Polysplenia syndrome and splenic hypoplasia associated with extrahepatic biliary atresia.

Developmental anomalies extrinsic to the extrahepatic biliary tree have been recognized in association with extrahepatic bile duct atresia. Reported are six cases of extrahepatic biliary atresia associated with polysplenia syndrome and two cases associated with splenic hypoplasia. There is a more than chance occurrence of splenic anomalies with anomalies of associated organ systems and extrahepatic biliary atresia. Organ systems most frequently involved were the cardiovascular, gastrointestinal, and respiratory systems.