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.