Dandy-Walker Malformation with an Occipital Cephalocele in an Infant: A Case Report

Dandy-Walker Malformation (DWM) is an unusual hereditary intracranial anomaly that affects the cerebellum and its components and is also characterised with an enlarged posterior fossa. DWM can appear dramatically or develop unnoticed and occurs with occipital cephalocele in about 5% of cases. This is a case report of a 10-month-old male child with DWM who had a co-existing occipital cephalocele and presented on account of hydrocephalus and poor developmental milestone. He had a Contrast Enhanced Computed Tomographic (CECT) scan of the brain; which showed a posterior fossa cysts, a hypoplastic cerebellar vermis with a dysmorphic fourth ventricle that appear continuous with the posterior fossa cyst giving the so called `key hole deformity'. There is also associated hydrocephalus with an occipital cephalocele.