Hydrocephalus

Introduction
 Defined as abnormal accumulation of CSF
in ventricles and/or subarachnoid space,
typically associated with ventricular
dilatation and raised ICP

 Incidence as isolated congenital disorder

1/1000 live births and with spina bifida in
1/1000 live births
 Normal CSF physiology
 Produced by choroid plexus in lateral,third
& fourth ventricles by ultrafiltration at rate
of 0.3 – 0.35 ml/min i.e. 500ml/day

 Average CSF volume is 65 to 140 ml

 Normal CSF pressure is 4-5cms of water

in infants, 4-10cms in older children &
15cms in adults
CSF flow
 Classification
On location of block
 Communicating
 Non communicating

On cause
 Physiologic – due to overproduction by CP
papilloma
 Nonphysiological – due to any other cause
Pathology
 Signs & Symptoms
Premature Infants Older children
infants
Drowsiness, irritability  Headache
 Apnea
Vomiting  Vomiting
 Bradycardia
Macrocephaly, tense  Lethargy
 Tense AF fontanelle
 Diplopia, blurred
 Rapid head Frontal bossing vision
growth
Distended scalp veins  Papilledema
 Globoid head ,Lateral rectus palsy
Poor head control
 Hyperreflexia,
Lateral rectus palsy, sun set clonus
sign
 Signs & Symptoms in adults

 progressive headache
 vomiting
 progressive dementia
 epileptic fits
 urinary incontinence
 limb weakness
 papilloedema
 Investigations
Goal of investigations:
 To confirm diagnosis

 Differentiating between communicating

and non communicating

 To know site of obstruction

 To know anatomical detail

 For follow up
 Head circumference
 35 – 37 cms at birth

Increases at rate of
 2cm/ mth for 1st 3 mths
 1cm/mth for next 3 mths
 0.5cm/mth for the next 6 mths
 CSF examination
 Lumbar puncture should be done with
care as coning can occur in non
communicating hydrocephalus

 Pyogenic meningitis, TBM, and

intraventricular bleed can be diagnosed
 Radiological investigations

X RAY SKULL
 Widening of sutures

 Silver beaten appearance

 Enlargement of pituitary fossa with erosion of

dorsal sella

 Shallow posterior fossa

 Ultrasonography

 Non invasive, no exposure to radiation

 Can show lateral & third ventricle but not 4th ventricle or
subarachnoid space

 Can measure resistive index which is a sensitive

indicator

 atrial size most useful measurement of ventricular size

 Ventriculohemispheral ratio more than 35% indicates

ventriculomegaly
 CT scan
 Provide greater anatomical detail

 Can distinguish between communicating and

non communicating

 With IV contrast tumours / abscess/ bleed/ Ca

deposit can be seen

 Provides only axial image

 Inferior to MRI for visualization of brain

stem/posterior fossa
CT scan
 Magnetic resonance imaging

 Provide greatest amount of anatomic detail

 Differentiate between subdural effusion & enlarge sub

arachnoidal spaces

 Visualization of posterior fossa and brain stem

 Cine MRI is useful to identify site of obstruction

Magnetic resonance imaging
 Medical Management

 Mannitol decreases ICP

 Loop diuretics, Acetazolamide decrease CSF

production for a few days

 Doesn't resolve ventriculomegaly or affect

intellectual outcome
 Surgical treatment
 Shunt surgeries

 Third Ventriculostomy

 Choroid plexectomies/ coagulation

 Shunt surgery
 Ventriculoperitoneal shunt – most
commonly done

 Ventriculoatrial shunt
 Ventriculopleural shunt
 Ventriculogallbladder shunt
 Lumboperitoneal shunt
 VP shunt classification
 According to type of valve
- spring ball
- slit valve
- diaphragm

 According to pressure of opening

- ultra low pressure
- low
- medium (most commonly used)
- high
 VP Shunt - Indications
In newborn and children:
 Idiopathic hydrocephalus
 Communicating / obstructive hydrocephalus
 Myelodysplactic children with healing wound under tension
 Signs and symptoms of brain stem compression develop in
presence of ventriculomegaly

In adults
 Signs of elevation of ICP in high pressure hydrocephalus
 Signs of brain herniation
 Progressive dementia, gait and urinary disturbance
 Arachnoid, porencephalic cyst
 Spontaneous/ iatrogenic CSF leakage
 Temporary neutralization of elevated ICP in tumours
 VP shunt
Contraindications
Absolute
 Infection specifically ventriculitis
 Intraventricular hemorrhage
 Recent peritonitis, Adhesions

Relative
 Arrested or atrophic hydrocephalus
 Pending abdominal surgery
 Lumbar Peritoneal Shunt
Indications
 Communicating hydrocephalus with or without small or collapsed
ventricular system

Advantages
 Extracranial course
 Avoid complication of IIIrd ventriculostomy

Contraindication
 Obstructive hydrocephalus

Complication
 Overdrainage (spinal headache)- most common)
 Transient root symptom and sign
 Scoliosis / hyper lordosis / kyphoscoliosis – rare
 Complications of Shunt surgery
 Three main groups
2. Mechanical failure – proximal, valve or distal

4. Infection – mainly by staph. Epidermidis &

aureus

6. Overdrainage – causing headache

 Endoscopic III Ventriculostomy
Criteria
 Obstructive hydrocephalus
 Dilated III ventricle defined as > 1 cm in by coronal plane
 Floor of the 3rd ventricle suitable for fenestration i.e., attenuated or
bulging downward into interpeduncular cistern.

Indication
 Posterior fossa tumor

 Late onset (over 24 yrs of age) aqueduct block such as tectal tumor

 New born with myelomeningocele and associated blockage either at

aqueductal or exists of the 4th ventricle

 In the patient with the repeated shunt failure

 Endoscopic III Ventriculostomy

Contraindication
 Chronic meningitis
 Sub dural haemorrhage / intra ventricular haemorrhage

Complications
 Infection
 Bleeding from basilar artery can cause death
 Hemiparesis, owing to damage to pedicle or its
perforating arteries
 Hypothalmic damage due to proximity to III ventricle
 Treatment of Hydrocephalus
diagnosed in utero
 Can cause cephalopelvic disproportion & inhibit labour

 USG used for diagnosis

 MRI after engagement of head used to visualise cerebral

morphology

 Severe brain malformation treated by cephalocentesis

 Results of ventriculoamniotic shunts discouraging

 Babies with normal cerebral morphology delivered by

LSCS when maturity documented & treated by shunt
surgery
Fetal USG
 Outcome & Prognosis
 Regular follow up essential

 Baseline scan post shunt for ventricular size

 Prognosis depends on brain morphology & factors like

perinatal ischemia, IVH, ventriculitis

 Number of shunt revisions / malfunctions not key factors

in outcome

 Cause of death in these pts is primary disease

progression or factors related neither to hydrocephalus
nor its treatment