All issues > Volume 32(5); 1989

Original Article

J Korean Pediatr Soc. 1989;32(5):653-658. Published online May 31, 1989.

Clinical Study on Asplenia and Polysplenia Syndrome.

Jung Yun Choi¹, Yong Soo Yun¹, Chang Yee Hong¹

¹Department of Pediatrics, Medical College, Seoul National University, Seoul, Korea

Received: July 19, 1988;  Accepted: November 1, 1988.

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.

Keywords :Asplenia, Polysplenia, Cardiac defects