All issues > Volume 33(3); 1990

Original Article

J Korean Pediatr Soc. 1990;33(3):367-372. Published online March 31, 1990.

A case of Beckwith-Wiedemann Syndrome.

Jin Soo Choi¹, Byung Hee Kim¹, Young Soo Chon¹, Hwa Il Kwang¹, Young Youn Choi¹, Tai Ju Hwang¹

¹Department of Pediatrics, Chonnam University Medical School, Kwangju, Korea

Received: June 15, 1989;  Accepted: October 7, 1989.

Abstract

The Beckwith-Wiedemann syndrome (exomphalos-macroglossia-gigantism: E M G Syndrome) is characterized by the presence of a wide variety of anomalies such as omphalocele, muscular macroglossia, gigantism, neonatal hypoglycemia, cytomegaly of the adrenal fetal cortex, hyperplasia of gonadal interstitial cells, and hyperplastic visceromegaly, particularly of pancreas and kidneys. Other abnormalities may include hemihypertrophy, microcephaly and mental retardation, prominent occiput, facial flame nevus, neonatal polycythemia, and linear indentations of the ear lobes. Since the original descriptions of this syndrome by Beckwith (1963, 1964) and Wiedemann, (1964) numerous examples of the association of these anomalies have been reported. We experienced a case of Beck with-Wiedemann in 1 day male baby who showed macroglossia, omphalocele, facial flame nevus, linear indentations of the ear lobes, hypoglycemia and prominent occiput. A brief review of related literatures is also presented.

Keywords :Omphalocele, Macroglossia, Hypoglycemia