III. HYDROCEPHALUS

A. General comments.


As mentioned previously, hydrocephalus and disorders related to defective closure of the neural tube (such as anencephaly and midline defects) are the most common central nervous system malformations.

Brief anatomical summary related to hydrocephalus and CSF flow: The forebrain includes the telecephalon which encloses the lateral ventricles and the diencephalon which encloses the third ventricle. The brainstem includes the midbrain which encloses the aqueduct of Sylvius and the hindbrain which encloses the fourth ventricle. After the fourth month of gestation, the foramina of Luschka (lateral) and Magendie (midbrain) open and CSF produced in the choroid plexus then flows into the subarachnoid space and is resorbed by the arachnoid granulations. Normally, dye introduced into the lateral ventricles can be removed from the lumbar subarachnoid space.

Hydrocephalus involves enlargement of all or part of the ventricular system due to production of CSF at a rate greater than the rate of removal. Usually this condition is associated with an increase in intraventricular pressure, but, rarely, "low pressure hydrocephalus" occurs in elderly patients who exhibit neurological signs. Hydrocephalus is associated with many inherited syndromes.

Clinically, the presence of hydrocephalus in the infant can be ascertained by analyzing head and fontanelle size and by evaluating skull x-rays and CAT scans. Treatment basically consists of shunting the cerebrospinal fluid directly from some point in the ventricular system, usually the lateral ventricle, to some other point beyond the obstruction (usually the jugular vein, right atrium or peritoneum). In children, prompt treatment of hydrocephalus uncomplicated by severe spina bifida allows about half of the children to compete successfully with their peers. Followup at six month intervals is mandatory. Infection is the most common and serious complication which mitigates against a higher percentage of successfully treated patients.

There are two major classes of hydrocephalus: noncommunicating and communicating. In non-communicating hydrocephalus, fluid in the lateral ventricle does not 'communicate' with the lumbar subarachnoid space because there is an occlusion at the foramen of Monro, aqueduct of Sylvius, or foramina of Luschka and Magendie. In communicating hydrocephalus, on the other hand, fluid from the lateral ventricles can be recovered from the lumbar subarachnoid space because there is no occlusion . Commonly, an obstruction occurs in the subarachnoid space.



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