All issues > Volume 34(12); 1991

Original Article

J Korean Pediatr Soc. 1991;34(12):1699-1706. Published online December 31, 1991.

Three cases of Watson-Alagille syndrome.

Eun Kyeong Bom¹, Dae Hyun Kim¹, Dong Hoon Koh¹, Young Youn Choi¹, Jae Sook Ma¹, Tae Ju Hwang¹

¹Department of Pediatrics, College of Medicine, Chonnam National University, Kwangju, Korea

Received: June 3, 1991;  Accepted: July 25, 1991.

Abstract

Watson-Alagille syndrome is a kind of familial intrahepatic biliary atresia associated with charac- teristic face and cardiovascular anomaly. Growth and developmental delay, anomalies of the eyes such as posterior embryotoxon, deformities of the skeletal system are often the manifestations of this syndrome. We experienced three cases of Watson-Alagille syndrome recently. These patients showed charac- teristic faces and had peripheral pulmonary artery stenosis diagnosed by pulmonary angiography or 2 dimensional echocardiography and intrahepatic biliary atresia confirmed by liver biopsy. Butterfly deformity of vertebrae was detected in case 1 only. We reported three cases of Watson-Alagille syndrome with a brief review of related literatures.

Keywords :Watson-Alagille syndrome;Jaundice;Intrahepatic biliary atresia;Pulmonary artery stenosis