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By Stephen J. Falchek, MD, Director, Residency Program and formerly Division Chief of Pediatric Neurology; Instructor, Nemours/Alfred I. duPont Hospital for Children; Sidney Kimmel Medical College of Thomas Jefferson University

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An encephalocele is a protrusion of nervous tissue and meninges through a skull defect.

The defect is caused by incomplete closure of the cranial vault (cranium bifidum). Encephaloceles usually occur in the midline and protrude anywhere along a line from the occiput to the nasal passages but can be present asymmetrically in the frontal or parietal regions. Small encephaloceles may resemble cephalhematomas, but x-rays show a bony skull defect at their base. Hydrocephalus (see Hydrocephalus) often occurs with encephalocele. About 50% of affected infants have other congenital anomalies. Symptoms and signs include the visible defect, seizures, and impaired cognition, including intellectual and developmental disability.

Prognosis depends on the location and size of the lesion. Most encephaloceles can be repaired. Even large ones often contain mostly heterotopic nervous tissue, which can be removed without worsening functional ability. When other serious malformations coexist, the decision to repair may be more difficult.