The Transsphenoidal Encephalocele: Associations and Elusive Origins

Abstract

Transsphenoidal encephaloceles comprise a rare subset of congenital basal encephaloceles in which brain tissue and overlying meninges herniate through an embryologic defect in the body of the sphenoid bone. The pathogenesis is thought to be multifactorial, possibly beginning with neural crestopathic arrest or insult, which results in subsequent mechanical and in utero events. Importantly, associated anomalies, such as optic, vascular, and endocrine abnormalities, can occur in association with transsphenoidal encephaloceles. As such, a cross-sectional multimodality approach is required to fully elucidate the extent of abnormalities, including CT, CTA, MR imaging, and/or MRA for complete characterization.