Hydrocephalus

Dr Yog Raj Khinchi

Definition
It is the dilatation of the ventricular system due to imbalance between production and absorption of CSF with or without increase in CSF pressure CSF is produced by choroid plexus by ultra filtration and active secretion . CSF is absorbed in arachnoid villi. About 20 ml CSF is secreted and absorbed in one hour The turn over of CSF is 3-4 times per day

CSF Production
Produced in the ventricles by the choroid plexus by ultra filtration of the plasma and carbonic anhydrase catalyzed sodium potassium pump Exits the ventricles via the foramina of Magendie and Luschka Enters subarchnoid space goes around the spinal cord and brain. CSF is reabsorbed by the arachnoid villi into the saggital vein Adult produces approximately 500 ml a day (20 ml/hr) CSF volume at one time is approx 150 ml Volume in the ventricles about 25 ml

Ventricular System

CSF pathway
Lateral ventricle Foramen of Monro

3rd ventricle Aqueduct of Sylvius 4th ventricle Foramina of Luschka and Magendie Cisterns Cerebral and spinal sub arachnoid space (Absorbed via arachnoid villi into venous channels)

Hydrocephalus:
Note the marked dilation of the cerebral ventricles

Types and Etiology

Hydrocephalus results due to an imbalance between production and absorption of CSF Two types: - Communicating - Non communicating Communicating : No block in pathway of CSF Non communicating: Block at any level of ventricular system (commonly at aqueductal or foramina of Luschka and Magendie)

 Congenital or Acquired hydrocephalus

Causes: Congenital hydrocephalus

1. Intra uterine infection: like rubella, CMV, toxoplasma
(Inflammation of ventricular ependyma, meninges, basal cistern)

2. Intra cranial hemorrhage 3. Malformations: (i) Aqueductal stenosis (ii) Dandy walker syndrome
(posterior fossa cyst continuous with 4th ventricle)

(iii) Arnold Chiarri malformation

Type 1 Presents in adolescence or adult life Not associated with hydrocephalus Herniation of cerebellar tonsils into cervical canal Type 2 Symptomatic in infancy Progressive hydrocephalus + myelomeningocele Herniation of cerebellum pons and medulla into cervical canal

4. Congenital midline tumours obstructing CSF flow

Causes: Acquired hydrocephalus

Tuberculous and pyogenic meningitis Post-intraventricular hemorrhage Posterior fossa tumours AV malformations, intracranial hemorrhage

Other types of hydrocephalus:

Pan ventricular hydrocephalus Dilatation of lateral, 3rd and 4th ventricles Double compartment hydrocephalus Also called trapped 4th ventricle There is aqueductal stenosis along with obliteration of the outlet of 4th ventricle Unilateral hydrocephalus Due to compression of opposite lateral ventricle Obstruction to foramen of monro Hemi parenchymal atrophy Hydrocephalus ex vacuo Ventricular dilatation associated with cerebral atrophyCSF pressure normal

Pathology
Un even dilatation of ventricles Ependymal lining of ventricles disrupted Peri ventricular ooze Sub ependymal oedema occurs White matter compressed Later Cortical atrophy Process reversible on early treatment

Cut sections at various levels showing dilated ventricles

Clinical features
Enlarging head size and delayed closure of fontanelles and sutures Symptoms Headache, nausea, vomiting, personality and behavior changes such as irritability, head banging, apathy, drowsiness

Signs
Papilloedema, pyramidal tract signs cranial nerve palsies, abnormals skull contour, prominent scalp veins Sunset sign- sclera above cornea becomes visible Limbs become spastic because of stretching of cortical fibres Distortion of brainstem bradycardia, systemic hypertension, altered respiratory rate Large head size at birth causes difficulty in labour. There may be associated malformations

Sunset Sign

Diagnosis
Accurate serial recording of head circumference + supportive evidence by USG early diagnosis If head circumference in the first 3 months of life of over 1 cm every fortnight should arouse suspicion of hydrocephalus Persistent widening of squamo parietal sutures, is not physiological

Diagnosis...
Cranial USG and CT helps evaluate ventricular size, cortical mantle, peri ventricular ooze and etiology of hydrocephalus X ray Sutural separation Erosion of posterior clinoid process Silver beaten appearance ( in convolutional markings )

CT Scan showing Dilated Ventricles

CT head showing dilated ventricles:

CT head showing dilated ventricles:

T1-weighted axial MRI shows markedly dilated lateral ventricles with low signal CSF.

T2-weighted axial MRI shows high signal CSF of the markedly dilated lateral ventricles.

Differential diagnosis of a large head

Megalencephaly No signs of ICT Ventricles normal Eg. Hurlers Disease, Taysachs disease, Metachromatic Leukodystrophy Chronic subdural haematoma Mostly located in parietal area without prominent scalp veins or sunset signs Other causes Hydrencephaly, rickets , achondroplasia, hemolytic anemia, macrocephaly (familial)

Treatment
Depends on Etiological diagnosis Associated malformations Clinical course and severity Arrested hydrocephalus No treatment Medical management If surgery is not indicated Acetazolamide 50 mg/kg/day CSF production in mild slowly progressive hydrocephalus Other drugs frusemide, isosorbide, mannitol

Treatment...
Surgical Rapidly progressive hydrocephalus with threat to life, vision Congenital obstructive hydrocephalus, peri ventricular ooze, acquired hydrocephalus Methods Shunt Choroid plexectomy Third ventriculostomy Ventriculocisternostomy

Treatment...
Shunts
Ventriculoatrial Ventriculo azygous Ventriculo peritoneal Theco peritoneal Ventriculo pleural Ventriculo thoracic ductal

VP Shunt

Complications of shunt
Blockage, infection, peritonitis, endocarditis, arrhythmias Shunt should be kept for entire life Revise the shunt using a longer tube as the child grows Shunt revision if there is infection

Prognosis
Natural history of untreated cases 50% die, 50% survive In survivors 50% have handicaps, 50% normal intelligence Prognosis depends on cause of hydrocephalus and not on the size of cortical mantle at diagnosis Congenital hydrocephalus with spina bifida has poor prognosis