Hepatopulmonary syndrome in a child.

We report a case of congenital biliary atresia and liver cirrhosis who developed hepatopulmonary syndrome thirteen years after Kasai operation. This 15-yea-old girl suffered from hyperbilirubinemia and acholic stool since birth. Congenital biliary atresia was diagnosed at 6 weeks of age and she received Kasai operation when liver cirrhosis was also found during the surgery. Dyspnea and cyanosis insidiously occurred since the age of 13 and she had been admitted to our hospital due to episodes of esophageal varices bleeding where esophageal varices ligation was performed. Serial examinations showed PaO2: 71 mmHg despite of 100% oxygen supplement. Pulmonary catheterization showed multiple arteriovenous shunts and macroaggregated albumin test revealed significant shunting. Contrast-enhanced echocardiogram showed microbubbles in the left heart. Thus, hepatopulmonary syndrome type II was diagnosed. Treatment is a dilemma although liver transplantation with concomitant lung transplantation is a possible choice.