Cervical Encephaloceles in Children Homepage

Author

Jodi Smith, M.D., Ph.D, FAANS

Section Editors

Bermans J. Iskandar, M.D.

Graham Fieggen, M.D.

Senior Editor

Ann Ritter, M.D.

Editor In Chief

Rick Abbott, M.D.

Introduction

A cervical encephalocele is a rare neural tube defect that presents as a skin-covered cystic sac protruding from the back of the neck. It results from herniation of neural tissue through a fusion defect in the posterior elements of the cervical spine. Contents of the sac-like protrusion can include occipital lobe, brainstem, cerebellum, and/or upper cervical spinal cord. Prognosis depends primarily on the size and contents of the encephalocele as well as associated syndromes, cerebral anomalies, and ease of surgical correction.

Key Points

• Contents predicts outcome: Outcome depends on the amount and type of neural tissue within the encephalocele.
• Anatomy defines treatment: Appropriate surgical treatment requires a detailed understanding of encephalocele anatomy including the neural structures contained in the encephalocele, the arterial supply, and the venous drainage, which is frequently anomalous.
• Hydrocephalus risk: Patients should be monitored closely for the development of hydrocephalus after encephalocele repair.
• Treatment of associated hydrocephalus key: If there is viable neural tissue in the encephalocele preventing immediate closure, CSF diversion can facilitate regression of the tissue into the cervical spinal canal and enable delayed closure.