary carcinoma 139, 247
syndrome 249 Hernia 172
tract tuberculosis 199 Herpes simplex encephalitis 287
Germ cell tumour 55, 223 Heterotopic grey matter islands 241
Germinal matrix haemorrhage 234 Hibernating myocardium 87
Germinoma 364 High-attenuation metastases on non-contrast
Gestational trophoblastic disease 217 CT 275
GI tract trauma 171 High-voltage technique 16
Giant cell Hip 92
astrocytoma 239 Hippocampal sclerosis 362
tumour 115 Hirschsprung’s disease 245
Glioblastoma multiforme 274 Histiocytosis X 299
Gliomas 274 HIV- and AIDS-related lung disease 38
Glomus jugulare 342 HIV
Glottic tumours 345 encephalitis 288
Glucagonoma 153 oesophagitis 164
Goodpasture’s syndrome 19 Hodgkin’s lymphoma 5
Gout 96 Horner’s syndrome 312
Gradenigo’s syndrome 328 Horseshoe kidney 249
Graft-versus-host disease 177 HRCT 21
Granulomatous disease, chronic 261 HSV oesophagitis 164
Granulosa cell tumour 223 Hyaline membrane disease 253
Graves’ disease 356 Hydatid disease 36
Ground-glass opacification 22 Hyoscine butylbromide 70
Growing fracture 300 Hyperextension 105
Growth rate assessment 7 Hyperflexion 105
Gynaecomastia 227 Hyperparathyroidism
primary 98, 359
H secondary 360
Haemangioblastoma 277, 325 Hyperprolactinaemia 297
Haemangioma 314 Hypoparathyroidism 360
Haematological diseases 101 Hypopituitarism 296
Haematoma signal on MRI 131 Hypoplasia 9, 282
Haematoma, subdural 301 Hypotension with bradycardia 69
Haemochromatosis 136 Hypothalamic hamartoma 299
Haemoperitoneum 171 Hypothalamus 296
Haemophilia 102 Hypothyroidism 99
Haemorrhagic metastases 274 Hypotympanum 327
Haemothorax 50 Hypovolaemia 171
Hair-on-end skull 362 Hysterosalpingography 216
Halo sign 21
Hamartoma 8, 228 I
Hangman’s fracture 304 Iatrogenic acquired TOF 157
Hashimoto’s thyroiditis 356 ICA 331
Head and neck lymph nodes 350 stenosis 294
Heart disease, left 47 Idiopathic pulmonary haemosiderosis 19

Index.indd 372 8/12/2010 12:19:29 PM


Index 373

Iliac artery 71 Juvenile


angioplasty and stenting 68 angiofibroma 259, 340
Image resolution 88 chronic arthritis 94, 270
Immature teratoma 223 pilocytic astrocytoma 237, 279
Infantile cortical hyperostosis 272 rheumatoid arthritis 94, 270
Infantile haemangioendothelioma 246 Juxta-articular osteoporosis 126
Infarction
acute 290 K
established 291 Kaposi’s sarcoma 39
Inflammatory Kidney 249
aneurysm 65 Klebsiella pneumonia 34
bowel disease 164 Knee
carcinoma 231 injuries 110
pseudotumour 203 joint 92
Infrahyoid pathology 352 Kruckenberg’s tumour 224
Infratentorial
intra-axial tumours 277
L
metastases 277 Lacrimal apparatus 316
Insulinoma 153 Lacrimal gland, enlarged 317
Interlobular septal thickening 22 Lacunar infarction 291
Internal Lamina dura 348
auditory canal 327 Large
carotid artery 289 bowel 171
hernia 172 bowel obstruction 178
Interstitial cell carcinoma 3
fibrosis 25 Laryngeal
lung diseases 28 carcinoma 344
Intervertebral disc calcification 324 oedema 69
Intestinal lymphoma 173 Laryngocele 345
Intra- and extracardiac shunts 46 Laryngomalacia 258
Intra-arterial thrombolysis 68 Laryngopharynx 339
Intraconal tumours 313 Laryngotracheobronchitis 259
Intraductal Larynx 344
papillary mucinous tumour of pancreas 151 Lateral
papilloma 227 cervical oesophageal pouch and diverticulum
Intrahepatic cholangiocarcinoma 146 165
Intralobar sequestrations 10 collateral ligament 112
Intramedullary pharyngeal pouch and diverticulum 165
arteriovenous malformation 319 sulcus 364
spinal tumours 324 Late-stage disease 39
Intramural haematoma 63 Left-to-right shunt 83
Intrathoracic metastasis 211 Legionella pneumophila 35
Ischaemic colitis 180 Leiomyoma 164, 220
IVC filters 45 Leptomeningeal
Ivory vertebra 129 carcinomatosis 325
metastases 276
J Leukaemia 6
Jaffe-Campanacci syndrome 116 Ligamentous injury 111
Jefferson fracture 304 Limb and skeletal injuries 267
Jejunal and ileal carcinoid 175 Limb ischaemia, acute 69
Joints of Limitations of ultrasound 76, 295
lower limb 92 Limited form of scleroderma 26
upper limb 91 Limping child 268
Jugular Linea aspera 92
bulb paraganglioma 342 Linear fracture 300
vein thrombosis 352 Lines and tubes 48

Index.indd 373 8/12/2010 12:19:29 PM


374 Index

Lingual thyroid 357 Mallory-Weiss tear 159


Lingula 16 Malrotation 172, 243
Linitis plastica, causes 170 Mandible 347
Lipoma 228, 353 Mandibular
Lipoma of corpus callosum 281 fractures 347
Liver hypoplasia 347
abscess 136 nerve 363
anatomy 134 pathology 347
and biliary system 245 Marchiafava-Bignami syndrome 309
Lobar anatomy 15 Marfan’s syndrome 97
Lobar collapse 16 Mature teratoma 223
Lobectomy 48 Maxillary nerve 363
Lobular carcinoma 231, 232 MCA infarction 290
Low McDonald MRI criteria for multiple sclerosis 308
attenuation lesions in basal ganglia 361 Meckel’s diverticulum 178
marrow signal 131 Meconium
signal on T2WI 130 aspiration syndrome 254
Lower ileus 243
gastrointestinal bleeding 73 Medial
limb trauma 109 cruciate ligament 112
lobe 15, 16 wall 316
oesophageal perforation 159 Mediastinal
Lung injury 52
cancer 2 masses 261, 262
diffuse disease 21 Mediastinitis 57
metastases 4 Mediastinum 54
transplantation 48 Medullary
Lymphadenopathy 24 carcinoma 231, 358
Lymphangioleiomyomatosis 28 metastases 325
Lymphangitis carcinomatosis 5 sponge kidney 189
Lymphocytic Medulloblastoma 237, 279
hypophysitis 296 Megaureter 251
interstitial pneumonia 39 Melanoma 314
Lymphoma 5, 299, 316, 334, 358 metastases 275
secondary 120 Meningioma 275, 325, 329
Lymphoma of Meningitis, bacterial 286
colon 174 Meniscal
small intestine 174 cyst 111
stomach 174 tears 110
Lymphoma versus toxoplasmosis 275 Menisci 93
Lytic bone lesions in children 129 Mesenteric adenitis 244
Lytic spondylolisthesis 323 Mesenteric ischaemia 179
Mesothelioma, malignant 32
M Mesotympanum 327
M staging 4 Metastases 175, 314, 334, 338
Macroadenoma 298 Metastases to
Macronodular parenchymal disease 24 liver 140
Malacoplakia 204 thyroid 358
Male pseudohermaphroditism 252 Metastatic
Malgaigne’s fracture 109 disease 116
Malignant tumours of invasion 341
bladder 204 Microadenoma 297
bone 119 Micrognathia 347
larynx 344 Micronodular parenchymal disease 24
pharynx 340 Microscopic polyangiitis 27

Index.indd 374 8/12/2010 12:19:29 PM


Index 375

Midazolam 70 Neonatal
Middle chest conditions 253
ear cavity 327 cranial ultrasound 234
lobe 15, 16 pneumonia 254
mediastinum 56 pneumoperitoneum 247
Midgut volvulus 243 spine 241
Mikulicz’s syndrome 317 Neoplastic tracheal disease 12
Miliary Nephrocalcinosis 189
metastases 5 Nephrogenic adenoma 203
tuberculosis 37 Nerve sheath tumours 325
Mimics of breast cancer on mammography 232 Neuroblastoma 251
Minor salivary glands 336 Neurocysticercosis 361
Mirizzi’s syndrome 146 Neurofibroma 128, 326, 352
Mitral stenosis 19 Neurofibromatosis 283
Mondini malformation 330 Neurogenic tumours 57
Morphological assessment 7 Neuropathic arthropathy 96
Mosaic attenuation 21 Nodal metastases 341
Mucocele 332 Non-accidental injury 267
Mucoepidermoid tumour 338 Non-atherosclerotic pathology 71
Mucormycosis 332 Non-functioning islet cell tumours 153
Mucosa-associated lymphoid tissue (MALT) Non-Hodgkin’s lymphoma 5, 341
lymphoma 167 Non-infectious pulmonary disease 39
Multicystic dysplastic kidney 249 Non-neoplastic tracheal disease 11
Multifocal leukoencephalopathy, progressive 288 Non-ossifying fibroma 115
Multinodular goitre 356 Non-small cell carcinoma 2, 3
Multiple Non-thrombotic
enchondromas 115 obstruction 141
endocrine neoplasia 154, 215 pulmonary embolism 45
myeloma 117 Non-traumatic subarachnoid haemorrhage 305
sclerosis 308, 319 Normal
Muscles of ocular motility 310 folds seen on imaging 178
Musculoskeletal effects of phenytoin 124 imaging features 354
Mycetoma formation 37 MRI appearances of pituitary 296
Mycobacterium avium-intracellulare complex 39 MRI findings in ageing brain 363
Mycoplasma pneumonia 34 variants and pitfalls 234
Myelitis 319 Normally
Myocardial calcified structures 362
infarction 86 enhancing structures 362
perfusion imaging 86 Nuclear cardiology 84
Myocarditis 87 Nutritional diseases 269
Myositis ossificans 114
O
N Obstructive cholangitis 146
N staging 3 Occupational lung disease 31
Narrow intervertebral disc spaces 324 Odontogenic keratocyst 347
Nasal Odontoid fractures 304
and paranasal sinus disease 27 Oesophageal
polyps 333 atresia 242
septal perforation 332 cancer 157
Nasolacrimal duct obstruction 317 perforation 159
Nasopharyngeal carcinoma 340 ring 160
Nasopharynx 339 strictures 160
Neck 350 varices 161
Necrotising web 160
enterocolitis 244 Oesophagitis 163
leukoencephalopathy 278 Oesophagus 156, 242

Index.indd 375 8/12/2010 12:19:29 PM


376 Index

Oligodendroglioma 274 Pancreas 147


Ollier’s disease 115 divisum 147
Omphalocoele 243 Pancreatic
Oncocytoma 195 anomalies 147
Ophthalmic nerve 363 duct adenocarcinoma 151
Optic islet cell tumours 153
glioma 313 pseudocyst 149
meningioma 314 Pancreatitis 148
nerve 331 abscess 150
Orbit and lacrimal gland 310 acute 150
Orbital chronic 150
foramina and contents 310 emphysematous 150
plate fracture 300 Papillary carcinoma 230, 358
pseudotumour 312 Papillitis 311
rim fractures 316 Papilloedema 311
trauma 316 Papilloma, inverting 334
tumours 313 Paraganglioma 342
varix 313 Paraglottic space 344
Orchitis 206 Paranasal sinuses 331
Oropharynx 339 Parapelvic cysts 189
Osgood–Schlatter disease 268 Parapharyngeal space 342
Osseous metastasis 211 Paraspinal abscess 57
Ossification centres 90 Parasympathetic tumour 57
Osteoarthritis 94 Parathyroid adenoma 56
Osteoblastoma 116 Parathyroid glands 359
Osteochondritis dissecans 113 Parenchymal disease 22, 24
Osteochondroma 120 Parieto-occipital sulcus 364
Osteogenesis imperfecta 123, 267 Parotid
Osteoid osteoma 117 gland 336
Osteoma 334 haemangioma 338
Osteomalacia 102 Particle disease 123
Osteomyelitis 100 Patella 93
Osteopetrosis 103 Pathways of tumour invasion 340
Osteoporosis 98 Peliosis hepatis 142, 247
hip, transient 124 Pelvic
Osteosarcoma 119 dislocation 109
Otitis inflammatory disease 218
externa 328 injury 109
media 327 kidney 249
Otosclerosis 330 lipomatosis 202
Ovarian Pelvis 92
metastases 224 Penetrating atherosclerotic ulcer 63
tumours 222 Peptic ulcer disease 167
Ovary 222 Percutaneous nephrostomy 77
Ovary in reproductive age group 216 Periarticular calcification 127
Pericardial
P cyst 56, 84
Paediatric effusion 83
brain tumours 237, 279 Pericardium 83
cardiovascular radiology 263 Periosteal reaction in children 267
gastrointestinal radiology 242 Peripheral vascular
musculoskeletal radiology 267 anatomy 71
neuroradiology 234 disease 66
respiratory radiography 253 Periventricular leukomalacia 235
Paget’s disease 103, 230 Permeative pattern in bone 129
Pancoast’s tumour 4 Persistent left SVC 59

Index.indd 376 8/12/2010 12:19:29 PM


Index 377

Perthes’ disease 112, 268 Prostate


Phaeochromocytoma 213 carcinoma 210
Phakomatoses 282 gland 210
Pharynx 339 Prostatitis 210
Phlegmonous gastritis 166 Prosthetic joint 123
Phyllodes tumour 227 Protrusio acetabuli 126
Pick’s disease 363 Prune belly syndrome 251
Pigmented villonodular synovitis 121 Prussak’s space 327
Pilocytic astrocytoma 277 Pseudoarthrosis 128
Piriform fossae 344 Pseudocoarctation 266
Pituitary Pseudohypoparathyroidism 360
disorders 296 Pseudomembranous colitis 180
gland 296 Pseudomonas pneumonia 35
metastases 299 Pseudopseudohypoparathyroidism 360
tumours 297 Psoriatic arthropathy 95
Pleomorphic adenoma 337 Pulmonary
Pleural agenesis 9
effusion 17, 25, 32, angiography 43
plaques 31 aplasia 9
space 50 arterial hypertension 46
thickening, diffuse 32 capillary haemangiomatosis 47
Pneumatosis intestinalis, causes 186 contusion 51
Pneumocystis disease 27
carinii pneunomia 38 embolism in pregnancy 44
jiroveci 38 fibrosis, patterns 28
Pneumomediastinum 58 haemorrhage 19
Pneumonectomy 48 hypoplasia 9, 256
Pneumonias 35 infections 34
Pneumothorax 51 Langerhans cell histiocytosis 28
Polyarteritis nodosa 72 lobule, secondary 21
Polycystic kidney disease manifestations 37
autosomal dominant 188 nodules 25
autosomal recessive 250 plethora without cyanosis 263
Polymyositis 26 sequestration 9, 257
Popliteal artery 71 thromboembolism 41
entrapment syndrome 72 vasculitides 26
Popliteus muscle 93 venolobar syndrome 256
Portal vein 134 veno-occlusive disease 47
thrombosis 142 Pulse oximetry monitoring 70
Post-cardiac surgery 49 Pyelonephritis
Posterior bacterial, acute 192
cruciate ligament 112 emphysematous 193
junctional line 16 Pyloric stenosis 169, 242
mediastinum 57 Pyogenic
scalloping 323 discitis 321
triangle 350 liver abscess 137
urethral valve 251 Pyonephrosis 193
Posterolateral corner injury 111 Pyrophosphate arthropathy 96
Postoperative chest radiography 48
Post-primary tuberculosis 37 R
Pre-epiglottic space 344 Radial scar 229
Preserved lung volumes 28 Radiation enteritis 176
Pre-vertebral Radiation effects, late 278
abscess 341 Radiation injury of
space 341 colon and rectum 177

Index.indd 377 8/12/2010 12:19:29 PM


378 Index

small intestine 177 Rotator cuff 91


stomach 176 Round atelectasis 32
Radiation necrosis versus tumour recurrence 278 Run-off vessels 71
Radiation-induced lung disease 29, 30
Radicular cyst 347 S
Radiological management 162 Sacroiliac joint 92
Radionuclide imaging 188 Sacroiliitis 127
Radiopharmaceutical agents 85 Salivary gland 336
Raised alkaline phosphatase 126 stones 337
Ranula 337 tumours, malignant 338
Raynaud’s syndrome 72 Salter–Harris injuries 268
Reduction of intussusception 244 Sarcoidosis 23, 87, 337
Reiter’s syndrome 95 Scaphoid 91
Relapsing polychondritis 12 Schatzki’s ring 160
Relations of pituitary gland 296 Scheuermann’s disease 271
Relevant vascular anatomy 289 Schistosomiasis 203
Renal Scimitar syndrome 9, 256
abscess 192 Scleroderma 26, 95, 164
adenoma 195 Sclerosing cholangitis, primary 146
agenesis 249 Sclerosing stromal cell tumour 224
artery occlusion 192 Sclerotic pedicle 126, 324
artery stenosis 75, 191 Scrotum 205
carcinoma 196 Scurvy 102, 270
cyst, simple 195 Second branchial cleft cyst 352
disease 27 Septic
failure from diffuse parenchymal disease, arthritis 101
chronic 198 emboli 45
haemorrhage 190 Serous and mucinous tumours 222
infarction 190 Sertoli and Leydig cell tumour 224
lymphoma 198 Severe acute respiratory syndrome (SARS) 34
metastases 198 Sex cord-stromal tumours 223
osteodystrophy 99 Shoulder
papillary necrosis 198 dislocation 108
sinus cysts 189 joint 91
system 188 Sickle cell anaemia and thalassaemia 103
trauma 194 Sigmoid volvulus 183
vein thrombosis 191 Silicosis and coal worker’s pneumoconiosis 31
Residual cyst 347 Sinusitis, bacterial 332
Restrictive cardiomyopathy 80 Sjögren’s syndrome 336
Retinoblastoma 239, 315 Skeletal hyperostosis, diffuse idiopathic 322
Retroperitoneal fibrosis 201 Skull base fractures 300
Retropharyngeal Skull vault
abscess 341 fractures 300
space 341 thickening 362
Retrorectal space, widered, causes 183 Slipped
Reversible posterior leukoencephalopathy 361 femoral epiphysis 113
Rhabdomyosarcoma 315 upper femoral epiphysis 269
Rheumatoid arthritis 25, 94, 346 Small airways disease 21
Rib fractures 50 Small bowel
Rickets 101, 269 aphthoid ulceration, causes 180
Riedel’s thyroiditis 357 dilated, causes 178
Right nodules, causes 179
aortic arch branch patterns 61 obstruction 178
atrial enlargement 79 strictures, causes 179
Rocker bottom feet 271 Small cell lung cancer 2

Index.indd 378 8/12/2010 12:19:29 PM


Index 379

Soft trachea 258 Suprasellar


Solitary germinoma 299
bone cyst 118 mass lesions 299
pulmonary nodule 7 meningioma 299
Somatostatinoma 154 Supratentorial
Spatial resolution 88 extra-axial tumours 275
Spermatic cord 205 intra-axial tumours 274
Spina bifida metastases 274
aperta 284 Surgical shunt procedures 82
occulta 284 Sutural bones 272
Spinal SVC obstruction 58
cord injury 303 Swan-Ganz catheter 48
dysraphism 284 Sylvian fissure 364
fusion 322 Sympathetic ganglia tumour 57
stenosis 323 Synovial
Spine 318 chondromatosis 121
Spondylolisthesis 105, 323 lesions 121
Spondylosis 105 sarcoma 121
Squamous Syringomyelia 285
cell carcinoma 2, 12, 334 Systemic lupus erythematosus 26, 95
papilloma 344
Staging of T
non-small cell lung cancer 3 Tachypnoea of newborn, transient 254
small cell lung cancer 2 Takayasu’s arteritis 64
Staphylococcal pneumonia 35 Talipes equino varus 271
Sternal fracture 50 Teeth 349
Stippled epiphysis 129 Tegmen tympani 327
Stomach 166 Temporal bone 327
Straddle fracture 109 fractures 300
Streptococcal pneumonia 34 Temporal resolution 88
Stress Temporomandibular joint (TMJ) 347
fractures 113 Tendon injuries in lower limb 114
imaging 85 Tension pneumothorax 51
Stroke 289, 290 Teratoma 56
chronic, imaging features 291 Territorial involvement 290
Sturge-Weber syndrome 282 Terminal ileum, causes of lesions in 180
Subacromial subdeltoid bursal impingement 108 Tertiary
Subarachnoid haemorrhage 305, 306 contractions in oesophagus 165
Subclavian steal syndrome 77, 295 hyperparathyroidism 360
Subdural Testicular
empyema 286 calcifications and microlithiasis 208
haematoma 301 cysts 208
Subfalcine herniation 303 neoplasm 207
Subglottic torsion 208
stenosis 345 trauma 208
tumours 345 Testis 205
Sublingual gland 336 Tethered spinal cord 241, 285
Submandibular gland 336 Tetralogy of Fallot 81, 263
Superior mediastinum 54 Thallium-201 85
Supernumerary teeth 349 Third nerve palsy 312
Supracondylar injuries 107 Thoracic
Supraglottic and lumbar spine injuries 105
hypermobility syndrome 258 aorta 60
tumours 344 aortic aneurysm 64
Suprahyoid pathology 352 duct 50
spine fracture 50

Index.indd 379 8/12/2010 12:19:29 PM


380 Index

Thornwald cyst 339 Tricuspid atresia 81, 264


Thromboembolic Trigeminal nerve 363
disease 41 True hermaphroditism 252
pulmonary hypertension, chronic 46 Truncus arteriosus 82, 265
stroke 290 Tuberculoma 38, 287
Thrombotic obstruction 141 Tuberculosis 36, 39, 287
Thumb fractures 105 post-primary 37
Thymic primary 275
carcinoid 55 Tuberculous
carcinoma 55 adenitis 353
cyst 55 discitis 321
hyperplasia 54 empyema 37
Thymolipoma 55 meningitis 287
Thymoma 55 oesophagitis 163
Thymus 261 osteomyelitis 100
Thyroglossal duct cyst 355 Tuberous sclerosis 29, 196, 240, 282
Thyroid Tubular cancer 230
eye disease 312 Tumours 115
gland 354 Tumours of renal tract 251
malignancy 358 Tunica albuginea cyst 208
masses 54 Type
nodule 357 A dissection 62
scintigraphy 354 B dissection 62
Thyroiditis, subacute 356 Types of polyp 182
Tibialis posterior tendon tear 114
TMJ U
arthritis 347 Ulcerative colitis 184
subluxation 347 Undescended testis 208
TOF, acquired, malignant 157 Unstable injury patterns 105
Tonsillar herniation 303 Upper
Total anomalous pulmonary venous connection 82, airway pathologies 257
264 airway problems in children 253
Toxoplasmosis 288 and mid-oesophageal perforation 159
Trachea 11 gastrointestinal bleeding 73
Tracheal limb trauma 105
bronchus 11 lobe 15, 16
stenosis 259 tract transitional cell carcinoma 197
Tracheobronchial Ureter 249
papillomatosis 12 Ureteral duplications 201
stenosis 12 Ureteritis cystica 202
tear 53 Ureters 201
Tracheobronchomegaly 12 Urethra 203, 251
Tracheoesophageal fistula 242 Urinary bladder 201
Tracheomalacia 258 Urticaria 69
Tracheo-oesophageal fistula (TOF) 156 Uterine
Tracheostomy 48 fibroid embolisation 74
Trans-jugular intrahepatic portosystemic shunt 77 tuberculosis 218
Transposition of great arteries 82, 264 Ureterocoele, ectopic 201
Transtentorial herniation 303 Uterus 216
Trauma 105, 346
Traumatic
V
injury 303 Vagus nerve paraganglioma 342
intracranial haemorrhage 301 Variants of aortic dissection 63
subarachnoid haemorrhage 302 Varicella-zoster virus 34
Tree-in-bud pattern 22 oesophagitis 164

Index.indd 380 8/12/2010 12:19:29 PM


Index 381

Varicocoele 206 Vitreous haemorrhage 316


embolisation 74 von Hippel–Lindau disease 196, 283
Vas deferens 205
Vascular W
anatomy 134 Warthin’s tumour 337
calcifications 73 Watershed infarct 293
complications 150 Wegener’s granulomatosis 26, 333
disease 21, 66 Wernicke’s encephalopathy 309
malformations 305 Whipple’s disease 176
Vasovagal reaction 69 White matter oedema, transient 277
Vein of Galen malformation 240 Widened
Ventricular enlargement cranial sutures 236
left 79 interpedicular distance 323
right 80 Widening of femoral intercondylar notch 126
Venous sinus anatomy 290 Wilms’ tumour 251
Vertebra Wormian bones 129, 272
plana 127 Wrist joint 91
artery 289
body metastases 321
X
Vertebral ossification, causes 364 Xanthogranulomatous
Vertebrobasilar infarction 291 cholecystitis 145
Vesicoureteral reflux 250 pyelonephritis 193
Vestibular aqueduct syndrome (enlarged) 330
VIPoma 154
Z
Viral pneumonia 34 Zenker’s diverticulum 165
Virchow’s triad 141

Index.indd 381 8/12/2010 12:19:29 PM

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