HYDROCEPHALUS

BY : DIPANSHA
ROLL NO. : 28
 INTRODUCTION
• CEREBROSPINAL FLUID IS A CLEAR, COLOURLESS FLUID THAT
CIRCULATES WITHIN BRAIN AND SPINAL CORD ACTING AS A
CUSHION TO PROTECT THEM FROM INJURY
• NORMAL VOLUME OF CSF :
1. IN ADULTS : 150 mL
2. IN INFANTS : 50 mL
• PRODUCTION FROM WALLS OF VENTRICLE AND CHOROID PLEXUS IS
ABOUT 20ml/hour (0.33 mL/kg/hr). ALMOST ALL THE FLUID
PRODUCED IS ABSORBED WITHIN 8 hours.
 NORMAL CSF PHYSIOLOGY
• Production of CSF occurs in the choroid plexus lining
the lateral ventricle
Lateral ventricle
Foramen of monro

3rd ventricle

Aqueduct of Sylvius
4th ventricle

Foramen of Foramen of
magendie luschaka
Laterally into
Spinal region
subarachnoid
• Absorption occurs in subarachnoid space
space by subarachnoid.
villi into venous circulation

Normally there Occurs a balance b/w formation by choroid plexus and absorption by arachnoid villi
 DEFINITION

• Any imbalance in production or absorption of CSF leads to

hydrocephalus
• Hydrocephalus refers to an increase in CSF volume with ventricular
enlargement , often presenting symptoms of raised intracranial
pressure
• The accumulation of fluid could be either:
1. Intracerebral (ventricular)
2. Extracerebral (subarachnoid spaces)
 CLASSIFICATION OF HYDROCEPHALUS
• Based on etiology:-
Based on onset:-

• ACUTE HYDROCEPHALUS :- develops within days or few weeks.(for

eg. hydrocephalus due to tumour)
Manifested with Rapid progression of symptoms
• CHRONIC HYDROCEPHALUS :- CSF accumulation during months or
even years
Present with subtle signs of memory impairment , walking
difficulty(unsteady gait, feeling of being stuck on floor) ,or urinary
incontinence
Classical example is normal pressure hydrocephalus
• CONGENITAL HYDROCEPHALUS :- present at birth. Sometimes it
is apparent a few weeks or months after birth ,even though the
process started while the child was in utero
These are commonly obstructive , can be communicating as seen in
intrauterine toxoplasmosis or CMV infections.
• ACQUIRED HYDROCEPHALUS :- increased CSF accumulation
occurs after birth
Includes post traumatic hydrocephalus , hydrocephalus associated
with tumour , and normal pressure hydrocephalus
 CLINICAL FEATURES OF RAISED ICP
• High pressure headache that worse on coughing or bending forward
• Accompanied nausea, vomiting , blurred vision, double vision
• Cranial nerve compression can result in eye movement , pupil abnormalities
• Papilloedema in fundoscopy later leads to sec. Optic atrophy
• In infants : increased head circumference
Split sutures
Prominent scalp veins
Tense bulging fontanelle
Child will be irritable, lethargic and will not accept feeds
• Loss of upgaze : sunset sign (visible upper sclera) (late sign)
• Obstructive hydrocephalus can cause very sudden deterioration with coma
and death
• Lumbar puncture in obstructive hydrocephalus is contraindicated as it
carries a risk of herniation of brain stem and cerebellar tonsils owing to the
resulting differential pressure changes
• For communicating hydrocephalus , lumbar puncture is of diagnostic
value( for assessment of CSF contents) and also of therapeutic value
( drainage of typically 10-30 mL of CSF can relieve hydrocephalus
temporarily)
• That is why distinction b/w obstructive and communicating hydrocephalus
is important
Diff b/w obstructive and communicating
hydrocephalus
 OBSTRUCTIVE HYDROCEPHALUS

• AQUEDUCTAL STENOSIS (m/c cause)

• DANDY WALKER MALFORMATION
• ARNOLD CHIARI MALFORMATION (type II)
• VEIN OF GALON MALFORMATION
 Aqueductal stenosis

• Stenosis of aqueduct of sylvius

• Causing less flow of CSF to 4th ventricle
• More CSF accumulates in 3rd and lateral
ventricle causing increase in size of these
ventricles ( ventriculomegaly )
 Dandy walker malformation

• Dilation of posterior fossa due to

cystic enlargement of 4th ventricle
• Causing blockage in CSF flow
thereby more CSF Accumulation
(hydrocephalus )
• Enlarged 4th ventricle compressing
cerebellum present in close
proximity preventing cerebellum to
develop normally causing cerebellar
dysfunctions ( ataxia, dysarthria,
nystagmus)
 Arnold chiari malformation (type II)

• Cerebral tonsils, brain stem , 4th ventricle herniates into cervical

canal
• Causing obstruction in CSF outflow
 Vein of galen malformation
• It is a form of arterio venous malformation
with absence of interconnecting capillaries
between arteries and veins
• Causing high pressure of arteries to transmit
directly to veins therefore increasing venous
pressure
• This increased venous pressure causes
dilation of veins thus compressing
/obstructing CSF pathway
• This increased venous pressure can also
increases load on heart thus may precipitate
heart failure
 COMMUNICATING HYDROCEPHALUS

• NORMAL PRESSURE HYDROCEPHALUS

• IDIOPATHIC INTRACRANIAL HYPERTENSION
• BENIGN EXTERNAL HYDROCEPHALUS
 Normal pressure hydrocephalus
• Type of communicating hydrocephalus seen in
elderly
• Develops in context to previous brain insults:
subarachnoid haemorrhage ,head injury,
meningitis
• CSF pressure at lumbar puncture is typically
normal , but it is believed that reduced brain
compliance in this condition results in transient
spike of ICP that contributes to clinical
deterioration
• Pt presents with gait disturbance, urinary
incontinence, cognitive decline, dementia
• On imaging, ventriculomegaly evident ,
• It is also known in these pt. That the brain parenchyma is less
stiff(more compliant) to allow it to be compressed by developing
ventriculomegaly and thus doesnot result in increased ICP , but
sometimes intermittent increase in ICP has been detected
• Diagnosis is usually a combination of clinical of clinical features
associated with prominent ventricles seen on CT and MRI with no
other abnormalities
• CSF drainage is Done in pt. suspected of normal pressure
hydrocephalus to predict clinical improvement associated with
drainage
• Rx: diversion of CSF by ventriculoperitoneal shunt , or from lumbar
space by lumboperitoneal shunt
 Idiopathic intracranial hypertension
• These pt. Develops raised ICP without an underlying mass lesion
• Classically in young overweight women
• Pt. Presents with High pressure headaches and visual disturbances
• On examination : papilloedema and occasionally cranial nerve palsies
• Lumbar puncture demonstrates raised opening pressure >25 mmhg
• Impaired csf absorption can occur as a result of sinus thrombosis or secondary
to raised intraabdominal pressure in obese patients
• Advice: for weight loss , cessation of certain medications ,including OCPs
• Medical therapy includes acetazolamide to reduce CSF production
• For pt. With visual field loss or visual failure despite medications ,
lumboperitoneal or ventriculoperitoneal shunting is offered
 Benign external hydrocephalus
• Seen exclusively in children
• It occurs due to immature arachnoid villi, which fails to absorb the
required amount of CSF into the blood stream
• Child usually presents with macrocrania with mild delayed
milestones
• CT and MRI shows prominent ventricular system with prominent
subarachnoid spaces
• Usually a self limiting condition corrected by 2 years of age
 INVESTIGATIONS FOR RAISED ICP
• Computed tomography ( first line investigation) can identify
1. Mass lesions causing obstruction
2. Bleeds
3. Cerebral oedema
4. Hydrocephalus
5. Ventriculomegaly : ventricular system dilates proximal to the
obstruction, whereas the CSF pathways distal to the obstruction are
not visualized well

one can infer the level of obstruction from the CT scan but cannot delineate the exact site
or nature of the obstruction.
• In newborn and infants with open fontanelle : cranial USG evaluation

• MRI has been the imaging modality of choice in newly diagnosed hydrocephalus
It helps in diagnosing the exact cause and site of obstruction due to it’s ability to
obtain images in 3 diff planes(coronal, Sagittal, axial)
Small tumours, cysts causing hydrocephalus can be visualized, and when these
are removed hydrocephalus can be relieved
For better visualisation of endoscopic third ventriculostomy used for treatment
of raised ICP and assessing its effectiveness during the follow up

• Gold standard for quantifying ICP and monitoring is by transducing CSF pressure
through external ventricular drain or insertion of a pressure monitor into brain
 TREATMENT OF HYDROCEPHALUS

• Acute obstructive hydrocephalus is an emergency because of risk of

rapid progression to coma and death
• It may be relieved by addressing the underlying pathology which is
obstructing the CSF flow for instance excision of tumour
• Ultimate goal of treatment is to reverse neurological damage caused
by raised ICP, reconstitution of cerebral mantle to allow normal
intellectual development and avoidance of shunt dependency
• Treatment for hydrocephalus includes :
1. Diversion of accumulated CSF by reopening the obstruction
to allow CSF to flow in its natural pathway by endoscopic
aqueductoplasty and excision of tumour causing
hydrocephalus
2. Creation of diversion before the obstruction to let the CSF
drain into the intracranial pathway distal to the block by
endoscopic third ventriculostomy
3. Diversion of CSF into another cavity to have it absorbed
into the blood stream by ventriculoperitoneal shunts
• Imaging studies and invasive procedures like ICP monitoring have not been
able to reliably predict the patients who are likely to develop intellectual
deterioration as a result of hydrocephalus.

• Children younger than 5 years with moderate to severe hydrocephalus

without any symptoms often are considered for a CSF diversion procedure as
it is often difficult to assess the intellectual development in this age group.
Insertion of a shunt in these children allows optimal intellectual development.

• children older than 5 years and adults with asymptomatic ventriculomegaly

often are closely watched with frequent assessment of intellectual
development before a shunt insertion is considered.
• Medical management is often used as a temporary
measure and in conjunction with the surgical
management.
Acetazolamide has been commonly used as it has been
found to reduce the CSF production.
However, the benefits are minimal, and high doses of
the drug causing metabolic acidosis are required to
achieve the effect.
 EXTERNAL VENTRICULAR DRAIN
• Effective temporary method to relieve
hydrocephalus
• Inserted through burr hole at kocher’s point : right
of midline, ant to coronal suture , perpendicular to
the bone surface so that catheter tip rests
adjacent to the foramen of monro in the lateral
ventricle
• Intrathecal antibiotics may also be delivered
through EVD

Alternative means of temporary CSF diversion :

lumbar drains
 VENTRICULOPERITONEAL SHUNTS
• It comprises the insertion of a
proximal/ventricular catheter into the lateral
ventricle while the distal catheter
subcutaneously into the abdomen
• A shunt valve inserted between proximal and
distal catheters regulates flow through the
system by opening at a predetermined
pressure
• Per cutaneous sampling can also be done
from shunt valves as it has CSF reservoir
 SHUNT COMPLICATIONS

• Shunts are vulnerable to :

1. Disconnection
2. Infection
3. Blockage
4. Over drainage
5. Brain injuries

ie why replacement of shunts is required within 3 years In 15-20% cases

• Shunt infection : etiology:- staph. Epidermidis, staph. Aureus ,propoinobacterium
species
symptoms:- fever, headache, meningism , redness along the shunt tube
and purulent discharge from the incision.
Diagnosis:- confirmed by CSF tap from the shunt reservoir or lumbar
Puncture if safe to do so
Treatment:- shunt is removed and external ventricular drainage or
Serial lumbar punctures instituted to cover the course of antibiotic
Therapy.
Once CSF sampling confirms resolution of infection and a normal
Protein conc., a shunt can be inserted at a new site
• Shunt blockage : etiology:- cellular and proteinaceous debris due to infection, choroid plexus adhesions
,blood clots, failure of valve mechanism
Symptoms:- of that of raised ICP
Diagnosis:- CT
• Over drainage : can result in low pressure headaches , typically worse
on standing
• Collapse of ventricles result in accumulation of fluid and blood in sub
Dural space , resulting in subdural hygroma or hematoma
• Slit ventricle syndrome in children treated with shunts is one of the
complication of over drainage as in children ventricles and
subarachanoid space are underdeveloped that results in poor brain
compliance
Therefore, normal fluctuation in ICP are exaggerated so that coughing
and
Straining may cause symptoms of raised ICP
ENDOSCOPIC THIRD VENTRICULOSTOMY
• Especially useful in obstructive hydrocephalus d/t aqueductal
stenosis
• Neuroendoscope inserted into frontal form of lateral ventricle and
then into third ventricle via foramen of monro .
• Floor of the ventricle is then opened allowing free drainage b/w third
ventricle and adjacent subarachanoid cisterns.
• Advantage:- no infection risk as posed by implanting tube
• Complications:-Reblockage of this route is common and many pt.
Subsequently require a shunt.
Damage to basilar artery or forniceal damage
resulting
in permanent memory impairment
 REFERENCES
• Bailey & love short practice of surgery 28th edition
• Sabiston textbook of surgery
• Ghai essential pedistrics