Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

BackgroundBiliary atresia (BA) with hilar cyst is an uncommon variant, which constitutes less than 10% of all types of BA, and the operative procedure for this type of BA remains controversial.MethodsWe have had 200 cases with BA from 1963 to 2008 in our institute and our branch hospitals, in which 12 cases (6%) were BA with a hilar cyst. The clinical records of all cases were evaluated retrospectively.ResultsTwelve BA patients with a hilar cyst included 2 boys and 10 girls. The diagnosis of BA was confirmed by intraoperative cholangiography (cloudy or treelike pattern). In all 12 cases, a hepaticojejunostomy was performed at a median age of 71.6 days (range, 24-136 days). The follow-up periods were 1.2 to 23.2 years. The current mean total bilirubin level was 0.8 mg/dL (range, 0.2-3.5 mg/dL), and the mean direct bilirubin level was 0.2 mg/dL (range, 0.0 to 0.8 mg/dL). Methylprednisolone or oral prednisolone was administered in 8 cases after operation, and 10 of 12 cases achieved a jaundice free state. The postoperative complications were cholangitis (n = 10), gastroesophageal varices (n = 7), splenomegaly (n = 3), ileus (n = 1), and pulmonary hypertension (n = 1). The overall survival rate with a native liver was 10 (83.3%) of 12 cases, of which 9 cases were jaundice-free and only 1 case showed recurrent jaundice. The other 2 cases underwent living-donor liver transplantation at age of 2 and 20 years, respectively, and they are currently doing well.ConclusionsMost cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy.