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

Infants
Drowsiness, irritability

Older children

Headache Vomiting Lethargy Diplopia, blurred vision Papilledema ,Lateral rectus palsy Hyperreflexia, clonus

Apnea Vomiting

Bradycardia
Tense AF Rapid head growth Globoid head

Macrocephaly, tense fontanelle

Frontal bossing

Distended scalp veins

Poor head control Lateral rectus palsy, sun set 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

1.

Three main groups Mechanical failure proximal, valve or distal Infection mainly by staph. Epidermidis & aureus Overdrainage causing headache

2.

3.

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