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Excess CSF in the intracranial cavity is
termed hydrocephalus and most cases are caused by obstruction of the flow of CSF.
The term 'hydrocephalus' is used to describe conditions in which there is
increase in the CSF volume within the brain, with expansion of the cerebral
The most common type, which is termed non-communicating
hydrocephalus or obstructive hydrocephalus, is caused by blockage of the CSF
pathway from the ventricles to the sub-arachnoid space. A less common type is
communicating hydrocephalus, in which there is impairment of resorption of CSF
at the arachnoid villi along the dural venous sinuses, usually precipitated by
previous infection or bleeding into the sub-arachnoid space.
Among the main causes of obstructive hydrocephalus is congenital hydrocephalus,
seen in about 1:1000 births. Some cases have stenosis of the aqueduct of Sylvius,
some have associated Arnold-Chiari malformation, and some are inherited as an
Tumours may obstruct the ventricular system, particularly those located in the
brain stem, cerebellum, or pineal region which block the cerebral aqueduct or
Scarring and blockage of the CSF exit foramina at the base of the brain are
common complications of meningitis or sub-arachnoid haemorrhage.
Haemorrhage in the brain or sub-arachnoid space may obstruct CSF drainage
Macroscopically, there is dilatation of the ventricular cavities of the brain
proximal to the site of obstruction. The effects of hydrocephalus depend on the
speed of development of disease and age of the patient.
In acute hydrocephalus, swelling of the brain may be rapid and may cause death
due to cerebral herniation.
In chronic hydrocephalus, signs and symptoms develop slowly and there are
clinical features of raised intracranial pressure. When hydrocephalus develops
in children, before fusion of the skull bones, there is progressive enlargement
of the head circumference. In adults, in whom the skull is rigidly fused,
prolonged disease causes thinning of the skull vault. In the absence of
treatment, long-standing disease causes axonal damage and gliosis in the white
matter. In children this leads to mental subnormality and in adults can lead to
the development of a dementia syndrome, with gait disturbance and incontinence
as prominent features.
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