A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly

The Dandy-Walker syndrome is a developmental disorder of the brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We experinced a case of Dandy-Walker syndrome with complex cardiac anomaly in female newborn who presented with apnea at birth. Physical examination showed coloboma on left eye, low estting malformed ear, and high arched palate. Autopsy revealed cystic dilatation of the fourth ventricle with secondary loss of cerebellar vermis. The floor of the fourth ventricle is exposed through this defect. Heart showed aortic atresia and univentricular heart. The case is reported with the review of the literatures.