All issues > Volume 27(2); 1984

Case Report

J Korean Pediatr Soc. 1984;27(2):191-196. Published online February 28, 1984.

2 Cases of Congenital Asplenia Syndrome.

Hyo Sup Joo, Chung Hye Chu, Byoung Soo Cho, Kyoo Hwan Rhee, Sa Jun Chung, Chang Il Ahn

¹Department of Pediatrics School of Medicine, Kyung Hee University

Abstract

The asplenia syndrome is rare, characterized by congenital abscence of spleen, cardiovascular anomalies and other malformations or malpositions such as total or partial situs inversus, symmetrical lobulation of the liver and/or lung. Associated multiple complex cardiovascular anomalies include common atrioventricular canal, pulmonary stenosis or atresia, anomalous venous drainage and transposition of great vessels. Two-dimensional echocardiography allows the accurate non invasive assessment of the above comple congenital cardiovascular anomalies which commonly occur in asplenia syndrome. We are reporting two cases of congenital asplenia syndrome that were suspected by two-dimensional echocardiography and confirmed by subsequent 99 m Technetium-Sulfur scanning.

Keywords :Asplenia syndrome; two-dimensional echocardiography