Large occipito-cervical encephalocele with Chiari III malformation.

Chiari type III is the rarest of the Chiari malformations and is usually associated with high morbidity and mortality. Treatment consists of primary closure of the encephalocele with or without cerebro-spinal fluid (CSF) shunting. In our case, the patient was treated with ventriculoperitoneal shunt followed by excision of the encephalocele. We propose that large encephaloceles should be treated with CSF shunting prior to repair of the sac so as to achieve optimal result.