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The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. It is therefore a condition in which the primary characteristic is excessive accumulation of fluid in the brain. The fluid is actually cerebrospinal fluid, a clear liquid surrounding the brain and spinal cord. The excessive accumulation of this fluid results in an abnormal dilation of the spaces in the brain called ventricles. This dilation causes potentially harmful pressure on the tissues of the brain.

Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at birth, and may be caused by either environmental influences during fetal development or genetic predisposition. Acquired hydrocephalus develops at the time of birth or at some point afterward. This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.

Associated Features:


Differential Diagnosis:

Some disorders have similar symptoms. The clinician, therefore, in his diagnostic attempt has to differentiate against the following disorders which need to be ruled out to establish a precise diagnosis.



The causes of hydrocephalus are not all well understood. Hydrocephalus may result from genetic inheritance or developmental disorders such as those associated with neural tube defects including spina bifida and encephalocele. Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage blocking the exit from the ventricles to the cisterns and eliminating the cisterns themselves.


Hydrocephalus is most often treated with the surgical placement of a shunt system. This system diverts the flow of CSF from a site within the central nervous system (CNS) to another area of the body where it can be absorbed as part of the circulatory process.


DSM Code

None Identified

None Identified

Disorder Sheets

Association for Spina Bifida and Hydrocephalus
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Tel: +448454507755
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Recommended Book

Hydrocephalus: a Guide for Patients, Families and Friends - Click Here to View


Misc Information

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