| Ivemark’s Syndrome 4 Cases |
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Soon II Lee, Woo Gill Lee, Bu Kyung Kang, Sun Hee Lee, Sung Kyu Lee, Dong Shik Chin, Duk Jin Yun |
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Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea |
| Ivemark 氏 症候群 4例 |
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이순일, 이우길, 강부경, 이선희, 이승규, 진동식, 윤덕진 |
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延世大學校 醫科大學 小兒科學敎室 |
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| Abstract |
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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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