What is Congenital Hydrocephalus?

Congenital hydrocephalus is an abnormality of the circulation of the cerebrospinal fluid - which is a fluid found within the brain - that is present at birth. Hydrocephalus may occur alone as a condition, or can also occur in association with spina bifida. This article provides a general overview of congenital hydrocephalus, including diagnosis and treatment.

In those affected by congenital hydrocephalus, the fluid builds up in the ventricles inside the brain and causes an increase in the pressure on the bones of the skull. In newborns, the skull is not yet joined together permanently, so the skull enlarges with this fluid and the fontanelles (also known as the “soft spot”) becomes wider than is considered normal. This occurs with about 1 in every 1,000 live births.

With modern medicine, congenital hydrocephalus can often be diagnosed prior to birth using an ultrasound examination. If the baby’s head is very large from the condition, it is unlikely that a vaginal delivery will be possible. In this case, the baby will probably be delivered via caesarian section. Additionally, in the instance where the baby has severe congenital hydrocephalus, in connection with other problems and defects, the baby may die in-utero. A baby born with hydrocephalus may have brain damage, and/or mental and physical disabilities and impairments.

Once the baby is delivered, and if the condition is not too severe, then an operation can be performed to reduce the pressure of the fluid within the brain. The surgical operation involves draining off the cerebrospinal fluid into the baby’s nervous system. While the operation can help a baby affected by congenital hydrocephalus survive, it is highly likely that mental disabilities and impairment, as well as the chance for physical disabilities, will result.