Children's Hospital Colorado
Craniofacial Center

Encephalocele

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What is an encephalocele?

An encephalocele is a condition in which part of the brain protrudes through the skull. Usually, in addition to the defect in the skull, there is an underlying defect in the dura, a membrane that separates the brain from the skull. Sometimes, the skin over the encephalocele is absent or thin, but it may be present and normal. Sometimes, there is no brain protruding into what appears to be an encephalocele. Then the encephalocele is not really an encephalocele, but is a meningocele, a protrusion of the membranes covering the brain, but not of the brain itself.

Why do encephaloceles occur?

Usually, encephaloceles and meningoceles are congenital, meaning babies are born with them. In North America, they are most commonly occipital (in the back of the head). In Asia, they are usually sincippital (between the eyes and the nose). Other locations are unusual.

The cause of congenital encephaloceles and meningoceles is unknown, although they can be associated with various inherited syndromes. Those that are not associated with syndromes are often sporadic and not inherited. Congenital encephaloceles occur in about 1 to 4 out of every 10,000 babies. In both syndromic and sporadic types, congenital encephaloceles and meningoceles are often associated with other malformations of the brain.

Encephaloceles can rarely also be acquired after birth, especially after head trauma. In such cases, the brain protrudes through a traumatic tear in the dura and an overlying skull fracture. With the exception of other injuries to the brain in the posttraumatic variety, acquired encephaloceles are not usually associated with other abnormalities of the brain.

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John Bealer, MD

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