Giant Asymptomatic Cystic Dilatation in Dandy-Walker Malformation.

ABSTRACT

Dandy-Walker malformation (DWM) is a rare congenital abnormality that involes the cerebellum and the fourth ventricle. It is characterized by cystic dilatation of the fourth ventricle, hypoplasia of the vermis cerebelli, separation of the cerebellar hernispheres, dilatation of the aqueductus mesencephali and absence of the lateral and median apertures of the fourth ventricle, an enlarged or normal-sized posterior cranial fossa and in some patients there may be hydrocephalus. We report a case of a 9-year-old male child with DWM who underwent right ventriculoperitoneal shunt at the age of three months. He is doing well since then except he is mentally retarded.