Case report of biliary atresia associated with preduodenal portal vein, ventricular septal defect and bilobed spleen.

A case report of biliary atresia associated with preduodenal portal vein is presented with a review of 27 similar cases previously reported. The occurrence of associated anomalies in these 28 cases has a much higher frequency (82%) than coincidental association. They were associated with cardiovascular anomalies in 71%, polysplenia in 68%, malrotation of the intestine in 61%, situs inversus in 22% and duodenal atresia in 9%. Developmental anomaly is considered to participate strongly in the aetiology of biliary atresia combined with preduodenal portal vein.