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Primary (congenital) encephalocele

Tadanori Tomita, MD
Hideki Ogiwara, MD, PhD
Section Editors
Marc C Patterson, MD, FRACP
Leonard E Weisman, MD
Deputy Editor
Carrie Armsby, MD, MPH


An encephalocele is a protrusion of the brain and/or meninges through a defect in the skull (cranium bifidum) that is "closed" or covered with skin. Encephalocele is one of the three most common neural tube defects (NTDs). The epidemiology, clinical features, diagnosis, and management of encephaloceles are discussed in this topic review.

The other main types of NTDs are anencephaly and myelomeningocele, which are discussed in separate topic reviews:

Anencephaly, which is an open NTD as the affected region of the cranial neural tube is exposed to the body surface. It is a severe defect, and is not compatible with survival. (See "Anencephaly".)

Myelomeningocele, which is characterized by a cleft in the vertebral column, with a corresponding defect in the skin so that the meninges and spinal cord are exposed. (See "Pathophysiology and clinical manifestations of myelomeningocele (spina bifida)" and "Overview of the management of myelomeningocele (spina bifida)".)


In this topic review we will use the term "encephalocele" to describe lesions that include brain and/or meninges. Some authors use the more general term "cephalocele," and reserve the term "encephalocele" for lesions that include brain and "meningocele" for those that include only meninges [1].

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Literature review current through: Nov 2017. | This topic last updated: Aug 15, 2017.
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