Dandy-Walker Syndrome in a Child at Rural Kelantan, Malaysia

Abstract

Dandy-Walker syndrome is a rare congenital malformation of the brain that involves the cerebellum and the fourth ventricle. It is characterised by a classical triad of hydrocephalus, cystic dilatation of the fourth ventricle and complete or partial agenesis of the vermis. Majority of cases are diagnosed during neonatal or early infantile period. In this case report, a seven-year-old boy complained of recurrent headaches for the past one year. Physical examination was unremarkable. Examination of the fundus on the same day revealed bilateral papilloedema. His subsequent computed tomography scan of the brain done at a major district hospital demonstrated features in keeping with Dandy-Walker malformation. Our case highlighted the importance of embarking on a detailed and thorough approach when dealing with a child with chronic headache, especially in rural settings where advanced medical equipment is not readily available.