Hydrocephalus

Condition Description

Hydrocephalus (HY-dro-SEF-ah-lus) is not a specific disease but a congenital or acquired condition with multiple causes resulting in impaired cerebral spinal fluid (CSF) circulation, absorption, or rarely production. This causes a build-up of spinal fluid within the brain. Congenital hydrocephalus is present at birth and may be caused by either events or influences that occur during fetal development, or genetic abnormalities. 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. When the flow of CSF is blocked, the increased pressure causes symptoms that can damage delicate brain tissue. Babies with hydrocephalus typically have a rapidly increasing head size, with open spaces between the bones of the skull (the cranial sutures and fontanelles) and a prominent forehead. Because of the increased pressure in the brain, they will show signs of increasing intracranial pressure which are: irritability, nausea, vomiting, headache, feeding difficulties, and even spasticity or clonus (tremor of the foot when suddenly flexed). Other symptoms can occur, depending on the underlying cause of the hydrocephalus. Surgical treatment for hydrocephalus can restore and maintain normal cerebrospinal fluid levels in the brain. A variety of interventions are often required to manage symptoms or functional impairments resulting from hydrocephalus. 

• Hydrocephalus occurs in about 1 of every 500 children, though the rate is hard to calculate because it occurs as a complication of so many different conditions. About 22 babies are born with hydrocephalus without spina bifida every year in Minnesota.

Parental education and support are essential, and local, regional, and national organizations may be very helpful.

Condition specific organizations

• Hydrocephalus Association
• Complex Child
• Hydrocephalus | National Institute of Neurological Disorders and Stroke