Encephalocele

Encephalocele is also known as cranium bifidum. It is a rare form of neural tube defect that is characterized by protrusion of the brain and meninges through openings in the skull. There are 3 types of encephaloceles: nasofrontal, nasoethmoidal and naso-orbital. There is often significant overlap in the type of encephalocele.

Although the exact etiology is unknown, trypan blue and arsenic have been associated as teratogens. Folic acid has been shown to decrease the incidence of neural tube defects and  it is recommended that women who may become pregnant take 400 micrograms of folic acid daily.

Encephaloceles are often accompanied by other skull abnormalities and/or brain malformations. The timing of surgery varies depending on the size and location of the tumor and also the general health of the patient, but is usually accomplished in early infancy. Outcomes vary widely and also depend on the size and location of the encephalocele and presence of associated malformations.

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