Congenital Hydrocephalus

Treatment Overview

Treatment for congenital hydrocephalus focuses on lowering the amount of cerebrospinal fluid (CSF) in the brain to relieve pressure. Early treatment—within a baby's first 3 to 4 months—is important to help limit or prevent brain damage. But the long-term effects of congenital hydrocephalus depend largely on the cause of the condition, its severity, and the response to treatment. Other problems within the brain can also affect a child's outcome.

Initial treatment

When a newborn is diagnosed with congenital hydrocephalus, a shunt usually is surgically inserted in the brain to drain excess cerebrospinal fluid (CSF). Generally, one end of the shunt is placed in the ventricles in the brain and then is threaded out of the brain just below the skin under the scalp. Continuing under the skin, it goes behind the ear, down the neck, and into another part of the body—usually the abdomen—which then absorbs the CSF. Removing the excess fluid lowers the pressure within the brain, which helps to prevent or minimize brain damage.

Sometimes temporary emergency measures are needed to reduce or drain fluid until a shunt can be inserted. Such treatments may include:

• Acetazolamide or furosemide medicines, to slow the production of CSF in the brain. But the safety and effectiveness of these medicines are questionable.³
• Occasionally a lumbar puncture, which can help drain CSF from the brain until a shunt can be surgically implanted.
• Draining the CSF from the skull so it collects in a bag outside of the body. This is often done when a child cannot get a permanent shunt placed right away.

For noncommunicating hydrocephalus (caused by an obstruction), a surgical procedure called endoscopic third ventriculostomy (ETV) may be done instead of a shunt placement. In ETV, a small hole is made in the third ventricle of the brain, allowing cerebrospinal fluid to flow freely. While ETV may be used during ongoing treatment as a way to prevent shunt placement, it is not used as initial treatment in newborns. If ETV fails during ongoing treatment, your child will need a shunt placed at a later time.¹ Regardless of whether ETV or a shunt is used, your child will need to be watched closely over time to make sure the cerebrospinal fluid drains properly.

Ongoing treatment

Ongoing treatment for congenital hydrocephalus usually requires lifelong shunt use and close monitoring by health professionals, which may include a neurologist, a neurological surgeon, a family medicine doctor, a pediatrician, and a developmental pediatrician.

During routine appointments, your doctor will usually measure the size of your child's head and check your child's eyes for signs of pressure. Your doctor will also assess your child's neurological development and find out whether he or she has the same abilities as most other children around the same age. For example, if your child is about 12 months old, your doctor may ask you whether he or she can say a few words. At some visits, your doctor may order a computed tomography (CT) scan or magnetic resonance imaging (MRI) of the head and spine to ensure that cerebrospinal fluid (CSF) is draining properly.

Be alert for signs of shunt infection or malfunction. Excess CSF buildup in the brain can result in permanent brain damage.

• About 40% of shunts fail within the first 2 years.⁴ Shunt failure or malfunction allows fluid to build up within the brain, producing symptoms of irritability, excessive sleeping, poor appetite, frequent vomiting, eye-tracking problems, and high-pitched crying. After early childhood, additional symptoms may be noticed, such as vision problems, neck pain, confusion or behavioral changes, problems walking, seizures, or urinary incontinence.
• If the shunt causes infection, these same symptoms may occur, along with a fever. The chances of the shunt becoming infected are about 3% to 15%.⁴ Infections can develop at any time, but they most often occur within the first 3 months after shunt placement.
• The cause of the shunt malfunction or type of infection must be determined in order to properly manage the problem. For example, a shunt tap, a lumbar puncture, and fluid analysis may be done to confirm a suspected infection and to identify the type of bacteria present. This information allows a health professional to prescribe the most effective antibiotic.

For noncommunicating (obstructive) congenital hydrocephalus, an endoscopic third ventriculostomy (ETV) may be done instead of inserting a shunt. Although babies usually are not able to have this procedure, it may be used later instead of shunt replacement or repair. For an ETV, an endoscope is inserted through a small hole and helps open ventricles within the brain so CSF fluid is diverted around the blockage and absorbed outside of the brain without an implanted shunt. Once thought to be a permanent solution for some cases of obstructive congenital hydrocephalus, ETVs can also fail over time. It is important to have close monitoring for any signs of CSF buildup within the brain.

Regardless of the type of treatment, pay close attention for signs of any brain damage that affects function, such as delayed learning, failure to reach developmental milestones, or losing any physical or mental skills. Problems or delays are treated according to the specific issue (for instance, speech therapy for speech delays).

Your health professionals will discuss whether your child will need special care or have lifestyle restrictions. If problems from brain damage develop, you will be guided to the appropriate therapy or developmental program.

Treatment if the condition gets worse

No permanent cure for congenital hydrocephalus exists. Even when initial treatment is successful, cerebrospinal fluid (CSF) can build up within the brain again, causing symptoms to recur. Usually, problems are related to shunt malfunction or infection, requiring repair or replacement.

• Shunts have a 2-year failure rate of 40%.⁴ Shunt failure or malfunction causes fluid to once again build up within the brain, producing symptoms of irritability, excessive sleeping, poor appetite, frequent vomiting, eye-tracking problems, and high-pitched crying. After early childhood, additional symptoms may be noticed, such as vision problems, neck pain, confusion or behavioral changes, problems walking, seizures, or urinary incontinence.
• If the shunt causes infection, these same symptoms may occur, along with a fever. The chances of the shunt becoming infected are 3% to 15%.⁴ Infections most often occur within the first 3 months after shunt placement.
• The cause of the shunt malfunction or type of infection must be determined in order to properly manage the problem. For example, a shunt tap, lumbar puncture, and fluid analysis may be done to confirm a suspected infection and to identify the type of bacteria present. This information allows a health professional to prescribe the most effective antibiotic. If an infection is present, doctors will likely remove and replace the shunt.

CSF can also build up again after failure of an endoscopic third ventriculostomy (ETV). If this happens, surgery is needed to either repeat the ETV or to implant a shunt.

If CSF buildup is an emergency, it can be drained by penetration through the scalp or skull until the child is stabilized enough to have further surgical treatment.

Permanent brain damage caused by excess CSF buildup requires treatment that focuses on specific developmental problems. For example, if brain damage causes cognitive disability (mental retardation), treatment will target a wide range of issues, such as speech and motor skill development.

Your health professionals will discuss with you whether your child will need special care or have lifestyle restrictions. You will also be taught to recognize developmental delays or other signs of brain damage. If problems from brain damage develop, you will be guided to the appropriate therapy or developmental program.

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Author:	Debby Golonka, MPH	Last Updated: February 6, 2008
Medical Review:	Michael J. Sexton, MD - Pediatrics Mark G. Luciano, MD - Neurological Surgery
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