Encephalocele

Encephaloceles are a group of malformations characterized by calvarial and dural defects with extracranial herniation of leptomeninges, brain, and cerebrospinal fluid (CSF). Several classification schemes have been proposed by multiple authors in the past, leading to confusion in the nosologic categorization of the lesions. Primary encephaloceles refer to those lesions that are either present at birth or have a congenital substrate. Secondary encephaloceles are acquired lesions that are most commonly the result of trauma or postsurgical defects. Primary encephaloceles are divided into three major types: cranial vault, fronto-ethmoidal, and basal.Encephaloceles comprise ~10 to 15% of all neural tube defects. Encephaloceles may present as a single isolated congenital anomaly or in association with other anomalies to comprise syndromes or associations. Surgery is the only treatment option.