Background: As the survival of cystic fibrosis patients improves due to better treatment of its pulmonary manifestations, the management of hepatobiliary complications becomes increasingly vital. While focal biliary cirrhosis is common, large duct manifestations are less frequently encountered.
Methods: We prospectively evaluated cases of large bile duct disease in a large adult cystic fibrosis practice at the Keck Hospital of the University of Southern California.
Results: Over a 5-year period, six patients presented with cholangiectasia, hepatolithiasis, and strictures. Their clinical presentation and course closely resembled recurrent pyogenic cholangitis (RPC). Treatment of cholangitis and strictures was primarily by endoscopic retrograde cholangiopancreatography, but major hepatobiliary surgery following pulmonary optimization was required in 33 %.
Conclusion: In adult populations, CF-RPC may not be as unusual as previously reported and recognition allows optimal endoscopic, medical, and surgical management.