胆道閉鎖症の合併奇形 : 特に多脾症候群との関連性について

抄録

In our hospital, 23 infants with biliary atresia have been operated on during the past five years. Three of them had major malformations consisting of preduodenal portal vein, absent inferior vena cava, intestinal malrotation, partial annular pancreas and splenic anomalies. These findings led us to a statistical study of associated anomalies in patients with biliary atresia based on 758 cases in the file of the Annual of the Pathological Autopsy Cases in Japan during last 20 years. In 87 of 758 cases, associated anomalies were observed. Cardiovascular anomalies were the most common, such as ventricular septal defect, patent ductus arteriosus, absent inferior vena cava and preduodenal portal vein. Malrotation and common mesentery were the most common gastrointestinal anomalies. Associated anomalies of the spleen were surprisingly frequently observed in this series (23 cases) and situs inversus were present in 5 cases. Nine of 16 complex anomalies were suspected to be polysplenia syndrome. These anomalies, except malrotation develop during from the 4th to 6th week of fetal life and the biliary tracts also develop in the same period. It is suggested that the coexisting malformations have been caused by a teratogenic factor which is common with biliary atresia. It seems very likely that some cases of biliary atresia are etiologically related to developmental anomalies.