Hepatobiliary anomalies associated with polysplenia syndrome

The report of a 29-year-old woman with polysplenia syndrome, Crohn's disease, and bilateral cataracts is presented. The patient was noted to have a right-sided stomach and small bowel, Crohn's ileitis, and a left-sided colon. Results of roentgenography of the chest and echocardiography were consistent with a diagnosis of hypoplasia of the inferior vena cava with azygos continuation. The patient underwent laparotomy with cholecystectomy, exploration of the common bile duct, and choledochoscopy for cholelithiasis, choledocholithiasis, and chronic cholecystitis. Laparotomy revealed a liver that had two lobes, each with the morphologic appearance of the left lobe. The gallbladder was centrally located. T-tube cholangiography revealed a quadruplication of the intrahepatic biliary ducts. To our knowledge, this patient is the only known adult with this syndrome in whom cholangiography demonstrated isomerism of the biliary tree. A review of the literature on this subject is given with emphasis on biliary anomalies.