Hydrocephalus, also known years ago as “water on the brain”, is a condition where the circulation system of the body’s cerebral spinal fluid (CSF) is not functioning properly. The CSF accumulates in the brain and causes intracranial pressure. A shunt is usually placed to equalize the flow of CSF, which requires surgery. The diagnosis and surgery can be very frightening for the parents as well as the child. It is important to find a Pediatric Neurosurgeon that you feel comfortable with, and that you trust. If you have time and a choice, it would be a benefit to interview the Neurosurgeons to find the one that is highly skilled, knowledgeable about hydrocephalus and willing to communicate with you.
The Hydrocephalus Association recommends a book that was published in 1999 that covers all issues concerning Hydrocephalus in a caring, understandable format. The book is called Hydrocephalus: A Guide for Patients, Families & Friends by Chuck Toporek & Kellie Robinson.
Hydrocephalus : A Guide for Patients, Families, and Friends by Chuck Toporek, Kellie Robinson, Linda Lamb (Editor) (April 1999)
Fact Sheet from the National Institute of Neurological Disorders and Stroke Hydrocephalus
What is hydrocephalus?
The balance between production and absorption of CSF is critically important. Because CSF is made continuously, medical conditions that block its normal flow or absorption will result in an over-accumulation of CSF. The resulting pressure of the fluid against brain tissue is what causes hydrocephalus.
What are the different types of hydrocephalus?
Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain. In these cases, brain tissue may actually shrink. NPH is an abnormal increase of cerebrospinal fluid in the brain’s ventricles that may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop NPH when none of these factors are present. An estimated 375,000 older Americans have NPH.
Who gets this disorder?
The number of people who develop hydrocephalus or who are currently living with it is difficult to establish since the condition occurs in children and adults, and can develop later in life. A 2008 data review by the University of Utah found that, in 2003, hydrocephalus accounted for 0.6 percent of all pediatric hospital admissions in the United States. Some estimates report one to two of every 1,000 babies are born with hydrocephalus.
What causes hydrocephalus?
The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) 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, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF within the cisterns.
What are the symptoms?
Symptoms of normal pressure hydrocephalus include problems with walking, impaired bladder control leading to urinary frequency and/or incontinence, and progressive mental impairment and dementia. An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel “stuck.” Because some of these symptoms may also be experienced in other disorders such as Alzheimer’s disease, Parkinson’s disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is often incorrectly diagnosed and never properly treated. Doctors may use a variety of tests, including brain scans such as computed tomography (CT) and magnetic resonance imaging (MRI), a spinal tap or lumbar catheter, intracranial pressure monitoring, and neuropsychological tests, to help them accurately diagnose normal pressure hydrocephalus and rule out any other conditions.
The symptoms described in this section account for the most typical ways in which progressive hydrocephalus is noticeable, but it is important to remember that symptoms vary significantly from person to person.
How is hydrocephalus diagnosed?
Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, CT, MRI, or pressure-monitoring techniques. A physician selects the appropriate diagnostic tool based on an individual’s age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.
What is the current treatment?
Hydrocephalus is most often treated by surgically inserting a shunt system. This system diverts the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process.
A shunt is a flexible but sturdy plastic tube. A shunt system consists of the shunt, a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites in the body such as a chamber of the heart or areas around the lung where the CSF can drain and be absorbed. A valve located along the catheter maintains one-way flow and regulates the rate of CSF flow.
A limited number of individuals can be treated with an alternative procedure called third ventriculostomy. In this procedure, a neuroendoscope — a small camera that uses fiber optic technology to visualize small and difficult to reach surgical areas — allows a doctor to view the ventricular surface. Once the scope is guided into position, a small tool makes a tiny hole in the floor of the third ventricle, which allows the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain.
What are the possible complications of a shunt system?
What is the prognosis?
What research is being done?
Where can I get more information?
For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute’s Brain Resources and Information Network (BRAIN) at:
P.O. Box 5801
Bethesda, MD 20824