History of Management of Cervical Encephaloceles in Children

Understanding of Disease

• 1590 – cervical encephalocele first described: Recorded descriptions of encephaloceles date back as early as 1590 (1), and many theories have been proposed over the years to explain the development of these congenital malformations. Jimenez and Barone provide a concise but thorough review of their history (1).
• 1891 – Chiari includes cervical encephaloceles in his description of Chiari III malformations: These extremely rare congenital defects occur as one of the main components of the so-called Chiari III malformation that was initially described by Hans Chiari in 1891 (2, 3). In Chiari’s original description, infants with Chiari III malformations exhibited herniation of the cerebellum below the foramen magnum through a cervical spina bifida and associated caudal displacement of the medulla.