All issues > Volume 30(2); 1987

Original Article

J Korean Pediatr Soc. 1987;30(2):234-239. Published online February 28, 1987.

A Cases of Dandy-Walker Syndrome.

Jong Lin Rhi¹, Yoon Dok Kim¹, Hyung Ro Moon¹

¹Dept, of Pediatrics, Seoul National Umiversity Hospital, Seoul, Korea

Abstract

The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. Other systemic anomalies are associated with the syndrome. We experienced a case in a 15 day old female infant who presented initially with frequent aspirations and respiratory difficulty. On physical examination an encephalocele on the occipital area, a rudimentary nipple below the left nipple, and polydactyly of the feet were present. On barium esophagogram the swallowed barium drained from the oropharynx into both the laryngotracheal and the esophagus due to malfunctioning of the epiglottis. On transillumination of the skull the halo was increased on the occipital area but the characteristic triangular area was not demonstrated. The brain CT revealed dilatation of the lateral and fourth ventricles, large posterior fossa cyst and hypoplastic cerebellum with no vermis. The case is reported with the brief review of the literatures.

Keywords : Dandy-Walker Syndrome.