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

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Pathology

Signs & Symptoms
Premature infants
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Infants
Drowsiness, irritability

Older children
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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

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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
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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

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

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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
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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

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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

Treatment of Hydrocephalus diagnosed in utero

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Fetal USG

Outcome & Prognosis
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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

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