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HYDROCEPHALUS term is derived from Greek
word,Hydro-water and cephalous-head.
It is excessive accumulation of fluid in
brain,also known as water on the brain,Here
water signifies CSF.
Excessive accumulation of CSF
Abnormal widening or ventricles

Harmful pressure on brain tissues

Signs and symptoms of
hydrocephalus
4Affects both pediatric and adult age group patient.
4It has been estimated that 7,00,000 children and
adults are living with hydrocephalus.
4Incidence in pediatric age group 1 in 500 live births
hence making it one of the most common disabilities
even more common than Down syndrome and
Deafness.
4Also it is the leading cause of brain surgery for
children.
4Pediatrics hydrocephalus may also be a heritable
condition mainly affecting males.
4Refrences have been found in ancient egyptian
medical literature from 2000 B.C.to 500A.D.
4First described by HIPPOCRATES in 4th
centuryB.C.
4But more accurate description given by roman
physician GALEN in 2nd century A.D.
4First clinical description and operative procedure
for hydrocephalus appears in Al-Tasrif(1000A.D.)by
Arab surgeon Abu-al-Qasim who clearly described
the evacuation of superficial intracranial fluid in
hydrocephalic children.
' Set of structures containing CSF in brain
which is continuous with central canal of
spinal cord.
Composed of 4 ventricles:
2 Lateral ventricles
Third ventricle
Fourth ventricle

Derived embryologically from

central canal of neural tube
ANTERIOR HORN

POSTERIOR HORN
INTERVENTRICULAR FORAMEN
THIRD VENTRICLE
INFERIOR HORN

CEREBRAL AQUEDUCT

FOURTH VENTRICLE

SPINAL CANAL
4It is produced by
ependymal cells of choroid
plexus found in
components of ventricular
system except from
cerebral aqueduct and
occipetal and frontal horns
of lateral ventricle
 Lateral ventricle
Foramen of Monro
3rd ventricle
Aqueduct of Sylvius
4rth ventricle

3 foramina central canal of spinal cord

Cisterns of sub
Lumbar cistern
arachnoid space

Around cerebral
cortex
4The fluid then flows around the superior sagittal sinus.
4It reabsorbed via arachnoid villi into venous system.
4ARACHNOID GRANULATIONS-these are small protrusions of
arachnoid which protrude into the venous sinuses of brain allows
csf exit from brain and enter blood.
4The largest arachnoid granulation lies along the superior
sagittal sinus.
4The arachnoid villi act as a one way valve.Normally PRESSURE
OF CSF >PRESSURE OF VENOUS SYSTEM.So CSF is drained
through villi into venous blood.
4Some amount of CSF is also drained through lymphatics
assossiated with extra cranial nerves,ex.-through axons of
olfactory nerve via cribriform plate.
VOLUME 150ml

PRODUCTION 20ml/hr(80% by
choroid plexus)

ABSORBTION Arachnoid
villi(pressure
dependent)
RELATIVE TO PLASMA Reduced K and Ca
ions
Increased Cl and Mg
ions
pH=7.33 to 7.35
PRESSURE 5 to 15 mmHg(6 to
18cm of water)
1. PROTECTION- It acts as a shock absorber.
2. TRANSPORT-Act as a vehicle for
delivering nutrients and removing waste.
3. It flows between the cranium and spine
and compensates for changes in intra
cranial blood volume.
Normal ventricles Dilated ventricles
4In foetus fontanelle and sutures are opened.
4Posterior fontanel - at birth.
4Anterior fontanel ± at 18 months.
4In initial 6 months of life brain expands at a greater rate.
4Opened sutures and fontanelle provide proper space for growth
of brain and make the skull pliable.
4If ther is accumulation of fluid in brain of neonate,the increased
pressure causes whole skull to swell.
4Fontanelle bulges and sutures remain seperated and this
presents as macrocephaly.
4Hence MACROCEPHALY and OPEN FONTANELLE provide
EARLY DIAGNOSIS in infants.
There are basic three mechanism involved in hydrocephalus
1. Over production of CSF.
2. Defective absorption of CSF into circulation
3. Blockage of CSF outflow in ventricle or subarachnoid
space.
As a result fluid accumulated in the ventricles will lead to brain
damage either through formation of internal or external
hydrocephalus
 Foramina of 4th ventricle or cerebral aqueduct blocked

CSF accumulated within the ventricles

Compression of nervous tissue

Irreversible brain damage and severely enlarged head

Subarachnoid hemorrhage
Block the return of CSF to circulation
CSF accumulation in sub arachnoid space
Pressure applied to brain externally
Compresses neural tissue and hence brain damage
1. COMMUNICATING HYDROCEPHALUS
4 Non-obstructive hydrocephalus
4 It is caused by impaired cerebrospinal fluid resorption
due to obstruction of CSF flow outside the ventricular
system,usually at the level of basal sub arachnoid cistern
or at the arachnoid granulations.
2. NON COMMUNICATING HYDROCEPHALUS
4 Obstructive hydrocephalus
4 It is caused by a CSF-flow obstruction with in the
ventricular system ultimately preventing CSF from
flowing into the subarachnoid space (either due to
external compression or intraventricular mass lesions).
Both communicating and non communicating can be
congenital or aquired.
CONGENITAL HYDROCEPHALUS
4Intrauterine infection-
' Rubella
' Cytomegalo virus
' Toxoplasmosis
4Intra cranial and Intra ventricular haemorrhage
4Congenital malformations-
' M ueduct stenosis,
' Dandy Walker Syndrome,
' Mrnold Chiari Syndrome,
' Midline tumors obstructinc CSF flow
ACQUIRED HYDROCEPHALUS

4Tuberculosis,Chronic & pyogenic Meningitis

4Post IVH

4Posterior Fossa tumors-

Medulloblastoma,Astrocytoma,Ependymoma

4Arterio-Venous malformation,Intra cranial

haemorrhage,ruptured aneurysm.

4Hydrocephalus ex vacuo
CHIARI TYPE I MALFORMATION

:Chiari type I malformation is the caudal displacement of the cerebellar

tonsils below the foramen magnum.
:Gives symptoms in adolescence or adult life(headache,neck pain,
myelopathy)
:The brain stem and lower cranial nerves are normal .

CHIARI TYPE II MALFORMATION

:Progressive hydrocephalus and myelomeningocele.

:Elongation of IV ventricle
:Involves caudal displacement of the lower brain stem and
stretching of lower cranial nerves
:Symptomatic patients may be treated with suboccipital
craniectomy.
Spina bifida is a developmental birth defect
caused by incomplete closure of embryonic
neural tube.

DM
DY WMR SY
DROM

4Cystic expansion of 4th ventricle in the

posterior fossa
4Developmental failure of roof of the 4th
ventricle during embryogenesis.
490% have hydrocephalus.
4Prominent occiput.
:2
r l
: r l r r
r l

4It is compensatory enlargement of cerebral

ventricles and subarachnoid space due to brain
atrophy or loss of brain parenchyma not the result
of increased ICP.
4 Can occur in post traumatic brain injuries and
even in some psychiatric disorders, such as
schizophrenia
4A chronic type of
communicating hydrocephalus
4Presents mainly in elderly
4The increase in intracranial
pressure (ICP) due to
accumulation of cerebrospinal
fluid (CSF) becomes stable
and that the formation of CSF
equilibrates with absorption.
4CSF pressure reaches a high
normal level of 150 to 200 mm
of H2O.
4Because of this equilibration, patients do not exhibit the
classic signs of increased intracranial pressure such as
headache, nausea, vomiting, or altered consciousness.

4patients do exhibit the classic triad of gait difficulties,

urinary incontinence, and mental decline

4It is often misdiagnosed as Parkinsons disease, Alzhiemer

disease, and senility due to its chronic nature and its
presenting symptoms

42 types a) Idiopathic
b) Secondary is due to subarachnoid haemorrhage,
head injury, cranial surgery, or CNS infection.
4 The patient of hydrocephalus develop symptoms due to raised ICP.
4 Headache which is raised in early morning or on lying down .
4 Vomiting, nausea, papilledema, sleepiness, or coma.
4 Elevated intracranial pressure may result in uncal and/or cerebellar tonsil
herniation, with resulting life threatening brain stem compression.
4 Further symptoms depend on the cause of the blockage, the person's age,
and how much brain tissue has been damaged by the swelling.
4 In infants with hydrocephalus, CSF fluid builds up in the central nervous
system, causing the fontanelle (soft spot) to bulge and the head to be
larger than expected.
Eyes that appear to gaze downward
Seperated sutures
Diplopia and blurred vision
Irritability
Seizures
Sleepiness
Symptoms that may occur in older children
The presentation in older children is more acute.Features
include:
4Brief shrill and high pitched cry

4Changes in personality, memory and ability to think or reason

4Changes in facial expression and eye spacing

4Crossed eyes and uncontrolled eye movements

4Difficult feeding, Excessive sleepiness

4Loss of bladder control

4Loss of coordination and trouble walking

4Muscle spasticity

4Slow growth(0-5years) and restricted movements

 Patient of hydrocephalus develop raised ICP

:Papilledema (more in old children)

:Cracked pot/Macewan sign(old children)
:Sixth nerve palsy
:Impaired upgaze
:Focal neurological deficits
:Impaired concious level
IN INFANTS
:Progressive macrocephaly
:Bulging anterior fontanelle
:Dilated scalp veins
:Sun setting signs
NPH may exhibit the classic triad (also known as Adam's triad) of
urinary incontinence, gait disturbance, and dementia
ë

 
4 is the first symptom of the triad
4Progressive
4Occurs due to expansion of the ventricular system(particularly
at the level of the lateral ventricles)
4 traction on the lumbosacral motor fibers
4unsteadiness and impaired balance, especially on stairs and
curbs.
4NPH gait disturbance is often characterized as a "magnetic gait,"
in which feet appear to be stuck to the walking surface until
wrested upward and forward at each step.
j   
4 is predominantly frontal lobe in nature,
4 with apathy, dullness in thinking, slight inattention. and
Memory problems.
4The dementia is thought to result from traction on
frontal and limbic fibers that also run in the
periventricular region
3) w   
4 appears late in the illness,
4consisting of increased frequency and urgency.
4 A physician selects the appropriate diagnostic tool based
on an individual¶s age, clinical presentation or type of
hydrocephalus and the presence of known or suspected
abnormalities of the brain or spinal cord.
4 Hydrocephalus is diagnosed through
1. Clinical neurological evaluation
2. Lumbar puncture
3. Cranial imaging techniques
: ultrasonography,
: computed tomography (CT),
: magnetic resonance imaging (MRI) and T2 weighted MRI
4. Other ICP-monitoring techniques.
1) CLINICAL NEUROLOGICAL EVALUATION
4 Signs symptoms and neurological examination with accurate
serial recording of head circumference will point towards the
diagnosis.
4 An increase in head circumference in 1st three months of life
>1cm every fortnight should arouse suspicion of
hydrocephalus
4 Persistent widening of squamo parietal sutures is not
physiological and should arouse suspicion of hydrocephalus.
2)LUMBAR PUNCTURE

a)Obstructive hydrocephalus-
This is a contraindication for LP because of the risk of causing tonsillar
herniation and death.

b)Non Obstructive hydrocephalus-

LP here may be both diagnostic-by measurement of opening pressure
therapeutic-by draining volume of csf

c) @

LP is usually the first step in diagnosis. In most cases, CSF pressure is
usually above 155 mmH2O.
  : Clinical evaluation is done before and after removal of CSF
(30 ml or more).It has a high predictive value for subse uent success with
shunting. This is called the "lumbar tap test" or Miller Fisher test. M "negative"
test has a very low predictive accuracy, as many patients may improve after
a shunt in spite of lack of improvement after CSF removal.
   :Infusing saline into the thecal sac while measuring
tha pressure to obtain and estimate of resistance to CSF
outflow>14mmHg/ml/min have a positive predictive value for responsiveness
to ventriculoperitoneal shunt insertion.
3) IMAGING STUDIES:
a) Uitrasonography(USG)
4 Serial USG helps to support the clinical diagnosis and to evaluate
serial ventricular size.
b) CT Scan and MRI
4 Ventricular size can be assessed more accurately with CT scan.
4 Information about cortical mantle, periventricular ooze and etiology
of hydrocephalus like Arnold chiari and dandy walker malformation
4 In children CT shows COPPER BEATING of skull because of chronic
raised ICP.
4 MRI/CT may be necessary to determine site of obstruction and in
congenital hydrocephalus to identify associated malformation.
4 MRI provide better anatomical detail of lesion and is particularly
helpful in diagnosis of aqueductal stenosis
4 Imaging however cannot differentiate between pathologies with
similar clinical picture like Alzheimer's dementia, vascular dementia
or Parkinson's disease.
Copper beating Mrnold chiari
appearence malformation
c) MID LINE T2 WEIGHTED MRI scan
Can be used to assess the suitability of patient for third
ventriculostomy by identifying the relationships of floor
of third ventricle,basilar artery and clivus.

d) ICP MONITORING
With a parenchymal probe placed into the frontal lobe
via a twistdrill burrhole is a useful diagnostic tool for
patients in whom hydrocephalus or CSF shunt
dysfunction is suspected.
Hydrocephalus is to be differentiated from conditions
manifesting as large head-
a) MEGANCEPHALY-causes include-
Hurlers syndrome
Metachromatic leukodystrophy
Taysachs disease
b)CHRONIC HAEMOLYTIC ANAEMIA-(widening of diploic bones)
c) VITAMINE D DEFICIENCY
d) SUB DURAL EFFUSION
e) CEPHALHEMATOMA
f) CAPUT SUCCEDENUM
g)OTHER CAUSES- HYDRANENCEPHALY,RICKETS,FAMILIAL
MACROCEPHALIES
Management of hydrocephalus depends on the underlying cause and
severity of symptoms
MEDICAL MANAGEMENT
This is a conservative approach for mild & slowly progressive
hydrocephalus or cases where surgery is not indicated
Acetazolamide[25 -100mg/kg/min].
Oral glycerol
SURGICAL MANAGEMENT
It includes-
a)Removing of a causative mass leison
Intracranial mass lesion usually present with obstructive hydrocephalus
It includes tumor removal and decompression of CSF pathway using
EVD(External ventricular drainage) to cover early post operative period.
b) Ventricular shunting
c) 3rd ventriculostomy
WHAT IS SHUNT?
4This system diverts the flow of CSF from the CNS to another
area of the body where it can be absorbed as part of the normal
circulatory process.
4A shunt is a flexible but sturdy plastic tube. A shunt system
consists of the shunt, a catheter, and a valve. One end of the
catheter is placed within a ventricle inside the brain or in the
CSF outside the spinal cord. The other end of the catheter is
commonly placed within the abdominal cavity, but may also be
placed at other sites in the body such as a chamber of the heart
or areas around the lung where the CSF can drain and be
absorbed. A valve located along the catheter maintains one-way
flow and regulates the rate of CSF flow.
CHMRM V P SHU
T
CHMRM V P SHU
T
WHAT IS VENTRICULO PERITONEAL SHUNT
4It involves shunt between lateral ventricle and peritoneal
cavity.
4Here there occurs the insertion of a catheter into lateral
ventricle{usually right frontal or occipital}.The catheter is then
connected to a shunt valve under the scalp and finally to a distal
catheter,which is tunnelled subcutaneously down to abdomen
and inserted into the peritonial cavity.if the CSF pressure > the
shunt valve pressure then CSF will flow out of the distal catheter
and can be absorbed by peritonial lining.
OTHER SHUNT SYSTEMS-
4Ventriculo atrial shunt
4Ventriculo pleural shunt.
4Lumbar peritonial shunt.
MECHANISM OF SHUNT VALVES
-Diaphragm system
-Ball in cone system
'These are pressure regulated which are sucseptible to
SIPHONING.
'There are three performance settings of shunts-high,medium,low.
'Shunt valves usually have an in-built CSF reservoir on the
ventricular side of the valve,which may be useful for shunt taping.

WHEN IS THE SHUNTING DONE

If the head size enlarges rapidly or is associated with a
progressive symtoms,where vision or life is endangered it
is desirable to treat surgically before irreparable damage
occurs specially in congenital obstructive
hydrocephalus,aquired hydrocephalus or periventricular
ooze with hydrocephalus and patients with tubercular
meningitis.
4An alternative procedure called third ventriculostomy. In this
procedure, a neuroendoscope ² a small camera that uses fiber optic
technology to visualize small and difficult to reach surgical areas ²
allows a doctor to view the ventricular surface. Once the scope is
guided into position, a small tool makes a tiny hole in the floor of the
third ventricle, which allows the CSF to bypass the obstruction and
flow toward the site of resorption around the surface of the brain.

4External drains can be placed within the ventricle (EVD) or the

Lumbar Thecal Sac (Lumbar drain).
4Useful for temporary CSF drainage.
4Can be also used to administer Intrathecal antibiotics to treat CSF
infections.
Possible complications include
4shunt malfunction
4shunt blockage
4shunt failure
4shunt infection.
4Over draining of shunts
4CSF leak
4Stroke & intracranial haemorrhage
4Shunt blockage may affect the ventricular catheter,shunt valve or
distal catheter.Causes include choroid plexus adhesion,blood or
cellular debris or misplacement of the distal catheter in the pre
peritoneal space.

4Shunt infection usually caused by skin commensals such as

staphylococcal epidermitis.Neonates are susceptible to E.coli and
hemolytic streptococcal infections.
4Risk factors for infections include-
Very young children
Open myelomeningocele
Longer operative time
Excessive staff movement into & out
of theater
490% infections become apparent clinically within 6 months.
4Treatment-Removal of shunts,
External drainage
treatment of infection prior to re-insertion of shunt at a
different site.
4Shunt system may overdrain leading to subdural haemorrhage.
4The prognosis for individuals diagnosed with hydrocephalus is
difficult to predict
4Prognosis depends the time of diagnosis and type of
hydrocephalus.
4Cognitive abilities appear to be better in non communicating
hydrocephalus and those with myelomeningocoele or chiari
malformation type II
4Communicating hydrocephalusis associated with more cognitive
impairment.Hydrocephalus due to intrauterine CNS infections
have grim developmental prognosis.
4If left untreated progressive hydrocephalus may be fatal.
4With good medical care & if there are no severe underlying brain
disorders that affect intelligence, patients of Hydrocephalus can
expect to live a productive & relatively normal life with adequately
functioning shunt.
CMS FROM DPMRTM
T OF
SURGRY,SGMH
4Ms we have seen that due to blockage of CSF outflow
or defective absorbtion or overproduction of CSF,there is
accumulation of fluid in ventricles which leads to brain
damage and produce signs and symptoms of increased
ICP.
4Only congenital form can be screened by proper
antenatal check ups & investigaions.
4It is diagnosed by neurological evaluation, Imaging,
umbar Puncture, & ICP monitoring.
4Only definitive treatment is SHU
TI
G of CSF either
into Peritoneal(VP shunt) or Pleural cavity.
4M team work of Gynaecologist, Paediatrician,
Psychiatrist, Physiotherapist and most importantly
Parents is re uired to combat this problem.
1. ailey & ove¶s short practice of of surgery,25th
di.(year of printing-2008)
2. Sabiston textbook of surgery Vol.II ,17th di.(2004)
3. Schwartz¶s Principles of surgery,8th di.(2005)
4. .D.Chourasia¶s Human Mnatomy Vol.III ,4th
di.(2009)
5. Concise textbook of surgery,S.Das,3rd di.(2001)
6. Rob and Smith¶s Paediatrics operative surgery,5th
di.(1995)
7. Harrison¶s Principles of Internal Medicine,17th
di.(2008)
8. Internet: www.wikipedia.com