Hydrocephalus

Hydrocephalus is a buildup of too much cerebrospinal fluid (CSF) in the brain. The condition can be present at birth or the result of an illness or injury. Cerebrospinal fluid bathes and cushions the brain and spinal cord. It also delivers nutrients and removes waste. The fluid flows through open spaces in the brain called ventricles. When there is too much fluid in the brain, the ventricles grow larger and can put pressure on brain tissue.

There are two kinds of hydrocephalus:

Congenital hydrocephalus is present at birth.
Acquired hydrocephalus occurs sometime after birth and can develop at any age.

Hydrocephalus can be the result of different causes and conditions, including:

Brain hemorrhaging due to complications of prematurity
Brain tumors
Developmental abnormalities
Genetic abnormalities such as aqueductal stenosis (narrowing of a space between two ventricles)
Meningitis
Severe head trauma
Spina bifida

The symptoms of hydrocephalus vary depending on what is causing the condition and the age of your child.

Symptoms of hydrocephalus in infancy include:

Unusually large head size
Downward looking eyes (sundowning)
Irritability
Seizures
Sleepiness
Vomiting

The symptoms of hydrocephalus in older children include:

Headache
Nausea
Vomiting
Blurred or double vision
Downward looking eyes (sundowning)
Sleepiness
Problems with balance and coordination
Developmental delay
Urinary incontinence
Coma

If hydrocephalus develops quickly, your child will become very sick and show the characteristic symptoms. When hydrocephalus occurs over time, children may have headaches, developmental delays and problems with vision. Babies with hydrocephalus who still have an open soft spot may develop an enlarged head.

A similar condition called pseudotumor cerebri also involves increased cerebrospinal fluid in the brain and leads to the same kinds of symptoms that occur with hydrocephalus. If left untreated, pseudotumor cerebri can cause blindness.

Diagnosis of Hydrocephalus

Doctors at Riley at IU Health perform the following exams and tests to diagnose hydrocephalus:

Clinical exam. A neurosurgeon will examine your child to check his or her head size and look for the specific signs of hydrocephalus, such as downcast eyes.
Imaging tests. A neurologist will recommend a computed tomography scan or a magnetic resonance imaging scan to see the tissues and structures of the brain.
Eye exam. If your child has been feeling long-standing head pressure, the doctor may recommend a thorough eye exam, which can detect changes in the eyes.
Lumbar puncture. A sample of cerebrospinal fluid is removed and checked for signs of central nervous system infections such as meningitis.

Treatments

Treatment for hydrocephalus focuses on reducing the amount of cerebrospinal fluid and the resulting pressure around the brain. Treatments include:

Shunt system. A neurosurgeon will surgically place a shunt system inside the skull to drain off extra cerebrospinal fluid. The shunt system typically includes a tube placed into a ventricle, a valve and another tube that carries CSF to other parts of the body (most commonly to the abdomen). This tube may also carry CSF to the heart, lining around the lungs or gallbladder. The valve inside the tube regulates the flow and makes sure fluid flows in only one direction. Shunts are often required for life and are rarely removed. Children with a shunt system can typically enjoy all sports and normal activities.
- Infection risk. Shunts become infected in about 5 percent of cases. The risk for infection is highest during the first year. If an infection does occur, it usually requires multiple surgeries and a few weeks in the hospital to recover.
- Shunt malfunctions. Shunts may fail, become plugged or require regular maintenance to keep the line clear. If the shunt malfunctions, your child will show the symptoms typical of hydrocephalus, plus a fever. Children with a suspected shunt problem should be taken to the emergency room for evaluation. About 50 percent of patients need surgery within two years to fix a problem with a shunt.
- Types of shunts. For a shunt to work, the pressure inside the head must be higher than the pressure inside the valve of the shunt to make the fluid flow. There are two kinds of shunts:
  - Nonprogrammable or fixed pressure shunts. Nonprogrammable or fixed pressure shunts may require surgery to make adjustments to the shunt system.
  - Programmable shunts. Programmable shunts are equipped with a magnetic device used to adjust the pressure level within the valve.
Endoscopic third ventriculostomy (ETV). This procedure is performed for some types of hydrocephalus. A neurosurgeon uses an endoscope (small flexible tube equipped with a camera) to locate the third ventricle and create a hole in the floor. This allows CSF to bypass an obstruction. ETV works well for certain types of hydrocephalus, but not for others. The advantage of ETV is that the hydrocephalus may be effectively and permanently treated without the need for an implanted shunt.

Key Points to Remember

Hydrocephalus occurs when there is too much cerebrospinal fluid around the brain.
The condition can be present at birth or acquired by injury or another condition such as an infection.
Hydrocephalus leads to a large head size in infants and can cause headaches, nausea and problems with vision and balance.
The most common treatment is the placement of a shunt system, which removes extra cerebrospinal fluid and reduces pressure in the brain.
While a shunt is often required for life, it does not limit children from fully participating in normal activities.

Support Services & Resources

Visit the links below to discover support groups and more resources for hydrocephalus.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Hydrocephalus Association

This group provides extensive support for families living with hydrocephalus. The website includes sections for parents, teens and caregivers.

Hydrocephalus Clinical Research Network

This is a multicenter collaborative research group that includes nine children’s hospitals and a centralized data center. The website highlights the latest research efforts and findings as well as clinical trials for hydrocephalus.

Hydrocephalus Research

Pediatric neurosurgeons at Riley at IU Health are involved in research related to treating and managing hydrocephalus.

Locations

In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.