Cholangitis as a late complication in long-term survivors after surgery for biliary atresia

A retrospective review was performed to assess the utility of diagnostic imaging (DI), efficacy of treatment, and outcome of late cholangitis in long-term survivors after surgery for biliary atresia.

Sixty-one patients surviving without liver transplantation (LTx) for more than 20 years were divided into 2 groups depending on whether cholangitis developed after age 20. Clinical factors including the type of obstruction, the age at the initial operation, and the early complication with cholangitis were compared between the 2 groups. DI such as computed tomography scan and magnetic resonance imaging, clinical courses after treatment of cholangitis, and current status of the patients were also evaluated.

Thirteen patients had cholangitis after age 20. There was no statistical difference in the clinical factors studied between the 2 groups. Abnormal DI findings including dilatation of intrahepatic bile ducts and hepatic fibrosis were seen in 10 patients with late cholangitis. One patient died, and 2 ultimately underwent LTx. The remaining 10 patients including 4 with normal or near-normal liver function have survived without LTx.

Although the majority of the patients had potential predisposing factors for cholangitis such as dilatation of intrahepatic bile ducts, a few patients unexpectedly had cholangitis without such abnormal findings after an excellent, long-term postoperative course.