Cervical encephalocele in a newborn--Chiari III malformation. Case report and review of the literature.

ABSTRACT

Chiari malformations are brain stem anomalies with or without spinal canal involvement. The combination of brain stem and cerebellar anomaly with a cervical cele is named Chiari III malformation. Patients with this extremely rare defect frequently present with severe neurological defects and can have a poor prognosis. Following a report on a newborn child with a Chiari III malformation that took a fatal but typical course the literature is reviewed.