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Encephalocele

What is an encephalocele?

An encephalocele (pronounced in-SEF-a-lo-seal) is when the skull does not close completely, and a part of the brain may extend into a sac.

Or, the defect may contain some of the brain’s cover (meninges) and cerebrospinal fluid.

Normally, a fetus’s central nervous system develops inside the neural tube. When this tube does not close properly, an encephalocele may result. Either skin or a thin membrane covers the defect.

An encephalocele can happen:

  • In the base of the skull
  • In the area of the nose, sinuses and forehead
  • From the top of the skull around to the back of the skull in the midline

Who gets an encephalocele?

This is a rare defect. It only occurs in about one of 5,000 live births worldwide. The rate may be even lower in North America. Other facts:

  • Girls are more likely to have an encephalocele in the back (occipital) area of their skull.
  • Boys are more likely to have the defect in the front area of their skull.
  • This defect happens more commonly in the back of the skull in North America.
  • It happens more commonly in the front of the skull in Southeast Asia.

What is your experience with encephalocele?

Because this defect is so rare, we operate on one to four babies with this problem a year. Some have small defects that we simply repair. Large defects require a more extensive repair.

Many children with an encephalocele may also have or develop hydrocephalus.

What surgery or procedure do you use to treat encephaloceles?

The goal of surgery is to close the opening. If the opening is not covered with normal skin at birth, we do the repair soon after your baby is born. If normal skin covers the opening we operate when your baby is a few months old.

Encephalocele surgery involves a craniotomy to open the area in need of repair.