HYDROCEPHALUS What is hydrocephalus? “The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. As its name implies, it is a condition in which the primary characteristic is excessive accumulation of fluid in the brain. Although hydrocephalus was once known as "water on the brain," the "water" is actually … a clear fluid surrounding the brain and spinal cord. The excessive accumulation of CSF results … causes potentially harmful pressure on the tissues of the brain.

What are the different types of hydrocephalus?
‘Hydrocephalus may be … present at birth (congenital).. or at some point afterward (acquired).” Hydrocephalus that occurs later in life (after birth) can affect individuals of all ages and may be caused by injury or disease.

Hydrocephalus can occur as part of many different disorders. These include:

Dandy Walker Syndrome

Spina Bifida

Who gets this disorder?
Hydrocephalus is believed to affect approximately 1 in every 500 children. Most of these cases are diagnosed prenatally, at the time of delivery, or in early childhood.

What are the symptoms?
Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to CSF. …In infancy, the most obvious sign of hydrocephalus is often the rapid increase in head circumference or an unusually large head size. Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures.

Older children and adults may experience different symptoms because their skulls cannot expand to accommodate the buildup of CSF. In older children or adults, symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is part of the optic nerve), blurred vision, diplopia (double vision), sunsetting of the eyes, problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of development, lethargy, drowsiness, irritability, or other changes in personality or cognition including memory loss.

The symptoms described in this section account for the most typical signs of hydrocephalus; it is, however, important to remember that symptoms vary significantly from individual to individual.

What is the current treatment?

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 safely absorbed.

A shunt is a flexible but sturdy silicone rubber tube. A shunt system consists of the shunt, a catheter, and a valve. One end of the catheter is placed in the CNS - most usually inside the brain. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites within the body. A valve located along the catheter maintains one-way flow and regulates the rate of CSF flow.

What are the possible complications of a shunt system?

Shunt systems are not perfect devices. Complications may include mechanical failure, infections, obstructions, and the need to lengthen or replace the catheter. Generally, shunt systems require monitoring and regular medical followup. When complications do occur, usually the shunt system will require some type of revision.

Some complications can lead to other problems such as overdraining or underdraining. Overdraining occurs when the shunt allows CSF to drain from the ventricles more quickly than it is produced. This overdraining can cause the ventricles to collapse, tearing blood vessels and causing headache, hemorrhage, or slit-like ventricles (slit ventricle syndrome). Underdraining occurs when CSF is not removed quickly enough and the symptoms of hydrocephalus recur (see "What are the symptoms of hydrocephalus?").

In addition to the common symptoms of hydrocephalus, infections from a shunt may also produce symptoms such as fever, soreness of the neck or shoulder muscles, and redness or tenderness along the shunt tract. When there is reason to suspect that a shunt system is not functioning properly (for example, if the symptoms of hydrocephalus return), medical attention should be sought immediately.

What is the prognosis?
The prognosis for patients diagnosed with hydrocephalus is difficult to predict.
Hydrocephalus poses risks to both mental and physical development. However, many children diagnosed with the disorder benefit from therapy and education, and go on to lead normal lives with few limitations. Treatment by a team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome.

Treatment of patients with hydrocephalus is life-saving and life-sustaining. Left untreated, progressive hydrocephalus is, with rare exceptions, fatal.

The National Institute on Neurological Diseases and Stroke provides a lot of detailed medical information, including how hydrocephalus is diagnosed and treated medically.

The National Library of Medicine Page on Hydrocephalus is more of a layperson's view, including discussion of coping, and what is neurosurgery.

The Hydrocephalus Association provides support and education for people with hydrocephalus and their families. This site doesn't have as much information as the other two sites above, but you will find the most down-to-earth information here.

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