Encephaloceles

How common are encephaloceles?

Encephaloceles occur rarely, at a rate of one per 5,000 babies worldwide.

Encephaloceles of the back of the head are more common in Europe and North America, while encephaloceles on the front of the head more frequently occur in Southeast Asia, Africa, Malaysia and Russia.

• Ethnic, genetic, and environmental factors, as well as parental age, can all affect the likelihood of a child having an encephalocele.

The condition can occur in families with a family history of spina bifida.

What causes encephaloceles?

Although the exact cause is unknown, encephaloceles are caused by failure of the neural tube to close completely during fetal development. Research has indicated that teratogens (similar to x-rays), trypan blue (a stain used to color dead tissues or cells blue), and arsenic may damage the developing fetus and cause encephaloceles.

Proper levels of folic acid have been shown to help prevent encephaloceles when taken before pregnancy, and early in pregnancy. We recommend that women who may become pregnant take 400 micrograms of folic acid daily.

What are the different types of encephaloceles?

Encephaloceles are generally classified into the following categories based on where they occur and what they involve:

• nasofrontal - present in the nose and the forehead
• nasoethmoidal - present in the nose and the ethmoid sinus
• naso-orbital - present in the nose and the eye.
• meningocele - if the bulging portion contains only cerebrospinal fluid and the overlaying membrane
• encephalomeningocele - if brain tissue is also involved

What are the symptoms of encephaloceles?

Encephaloceles are often accompanied by craniofacial abnormalities or other brain malformations.

Your child's symptoms may include:

• neurologic problems
• hydrocephalus (cerebrospinal fluid accumulated in the brain)
• spastic quadriplegia (paralysis of the limbs)
• microcephaly (an abnormally small head)
• ataxia (uncoordinated muscle movement)
• developmental delay
• vision problems
• mental and growth retardation
• seizures