Abstract

Hydrocephalus associated with a posterior fossa cyst and hypoplasia of the cerebellar vermis constitute a triad of Dandy-Walker syndrome. Since this syndrome was first described by Sutton in 1887, it has been the source of much controversy not only as to its embryogenesis but also concerning the correct diagnosis and surgical management. Probably the most widely accepted view about the pathogenensis is a complex developmental anomaly rather than previously believed atretic foramina of the 4th ventricle. This is an autopsy case report of a male infant who was born after 32 weeks of gestation period and expired 90 minutes after the birth. The case showed the characteristic findings of marked cystic dilatation of 4th ventricle and hypoplasia of the cerebellar vermis. In addition, there were occipital meningoenceptalocele that was connected with the roof of the attenuated cyst wall of fourth ventricle. There was also bilateral polycystic kidney of Potter type 1. This case is reported on the base of rarity of this interesting combination of two anomalies.