Occlusive hydrocephalus in congenital myotonic dystrophy

A case of congenital myotonic dystrophy is reported which was complicated by the development of a hydrocephalus that needed ventricular-peritoneal shunting at the age of 4 months. Although dilatation of cerebral ventricles is a common feature in these patients, an occlusive hydrocephalus has not so far been reported.