Chiari type III malformation

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Abstract

Background. Chiari type III is the rarest of the Chiari malformations, and it is usually associated with a dismal prognosis in terms of early death or severe disability in long-term survivors. Surgical treatment usually consists of primary closure of the occipital/cervical encephalocele, followed by delayed CSF shunting for the associated hydrocephalus.

Methods. In our case, we introduced a technical variant consisting in primary external drainage from the malformative sac, followed by closure of the encephalocele and, eventually, by CSF shunting.