Surgical Treatment of Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease of unknown cause characterized by diffuse inflammation and fibrosis of intrahepatic and/or extrahepatic bile ducts. PSC is ultimately progressive, leading to obliteration of the biliary tree and subsequently to biliary cirrhosis. Surgical management of PSC can be undertaken in both the setting of benign and malignant complications of the disease. Endoscopic dilatation and stenting is the optimal initial treatment of symptomatic dominant biliary strictures. Liver transplantation has demonstrated excellent results for patients with advanced PSC. Patients with PSC have a 10% to 15% lifetime risk of developing cholangiocarcinoma (CCA). Liver transplantation has demonstrated excellent results for select patients with hilar CCA undergoing established protocols involving neoadjuvant chemoradiation, while liver resection can be indicated in the setting of both intrahepatic cholangiocarcinoma and hepatocellular carcinoma.