Hydrocephalus

By Dr. Rasha Rashid Moderator Dr. Afaf Al-areeny

Hydrocephalus
Is defined by as a disproportionate increase in the amount of CSF within The cranium, usually in association with a rise in ICP that result from Impaired circulation and absorption of CSF, or in the rare circumstances From increased production by a choriod plexus papilloma

Physiology & Circulation of CSF

The total volume of CSF approximate 50 ml in an infant and 150 ml in an adult. Probably two thirds or more of this fluid originates as a secretion from the choriod plexuses in the four ventricles, mainly in the two lateral ventricles.

Additional amounts of fluid are secreted by all the ependymal surfaces of the ventricles and the arachnoid membranes, and a small amount comes from the brain itself through the perivascular spaces that surround the blood vessels entering the brain.
Production of CSF in regulated by the hemeostatic environment , in response to alterations in CSF pressure and neurogenically.

The fluid flow from the choriod plexuses and then through the CSF System. The fluid secreted in the lateral ventricles and the third ventricle passes along the aqueduct of Sylvius into the forth ventricle, where a small amount of additional fluid is added. It then passes out of the forth ventricle through three small openings, two lateral foramina of Luschka and a midline foramin of Magendie, entering the cisterna magna, a large fluid space that lies behind the medulla and beneath the cerebellum. The cisterna magna is continuous with the subarachnoid space that surrounds the entire brain and spinal cord. Almost all the CSF then flows upward from the cisterna magna through the subarachnoid space surrounding the cerebrum. From here the fluid flows into multiple arachnoidal villi that project into the large sagittal venous sinus and other venous sinuses of the cerebrum.
Finally the fluid empties into the venous blood through the surfaces of these villi.

Types of Hydrocephalus
1. Obstructive or non communicating hydrocephalus. 2. Non-obstructive or communicating hydrocephalus.

Pathophysiology and Etiology

1. Obstructive of non communicating hydrocephalus

Aqueductal stenosis . Aqueductal gliosis. Intrauterine viral infections . Lesions or malformations of the posterior fossa.
Vein of Galen malformation.

Chiari malformation and the Dandy-Walker syndrome.

2. Nonobstructive

or communicating hydrocephalus.

Most commonly follows a subarachnoid hemorrhage.

Pnumococcal and tubrculous meningitis .

Leukemic infiltrates .

Clinical Manifestations
The clinical presentation of hydrocephalus is variable and depends on many factors, including : The age at onset. The nature of the lesion causing obstruction. The duration and rate of rise of the ICP.
The clinical manifestation of hydrocephalus are caused by ventricular distention and increased ICP.

Ventricular distention : Dilation of the lateral ventricles results in stretching of the Corticopontocerebellar and corticospinal pathways, which sweep around the lateral margins of these ventricles to reach the cerebral peduncles. ICP: Manifestations of increased ICP may evolve slowly in obstructive hydrocephalus when there is time for tarnsependymal absorption of CSF to compensate partially for the obstruction, or they may evolve acutely when compensation is absent.

Manifestations According to Age

a. In an infant. b. In an older child.

Physical Examination
Serial measurements of the head circumference indicate an increased velocity of growth. Pircussion of the skull may produce a (cracked-pot) or (Macewen sign), indicating separation of the sutures. A foreshortened occiput suggests Chiari malformation, and a prominent occiput suggests the Dandy-Walker malformation. Papilledema, abducent nerve palsy, and pyramidal tract signs which are most evident in the lower extremities are apparent in most cases.

Chiari Malformation
Consists of two major subgroups: 1. Type I Produces symptoms during adolescence or adult life and is usually not associated with hydrocephalus.

The deformity consists of displacement of the cerebellar tonsils into the cervical canal.

Chiari Malformation
2. Type II

Is characterized by progressive hydrocephalus and a myelomeningocele. This lesion represents an anomaly of the hindbrain, probably due to a failure of pontine flexure during embryogenesis, and result in elongation of the forth ventricle and kinking of the brain stem, with displacement of the inferior vermis, pons, and medulla into the cervical canal.

The Dandy-Walker Malformation

of a cystic expansion of the forth ventrical in the posterior fossa, which results from a developmental failure of the roof of the forth ventrical during embryogenesis . Approximately 90% of patients have hydrocephalus, and a significant number of children have associated anomalies, including agenesys of the posterior cerebellar vermis and a corpus callosum.
Consists

Diagnosis
History Familial cases . Past history. Neurofibromatosis.

Physical examination Inspection. Palpation. Auscultation.

Investigation Plain skull films.

CT and MRI.

Differential Diagnosis
1. The head may appear enlarged secondary to a thickened cranium. 2. Chronic subdural collections.

3. Various metabolic and degenerative disorders of the CNS. 4. Cerebral gigantism and neurofibromatosis.
5. Familial megalencphaly. 6. Hydranencephaly.

Treatment
Therapy for hydrocephalus depends on the cause. 1. Medical management. 2. Surgical management.
Prognosis This depends on the cause of the dilated ventricles.

Internal Hydrocephalus: Due to an obstruction in the aqueduct of Monro ,it cannot be shunted and baby dies External Hydrocephalus: Due to an obstruction in the aqueduct of Sylvius, it can be shunted. Hydrocephalus Ex-Vaceu Due to an increase in the CSF production to compensate the brain atrophy.