A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl t ransferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the r ight side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia synd rome, a hepatic portojejunostomy was performed at 80 days of age. The posto perative course was uneventful, and the jaundice cleared. Although the occu rrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and p olysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice.