J Korean Pediatr Soc.  1995 Jan;38(1):110-116.

A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly

• Yoon SJ
• Hong SJ
• Cho HG
• Park DC

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.

Keyword

Dandy-Walker syndrome; Aortic Atresia; Univentricular heart

MeSH Terms

Apnea
Autopsy
Brain
Coloboma
Dandy-Walker Syndrome*
Dilatation
Ear
Female
Fourth Ventricle
Heart
Humans
Infant, Newborn
Palate
Parturition
Physical Examination