Dandy Walker Syndrome with Unusual Associated Findings in a Fetal Autopsy Study

摘要

Dandy Walker Syndrome (DWS) is a congenital brain malformation characterized by hypoplasia or absence of cerebellar vermis, cystic dilatation of fourth ventri- cle and hydrocephalus. It is frequently associated with other congenital anomalies. Associated central nervous system anomalies such as agenesis of corpus callosum and vermis are associated with poor prognosis. Associ- ation of DWS with congenital absence of spleen is life threatening condition and has been reported very rarely. Autopsy findings of DWS with association of congenital absence of corpus callosum, vermis and spleen are reported in a stillborn fetus of 28 weeks gestation and review of relevant literature was done.