KABWE CENTRAL HOSPITAL

“HYDROCEPHALUS”
Wisdom’s Lecture Notes

Banda Wisdom Chilufya

Outline

• Relevant anatomy and Normal flow of CSF

• Definition of hydrocephalus
• Epidemiology
• Aetiology
• Pathophysiology
• Clinical presentation
• Diagnosis
• Treatment and complications
Normal flow of CSF

· 50ml (neonates) 120-150ml (adults), pressure 100mm

CSF
· Production – Choroid Plexuses ventricles
· Lateral,3rd Ventricle,AS,4th Ventricle
· Lateral Foramina (Luschka) and Median Foramina
(Magendie)
· Reabsorbed Arachnoid Villi
· Function: shock absorber,nutrients,link Circulation and
the Brain
Hydrocephalus Definition

• Hydrocephalus is a dilatation of the cerebral

ventricular system, which is due to either an
obstruction to the flow of CSF, overproduction of
CSF, or a failure of reabsorption of CSF

• Hydrocephalus can be defined broadly as a

disturbance of formation, flow, or absorption of
cerebrospinal fluid (CSF) that leads to an
increase in volume occupied by this fluid in the
central nervous system (CNS)
Epidemiology

• Incidence of congenital hydrocephalus is 3 per 1,000

live births, while the incidence of acquired
hydrocephalus is not known exactly
• Internationally: About 100,000 shunts are implanted
each year in the developed countries, but little
information is available for other countries
• Generally, incidence is equal in males and females.
• Age: presents a bimodal age curve. One peak occurs
in infancy and is related to the various forms of
congenital malformations. Another peak occurs in
adulthood
Etiology

• Hydrocephalus results from an imbalance between

production and absorption of CSF

• It may be communicating or non-communicating

Communicating hydrocephalus

• Communicating hydrocephalus occurs when full

communication exists between the ventricles and
subarachnoid space.

• It is caused by overproduction of CSF (rarely),

defective absorption of CSF (most often), or venous
drainage insufficiency (occasionally).
Obstructive or noncommunicating

• Noncommunicating hydrocephalus occurs when

CSF flow is obstructed within the ventricular system
or in its outlets to the arachnoid space, resulting in
ventricular/subarachnoid space noncommunication.

• Commonly at the aquiduct or foramen of Magendie

and Luschka
CAUSES

• Hydrocephalus may either be congenital or

acquired
• Therefore causes can be classified into:
1. Congenital hydrocephalus causes
2. Acquired hydrocephalus causes
Causes of congenital hydrocephalus
• Congenital malformations

– Stenoses of the aqueduct of Sylvius due to malformation: This is

responsible for 10% of all cases of hydrocephalus in newborns.
– Dandy-Walker malformation: This affects 2-4% of newborns with
hydrocephalus.
– Arnold-Chiari malformation type 1 and type 2
– Agenesis of the foramen of Monro
– Congenital toxoplasmosis
– Bickers-Adams syndrome: This is an X-linked hydrocephalus
accounting for 7% of cases in males. It is characterized by
stenosis of the aqueduct of Sylvius, severe mental retardation,
and in 50% by an adduction-flexion deformity of the thumb.
Causes of acquired hydrocephalus

• Intraventricular hemorrhage (can be related to

prematurity, head injury, or rupture of a vascular
malformation)
• Infection: tuberculosis , chronic and pyogenic
meningitis
• Mass lesions (20% of all cases of in children)
usually tumors (eg, medulloblastoma, astrocytoma),
but cysts, abscesses, or hematoma also can be the
cause
Causes of acquired hydrocephalus

• Iatrogenic: Hypervitaminosis A, by increasing

secretion of CSF or by increasing permeability of
the blood-brain barrier, can lead to hydrocephalus.
• Idiopathic
• Note: there is an association between the
occurrence of spina bifida and hydrocephalus
Pathophysiology

• ICP rises if production of CSF exceeds absorption

• This occurs if CSF is overproduced, resistance to
CSF flow is increased, or venous sinus pressure is
increased.
• CSF production falls as ICP rises
• Compensation may occur through transventricular
absorption of CSF and also by absorption along
nerve root sleeves
Pathophysiology

• Ventricles are dilated

• Temporal and frontal horns dilate first, often
asymmetrically
• may result in enlargement of the third ventricle
downward into the pituitary fossa (which may cause
pituitary dysfunction).
• Ependymal lining of ventricles disrupted-
periventricular ooze- periventricular white matter
compressed
Pathophysiology

• Ventricular distension and increased ICP cause the

clinical manifestations of hydrocephalus
The signs and symptoms increased ICP include:
• Headache
• Nausea
• Vomiting
• Increased blood pressure
• Decreased mental abilities
• Confusion about time, and then location and people as
the pressure worsens
• Double vision
• Pupils that don’t respond to changes in light
• Shallow breathing
• Seizures
• Loss of consciousness
• Coma
Clinical manifestations

• Clinical features of hydrocephalus are influenced

by the following:
– Patient's age
– Cause
– Location of obstruction
– Duration
– Rapidity of onset
Clinical manifestations

• Symptoms in infants
– Poor feeding
– Irritability
– Reduced activity
– Vomiting
• Symptoms in children
– Slowing of mental capacity
– Headaches (initially in the morning) that are more
significant than in infants because of skull rigidity
Clinical manifestations

– Neck pain suggesting tonsillar herniation

– Vomiting, more significant in the morning
– Blurred vision - Consequence of papilledema and later of
optic atrophy
– Double vision - Related to unilateral or bilateral sixth nerve
palsy
– Stunted growth and sexual maturation -third ventricle
dilatation: obesity and precocious or delayed onset of
puberty
– Difficulty in walking secondary to spasticity: affects the lower
limbs preferentially (stretched periventricular pyramidal tract)
– Drowsiness
Clinical manifestations (Physical)

• Infants
– Head enlargement: Head circumference is in the
98th percentile for the age or greater.
– Dilated scalp veins: The scalp is thin and shiny with
easily visible veins.
– Setting-sun sign: In infants it is characteristic of increased
ICP. Both ocular globes are deviated downward, the
upper lids are retracted, and the white sclerae may be
visible above the iris.
Congenital hydrocephalus. Note the
enlarged cranium and prominent
scalp veins.
Clinical manifestations (Physical)

– Tense fontanelle: The anterior fontanelle in

infants who are held erect and are not crying
may be excessively tense.
– Dysjunction of sutures: This can be seen or
palpated.

– Increased limb tone: Spasticity preferentially

affects the lower limbs. The cause is stretching
of the periventricular pyramidal tract fibers by
hydrocephalus.
Clinical manifestations (Physical)

• Children
– Papilledema: if the raised ICP is not treated, this can lead
to optic atrophy and vision loss.
– Failure of upward gaze: This is due to pressure on the
tectal plate
– MacEwen’s sign: A "cracked pot" sound is noted on
percussion of the head indicating separation of the
sutures
Clinical manifestations (Physical)

• Children
– Unsteady gait: This is related to spasticity in the lower
extremities.
– Large head: Sutures are closed, but chronic increased
ICP will lead to progressive abnormal head growth.
– Unilateral or bilateral sixth nerve palsy is secondary to
increased ICP.
Investigations HYDROCEPHALUS

• Ultrasound (in infants), CT, or MR for diagnosis of

communicating hydrocephalus
• MR for diagnosis of noncommunicating
hydrocephalus
• Ultrasound (in infants) or CT to follow ventricular
size in response to treatment
• A CT scan or MRI of the head is recommended for
children with a meningocele because of the
association with hydrocephalus in some cases
Differential diagnosis

• Megaencephally: no raised ICP, no dilated

ventricals
• Chronic subdural heamatoma: large head mostly in
parietal regions, no prominent scalp veins, no
sunset sign
Management

• Medical Care:
• Medical treatment is used to delay surgical
interventionMedications affect CSF dynamics by
the following mechanisms:
– Decreasing CSF secretion by the choroid plexus -
Acetazolamide and furosemide
– Increasing CSF reabsorption - Isosorbide (effectiveness
is questionable)
Management

• Surgical Care:
• Surgical treatment is the preferred therapeutic
option
• Choroid plexectomy or choroid plexus coagulation
may be effective.
• Endoscopic fenestration of the floor of the third
ventricle (endposcpic third ventriculostomy)
establishes an alternative route for CSF toward the
subarachnoid space. It is contraindicated in
communicating hydrocephalus
Management

• Shunts eventually are performed in the majority of

patients
• A ventriculoperitoneal (VP) shunt is used most
commonly
• A ventriculoatrial (VA) shunt also is called a
"vascular shunt.“
• A ventriculopleural shunt is considered second line.
It is used if other shunt types are contraindicated.
• Other types: lumboperitoneal,
Complications of management

• Related to medical treatment

– Electrolyte imbalance
– Metabolic acidosis
• Related to surgical treatment
- shunt obstruction or disconnection
- VP shunt complications include peritonitis, inguinal
hernia
- VA shunt complications include septicemia, shunt
embolus, endocarditis, and pulmonary hypertension
complications

.Shunt Malfunction
A shunt blockage from blood cells, tissue or bacteria can
occur in any part of the shunt

Shunt malfunction is a partial or complete blockage of the

shunt that causes it to function intermittently or not at all.

Components can become disengaged or fractured as a result

of wear or as a child grows, and occasionally they dislodge
from where they were originally placed.
• Shunt Infection
The most common infection is Staphylococcus
Epidermidis, which is normally found on the surface
of a person’s skin and in the sweat glands and hair
follicles deep within the skin. May cause Peritonitis
• Over drainage
Ventricles to decrease in size creating slit-like ventricles
as a result of the brain and its meninges pulling away
from the skull. slit-ventricle syndrome (SVS)

• Under drainage
ventricles to increase in size and can fail to relieve the
symptoms of hydrocephalus
• Subdural hematoma
• Multiloculated hydrocephalus
• Disjunction sutures
• 35-38 cm at birth
• 0-3 months at 2cm
• 3-6months at 0.5cm
• Reduces in growth copper panel
• -
 Faith is like
electricity, you can’t
see it, but you can
see the light.
REFERENCES
• Clark. M., Kumar. P.(2009) Kumar & Clark’s Clinical Medicine
7th edition. Saunders Elsevier, london, UK.

• Parthasarathy .A. et.al (2009) IAP Textbook of Pediatrics 4th

edition. Jaypee Brothers Medical Publishers (P) Ltd, New
Delhi, India.

• Rubin. R., Strayer D.S. (2012) Rubin’s pathology:

Clinicopathologic Foundations of Medicine 6th edition.
Lippincott Williams & Wilkins, a Wolters Kluwer business,
Baltimore, USA.

• http//emedicine.medscape.com/article/244631.

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