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Congenital Hydrocephalus - Topic Overview

How is congenital hydrocephalus diagnosed?

A fetal ultrasound can sometimes show the problem before birth. But most cases are found during a physical exam soon after birth. Your doctor may suspect that your baby has congenital hydrocephalus if your baby’s head is larger than normal.

Your baby may need imaging tests, such as a CT scan, an MRI, or an ultrasound, that can give a picture of the brain with more detail. Genetic tests may be done in some cases.

How is it treated?

Early treatment—before age 4 months—is important to help limit or prevent brain damage. Treatment focuses on reducing the amount of fluid in the brain to relieve pressure.

In most cases, the doctor places a flexible tube, called a shunt, in the brain to drain the fluid. The shunt carries fluid to another part of the body (usually the belly or the heart), which then absorbs the fluid. The shunt may stay in the brain for life, though it may be have to be fixed or replaced later if it becomes blocked or infected.

A surgery called endoscopic third ventriculostomy (ETV) may be used instead of a shunt in some cases. In ETV, a small hole is made in the deep part of the brain so that the fluid in the brain can flow freely.

Sometimes emergency treatment is needed to reduce the fluid. This may include medicines, a lumbar puncture (sometimes called a spinal tap), or a procedure to drain fluid from the brain until a shunt can be put in.

If your child has any developmental problems or delays caused by damage to the brain, your doctor can help you find the care you need. Treatment will focus on the specific problems your child has. For example, speech therapy can help with speech delays. Physical therapy can help with motor skill problems.

What kind of ongoing care will your child need?

No matter what kind of treatment your child has had, you and your child's doctors will need to watch your child closely to make sure that the fluid in the brain continues to drain properly. Pressure can build up in the brain again. Shunts can become blocked or infected. These problems need to be treated right away to prevent permanent damage.

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