Hydrocephalus, Pediatric

Hydrocephalus is a condition in which there is too much fluid (cerebrospinal fluid or CSF) around the brain. A child can be born with hydrocephalus (congenitalhydrocephalus) or develop the condition during or after birth (acquiredhydrocephalus).

The brain constantly makes CSF. It fills up spaces inside the brain (ventricles) to protect and nourish the brain and spinal cord. The CSF is normally absorbed into the blood and carried out of the brain as new CSF is made. In hydrocephalus, the CSF continues to build up in the brain. CSF can cause brain injury if too much fluid builds up.

What are the causes?

The most common cause of congenital hydrocephalus is a blockage between two ventricles that prevents CSF from moving through the brain. Several other types of brain development problems can cause hydrocephalus. They include:

Spinal cord abnormalities.
Brain cysts.
Brain malformations that your child is born with.
Infections during pregnancy.
Other genetic disorders.

Causes of acquired hydrocephalus may include:

Bleeding in the brain, which results from being born too early (prematurely).
Head injury.
Brain tumors.
Brain infections (meningitis).

What increases the risk?

Your child is more likely to develop congenital hydrocephalus if:

He or she has a family history of congenital hydrocephalus. This is the most important risk factor for this condition.
The child's mother received poor care before she gave birth (prenatal care).
Your child is a twin or other multiple.
His or her mother had diabetes during pregnancy (maternal diabetes).

Your child is more likely to develop acquired hydrocephalus if he or she:

Was born prematurely.
Had head injuries.
Had brain tumors.
Had stroke.
Had bleeding or blood clots inside the brain.
Had infections, such as meningitis.
Had a low birth weight.
Is male.

What are the signs or symptoms?

Signs and symptoms of hydrocephalus in babies may include:

Rapid increase in head size.
Bulging of the soft spot (fontanel) on a baby's head.
Sleepiness.
Irritability.
Vomiting.
Downward-gazing eyes.
Seizures.

Signs and symptoms in older children may include:

Headache.
Nausea and vomiting.
Clumsiness.
Downward-gazing eyes.
Sleepiness and irritability.
Visual problems, such as blurred or double vision.
Loss of bladder control.
Personality changes and memory problems.

How is this diagnosed?

Hydrocephalus may be diagnosed by:

Medical history and physical exam.
Brain imaging tests, including a CT scan and MRI.

Sometimes congenital hydrocephalus can be diagnosed before birth. This may occur if the mother has an imaging test using sound waves (fetal ultrasound) during pregnancy. This test may show widening of the ventricles.

How is this treated?

Surgery is the only treatment for hydrocephalus. Surgery may involve:

Placing a flexible tube (shunt) in the brain or spinal cord to drain the fluid into another part of the body. This is the most common type of surgery for this condition. The shunt may need to be adjusted or replaced over time as your child grows.
Having a thin, flexible tube with a camera (endoscope) threaded into the ventricle. The surgeon then makes an opening in the floor of the ventricle to drain CSF and allow it to be absorbed into the blood. In some cases, a special device called a cautery is also used to treat the blood vessels in the area where CSF is made.

Follow these instructions at home:

Work closely with all of your child's health care providers to manage your child's condition. Managing hydrocephalus requires a team approach. The treatment team may include the following:
- Pediatricians.
- Neurologists.
- Surgeons.
- Nurses.
- Physical therapists.
- Child life specialists.
Ask your child's health care provider if your child can safely participate in sports activities.
Have your child wear head protection when doing any activities that may cause head trauma, such as biking or skating. Talk to your child's health care provider about appropriate head protection.
Keep all follow-up visits as told by your child's health care providers. This is important.

Where to find more information

Learn as much as you can about your child's condition. The Hydrocephalus Association is a good resource for information and support: www.hydroassoc.org

Contact a health care provider if your child:

Has chills or fever.
Has changes in symptoms or new symptoms.
Becomes more irritable.
Has a lack of appetite.

Get help right away if your child:

Has trouble breathing.
Has a seizure.
Has a severe headache or stiff neck.
Has vision changes.
Has persistent vomiting.
Is very sleepy or is hard to wake up.
Has an enlarged head or a bulging fontanel.

Summary

Hydrocephalus is when there is too much fluid (cerebrospinal fluid or CSF) around the brain.
In children who are born with the condition, the most common cause is a blockage between two ventricles that prevents CSF from moving through the brain.
Surgery is the only treatment for hydrocephalus.
Work closely with all of your child's health care providers to manage your child's condition.