Hydrocephalus

Hydrocephalus is a congenital or acquired condition characterised by marked dilatation of the cerebral ventricles, accompanied by the accumulation of cerebrospinal fluid within the skull. More commonly it results from impaired absorption of CSF than from excessive secretion. Hydrocephalus may be a result of an obstruction outside the ventricular system or an intraventricular blockage; termed communicating and non-communicating hydrocephalus respectively. Another condition, hydrocephalus ex vacuo, manifests as enlarged ventricles because of destruction of cerebral tissue and the consequent expansion of the ventricles.

types of hydrocephalus
The types of hydrocephalus can be divided into: Major categories:

communicating non-communicating

Specific forms:

normal pressure ex vacuo otitic

hydrocephalus (communicating)
Communicating hydrocephalus is caused by obstruction to CSF flow from outside the ventricular system, usually in the subarachnoid space. All the ventricles show a generalised dilatation on a CT scan. It is safe to perform a lumbar puncture since the increased intracranial pressure is evenly distributed throughout the subarachnoid space.

hydrocephalus (non-communicating)
Non-communicating hydrocephalus results from obstruction to CSF flow within the ventricular system. Fluid accumulates proximal to the site of the blockage causing dilation. Complete blockage is untenable. The aqueduct is the most narrow part of the normal CSF pathway and that most likely to become obstructed - the third and lateral ventricles will be dilated, the fourth will remain small. All four ventricles are dilated when obstruction occurs at the level of the foramina of Luschka and Magendie, or the basal cisterns; causes of this include:

congenital: Dandy Walker and Arnold Chiari malformations acquired - meningeal scarring due to meningitis, subarachnoid haemorrhage and cranial trauma

normal pressure hydrocephalus

Normal pressure hydrocephalus is a form of communicating hydrocephalus in which the intracranial pressure, as measured by lumbar puncture, is normal or intermittently raised. Failure to reabsorb CSF is compensated by reduced production.

hydrocephalus ex vacuo
Hydrocephalus ex vacuo is not a pressure phenomenon. It signifies compensatory ventricular expansion secondary to cerebral atrophy and shrinkage, for example, in Alzheimer's disease and in Pick's disease. Shunts are not useful in the management of these patients and carry a substantial risk of subdural haemorrhage if implanted.

hydrocephalus(otitic)
Otitic hydrocephalus is an abnormal increase in the amount of CSF within the brain secondary to otitis. It is characterised by headache and severe papilloedema and results from aseptic thrombosis of the lateral or superior sagittal sinus causing impaired CSF absorption. The condition is usually self-limiting. There is complete recovery provided there is no associated venous cortical infarction and vision is preserved.

aetiology
The aetiology can be considered in terms of the type of hydrocephalus.

aetiology
Communicating hydrocephalus most commonly results from deficient reabsorption of CSF absorption due to thickening of the leptomeninges - pia-mater and arachnoid - and / or damage to the arachnoid granulations. Causes include:

post-meningitis: o bacterial - pneumococcus more than meningococcus o tuberculosis o fungal subarachnoid haemorrhage - 80% of cases develop some degree of hydrocephalus trauma neoplastic infiltration in the subarachnoid space dural sinus thrombosis - not generally considered as a major cause of hydrocephalus

Less common mechanisms include:

increased CSF viscosity - high protein content excess CSF production - choroid plexus papilloma

aetiology of non-communicating hydrocephalus

Classification: Congenital:

atresia, stenosis, forking, or gliosis of the aqueduct of Sylvius atresia of foramina of Magendie or Luschka - the Dandy-Walker syndrome obstruction of 4th ventricular outflow - Arnold Chiari malformation aneurysm of vein of Galen

Acquired:

aqueduct stenosis - due to adhesions following infection or haemorrhage supratentorial masses causing tentorial herniation intraventricular haematoma tumours: o intraventricular - meningioma, colloid cysts, ependymona o parenchymal - pineal region, posterior fossa abscesses and granuloma arachnoid cysts cerebellar haemorrhage

aetiology of normal pressure hydrocephalus

Commonly, normal pressure hydrocephalus follows:

meningitis subarachnoid haemorrhage severe head injury radiotherapy

Frequently, no predisposing cause is found.

clinical features
The presentation of hydrocephalus will vary according to the age of the patient and the cause. Massive head enlargement is a feature of infantile hydrocephalus. Raised intracranial pressure is symptomatic of an acute cause; dementia indicates a gradual onset.

clinical features of infantile hydrocephalus

Clinical features of infantile hydrocephalus include:

characteristic macrocephaly - enlargement before birth may obstruct labour. Usually, it is slowly progressive and the head may attain a huge size with a circumference of 30 inches or more. Enlargement occurs in all diameters. The cranial sutures separate to accommodate the expanding ventricles so that signs of raised intracranial pressure are not an immediate feature. o the anterior fontanelle is much enlarged and tense o in extreme cases, the head may be translucent and yield a fluid thrill on percussion o scalp veins are prominent the lids are retracted, the eyes are depressed forwards and downwards, and upward gaze is impaired - the setting sun appearance. convulsions and mental impairment are common there is often optic atrophy, under-development of the limbs, and spastic diplegia of varying severity.

clinical features of adult hydrocephalus

The presentation of hydrocephalus in adults is variable and dependent on the type and the underlying aetiology. Features of an acute onset include:

features of raised intracranial pressure - headache, vomiting, papilloedema and impaired conscious level impaired upward gaze

Features of a gradual onset are similar to those seen in normal pressure hydrocephalus. These develop progressively and include:

mental impairment gait apraxia; plantar reflexes often extensor; tendon jerks exaggerated or diminished

urinary incontinence due to sphincter dysfunction hypopituitarism due to enlargement of the third ventricle into the posterior fossa

investigations
A minimal series of investigations in hydrocephalus includes:

skull X ray: o assess skull size, suture diastasis; may be obvious in infants o look for evidence of chronic raised pressure - erosion of the posterior clinoids o look for associated defects - e.g. basilar invagination CT scan including contrast - reveals pattern of ventricular enlargement and, frequently, the site and cause of obstruction: o generalised dilatation - suggests communicating hydrocephalus o lateral and 3rd ventricles dilated: 4th ventricle normal - suggests aqueduct stenosis 4th ventricle absent or deviated - suggests a posterior fossa mass ultrasonography through the anterior fontanelle in infants - demonstrates ventricular enlargement but is less informative than CT

management
The cause of hydrocephalus should be eliminated if possible - e.g. colloid cysts of the 3rd ventricle, intraventricular meningioma, other obstructive causes. Otherwise, relieve pressure by shunting, or if the patient is rapidly deteriorating, by draining the ventricle directly. Endoscopic third ventriculostomy has become a more recent, and important, treatment option for occlusive hydrocephalus associated with aqueductal stenosis or space-occupying lesions of or around the posterior third ventricle and upper brainstem (1,2):

this procedure appears to be more successful in adults than in young children it is efficacious in both previously shunted and non shunted patient complication and mortality rates compare favorably with those for shunts has also been increasingly used as an alternative treatment option for shunt complications

Lumbar puncture may be used to relieve pressure in an acute communicating hydrocephalus.

Reference: 1. Buxton N et al. Neuroendoscopic third ventriculostomy for hydrocephalus in adults: report of a single unit's experience with 63 cases. Surg Neurol 2001;55:p. 7478. 2. Boschert J et al. Endoscopic third ventriculostomy for shunt dysfunction in occlusive hydrocephalus: long-term follow-up and review. J Neurosurg 2003;98 :10321039

prognosis
The prognosis of hydrocephalus varies according to whether the condition is congenital, or has been acquired:

untreated congenital hydrocephalus is often fatal within the first four years of life; rarely, the disorder arrests and a state of equilibrium is reached between the formation and absorption of CSF - varying degrees of mental deficiency, blindness, and epilepsy may occur long-term outcome of acquired hydrocephalus varies according to the cause and management