All issues > Volume 30(10); 1987

Case Report

J Korean Pediatr Soc. 1987;30(10):1166-1169. Published online October 31, 1987.

A Case of Beckwith-Wiedemann Syndrome.

Hyo Sook Hong¹, Hong Chul Lee¹, Oh Kyung Lee¹, Myung Ho Lee¹

¹Department of Pediatrics. Chonju Presbyterian Medical Center

Abstract

Beckwith-Wiedemann syndrome constitutes a clinicopathologic entity characterized macroglossia, omphalocele or umblical hernia, visceromegaly, postnatal gigantism, microcephaly, Nevus flammeus, ear lobe grooves, hemihypertrophy and other abnormalities. The anomalies of the syndrome are facultative and not obligatory. We experience one case of Beck with-Wiedemann syndrome. This 1-day-aged female neonate showed macroglossia, gigantism, omphalocele and hypoglycemia. Review of literature was done briefly.

Keywords :Beck with-Wiedemanrx syndrome; Omphalocele