Congenital hydrocephalus is a birth defect where the brain is surrounded by too much cerebrospinal fluid, causing pressure and potential damage. Treatment options include a shunt or endoscopic third ventriculostomy, and ongoing care and monitoring is necessary for the child’s life. Only 1% of babies have this condition.
Congenital hydrocephalus, commonly referred to as water on the brain, is a congenital birth defect in which the brain is surrounded by too much cerebrospinal fluid, causing excessive pressure on the brain. This pressure can damage the baby’s brain, causing mental and physical problems. With early detection, treatment is available to limit long-term effects, although the success of treatment depends on how early the condition is diagnosed, what is causing the excess fluid, how much excess fluid is present, and how well the child responds to treatment. Only 1 percent of babies have congenital hydrocephalus.
A number of factors can cause congenital hydrocephalus, such as fetal bleeding in utero, syphilis or other maternal infections present during pregnancy, and some birth defects, such as spina bifida. Symptoms of congenital hydrocephalus include a head growth rate that is out of proportion to the baby’s overall growth, swelling or firmness of the fontanel, or soft spot, in the baby’s skull, irritability, lack of appetite, vomiting, and sleeping more than normal. A computed tomography (CT) scan, magnetic resonance imaging (MRI), or other imaging test can confirm the diagnosis. If congenital hydrocephalus is suspected, it is important to seek treatment as soon as possible. Treatment within the first three to four months of life usually offers the best prognosis.
If a baby is diagnosed with congenital hydrocephalus, the baby’s doctor will likely install a shunt in the baby’s brain to drain the excess fluid. This may be a permanent element in your child’s brain, but may need to be fixed or replaced over time. Outside of the United States (USA), surgery is sometimes performed in the uterus to drain fluid before birth, but this procedure is no longer available in the United States as many doctors feel that the benefits of this procedure are not enough large enough to justify the risks to the fetus. In emergency situations, a lumbar puncture may be done to relieve pressure until a shunt can be installed or fluid can drain into a sac from the baby’s skull. Drugs are available to slow or temporarily stop the production of cerebrospinal fluid, but the efficacy and safety of these drugs are not well studied.
In cases where fluid buildup is caused by an obstruction, the shunt may be stopped in favor of a surgical procedure called an endoscopic third ventriculostomy (ETV). ETV is not a treatment option for newborns, but rather is part of ongoing treatment for older children with congenital hydrocephalus. In ETV, the surgeon creates a small hole in the third ventricle of the brain, allowing fluid to drain out of the skull. However, ETV is not always successful, and where ETV fails, a shunt will need to be installed.
Babies diagnosed with congenital hydrocephalus will need ongoing care and monitoring for the rest of their lives. In addition to standard pediatric treatment, children with congenital hydrocephalus will need a neurologist, neurological surgeon, and developmental pediatrician to monitor progress and ensure the best prognosis with minimal complications. Developmental tests should be done to make sure the baby reaches appropriate developmental milestones, and imaging tests may be needed as the baby grows to ensure that fluid is draining properly from the skull. The child should also be monitored closely for signs of infection or shunt failure, which may include vomiting, irritability, high-pitched crying, trouble walking, confusion, seizures, tracking problems with the eyes or other vision problems, and neck pain.
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