All issues > Volume 18(6); 1975

Case Report

J Korean Pediatr Soc. 1975;18(6):468-472. Published online June 30, 1975.

Ivemark’s Syndrome 4 Cases

Soon II Lee¹, Woo Gill Lee¹, Bu Kyung Kang¹, Sun Hee Lee¹, Sung Kyu Lee¹, Dong Shik Chin¹, Duk Jin Yun¹

¹Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea

Abstract

Ivemark’s syndrome is rare disease and characterized by congenital absence of the spleen as sociated with characteristic group of anomalies of the cardiovascular and gastroenteric systems. We had experienced 4 cases of Ivemark’s yndrome: Case 1 had asplenia, situs inversus, dextrocardia, ASD, and pulmonary stenosis; Case 2 had asplenia, bilobulated liver, centraly located stomach but deviated to right side, single ventrilce, and transposition of the great vessels with pulmonary atresia; Case 3 had asplenia, bilobulated liver, centraly located stomach but deviated to left side, tricuspid atresia, high VSD, hypoplasia of right ventricle, and right sided aortic arch; Case 4 had asplenia, bilobulated liver, dextrocardia, single ventricle, and truncus arteriosus. We had diagnosed these cases by EKG, chest X-ray, cardiac catheterization with cardiac angiogram, and liver and spleen scanning.

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