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A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly

Journal of the Korean Pediatric Society 1995;38(1):110-116.
Published online January 15, 1995.
A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly
Ssack Joong Yoon, Sung Jin Hong, Hyung Goo Cho, Dong Chul Park
Department of Pediatrics, Konkuk University College of Medicine, Chungju, Korea
복합 심기형이 동반된 Dandy-Walker 증후군 1례
윤석중, 홍성진, 조형구, 박동철
건국대학교 의과대학 소아과학교실
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.
Key Words: Dandy-Walker syndrome, Aortic Atresia, Univentricular heart

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