A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly.
- Author:
Seack Joong YOON
1
;
Sung Jin HONG
;
Hyung Goo CHO
;
Dong Chul PARK
Author Information
1. Department of Pediatrics, Konkuk University College of Medicine, Chungju, Korea.
- Publication Type:Original Article
- Keywords:
Dandy-Walker syndrome;
Aortic Atresia;
Univentricular heart
- MeSH:
Apnea;
Autopsy;
Brain;
Coloboma;
Dandy-Walker Syndrome*;
Dilatation;
Ear;
Female;
Fourth Ventricle;
Heart;
Humans;
Infant, Newborn;
Palate;
Parturition;
Physical Examination
- From:Journal of the Korean Pediatric Society
1995;38(1):110-116
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
