Clinical Study on Asplenia and Polysplenia Syndrome. |
Jung Yun Choi, Yong Soo Yun, Chang Yee Hong |
Department of Pediatrics, Medical College, Seoul National University, Seoul, Korea |
무비증후군 및 다비증후군에 관한 임상적 연구 |
최정연, 윤용수, 홍창의 |
서울대학교 의과대학 소아과학교실 |
Received: 19 July 1988 • Accepted: 1 November 1988 |
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Abstract |
The clinical characteristics of sixteen cases of asplenia and four cases of polysplenia were studied
at Seoul National University Hospital from October 1986 to February 1988. Cardiac anomalies were
confirmed by echocardiography and cardiac catherization. Splenic status was confirmed by abdominal aortography in most cases.
Mean age of asplenia was 1 year 10 months and that of polysplenia was 8 years 6 months.
Male-Female ratio of asplenia was 1.7:1 and that of polysplenia was 1:1. Bronchial arrangement was
appropriate for the splenic status in 16 cases out of 17 who had bronchial tomography. In 16 cases of
asplenia, abdominal organ heterotaxia was found in 10 and rest of them showed normal or mirror
image abdominal arrangement. Three out of 4 cases of polysplenia showed abdominal heterotaxia.
Cardiac lesions were similiar to the result of the provious reports. Total anomalous pulmonary
venous return, univentricular atrioventricular connection and pulmonary atresia were common in
asplenia. Interruption of inferior vena cava and less severe cardiac defect were found in polysplenia. |
Key Words:
Asplenia, Polysplenia, Cardiac defects |
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