Dandy-Walker syndrome: clinico-pathological features and re-evaluation of modes of treatment.

Eighteen cases of the Dandy-Walker syndrome are presented. The clinical manifestations are analyzed. Almost half of these children had associated congenital anomalies. Only four had the "characteristic" radiological findings. Direct surgical attack upon the cyst was not a successful form of treatment. Satisfactory treatment consisted of shunting of the ventricular system but the incidence of complications was high. There was a 27% mortality. The etiology and pathology are discussed.