Polysplenia syndrome and splenic hypoplasia associated with extrahepatic biliary atresia
Maksem, J.A.
Archives of Pathology and Laboratory Medicine 104(4): 212-214
1980
ISSN/ISBN: 0003-9985 PMID: 6892684 Document Number: 163868
Developmental anomalies extrinsic to the extrahepatic biliary tree were recognized in association with extrahepatic bile duct atresia in humans. Reported are 6 cases of extrahepatic biliary atresia associated with polysplenia syndrome and 2 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.