Hydrocephalus is a Greek word that means, waterhead. Hydrocephalus is an abnormal build up of cerebrospinal fluid (CSF) in the brain that causes ventricles to enlarge and the pressure inside the head to increase. Hydrocephalus occurs when there is an imbalance in the amount of CSF being produced and absorbed.

• enlarged head (at birth or shortly after)
• prominent scalp veins
• "sunsetting" of eyes (downward look)
• large or tense fontanel
• fever, irritability or sleepiness

Hydrocephalus is one of the most common "birth defects" and afflicts more than 10,000 babies each year. One out of every 500 births are affected by hydrocephalus.

CSF is produced in the ventricles, circulates through them and is then absorbed into the bloodstream. This cycle functions to maintain a protective environment to the nervous system. Congenital hydrocephalus is thought to be caused by an interaction of environmental and genetic factors. The development of hydrocephalus has also been associated with intercranial bleeding, cysts, head trauma, infections and tumors.

Diagnosis: Depending on the age of the child at discovery, various techniques can be used to confirm diagnosis. An ultrasound of the brain is often used during the first 6-12 months of life. After the skull has fused a better diagnosis can be made with MRI or other brain imaging techniques.

Treatment: Hydrocephalus is most commonly treated with the surgical insertion of a flexible tube called a shunt. The shunt is placed in the ventricular system of the brain to divert the flow of CSF into another region of the body, such as the abdominal cavity, chest cavity, or heart. Shunts usually have some type of valve system that controls and maintains the CSF at normal pressures. Shunts will need to be replaced as the child grows. Shunts can also malfunction and become infected leading to more surgery for shunt replacement.

Another option is a procedure called a ventriculostomy which is making a tiny hole in the ventricle to reestablish CSF flow. Only certain types of hydrocephalus can be treated this way, but the procedure is becoming more popular due to better imaging techniques and surgical instruments.

Associated Problems: About 80% of babies with hydrocephalus are born with other defects.

• Arachnoid Cysts
• Brain Injury
• Dandy-Walker Syndrome
• Head Trauma - Tumors
• Heart Disease
• Learning Disabilities
• Meningitis
• Porencephaly
• Spina Bifida

Learning disabilities are among the most common complications for people with hydrocephalus. Intelligence levels can range from mild to severe mental retardation. While surgery can correct the CSF balance, any associated brain damage is irreversible. Some children may have difficulty with memory retention but they can still be taught by using different learning strategies.

Motor disabilities affect 75% of children with hydrocephalus. While children can participate in some physical activities, they should stay away from sports that require the use of a helmet.

Visual impairments, particularly related to depth reception, are present in 25-35% of children with hydrocephalus.

Prognosis: The outcome for most children with hydrocephalus is optimistic. With shunt treatment, 80% of infants will reach five years of age and of those survivors, 80% will have normal intelligence.

The Association of Birth Defect Children
930 Woodcock Road, Suite 225
Orlando, FL 32803
1-800-313-ABDC (2232)
Phone: (407) 245-7035
Fax: (407) 895-0824 www.birthdefects.org

HEALTHY (Hydrocehalics Encouraging Active Lives Through Helping Yourself)
434 Cardinal Lane
Bedminster, NJ 07921
(908) 781-2519

Hydrocephalus Association
870 Market Street, Suite 955
San Francisco, CA 94102
(415)-776-4713

Association for Children and Adults with Learning Disabilities (ACLD)
4156 Library Road
Pittsburgh, PA 15234
(4210-341-1515

National Hydrocephalus Foundation
22427 South River Road
Joliet, IL 60436
(815) 467-6548

Vestibular Disorders Association
P.O. Box 4467
Portland, OR 97208
Phone: (503) 229-7705
Fax: (503) 228-8064