Primary sclerosing cholangitis - Resect, dilate, or transplant?

Objective The current study examines the results of extrahepatic biliary resection, nonoperative endoscopic biliary dilation with or without percutaneous stenting, and liver transplantation in the management of patients with primary sclerosing cholangitis (PSC) Summary Background Data Primary sclerosing cholangitis is a progressive inflammatory disease leading to secondary biliary cirrhosis, The most effective management of sclerosing cholangitis before the onset of cirrhosis remains unclear. Methods From 1980 to 1994, 146 patients with PSC were managed with either resection of the extrahepatic bile ducts and longterm transhepatic stenting (50 patients), nonoperative endoscopic biliary dilation with or without percutaneous stenting (54 patients), medical therapy (28 patients), and/or liver transplantation (21 patients). Results Procedure-related morbidity and mortality rates were similar between surgically resected and nonoperatively managed patients. In noncirrhotic patients, the serum bilirubin level was significantly (p < 0.05) reduced from preoperative levels (8.3 +/- 1.5 mg/dl) 1 (1.7 +/- 0.4 mg/dL) and 3 (2.7 +/- 0.9 mg/ dr) years after resection, but not after endoscopic or percutaneous management. For noncirrhotic PSC patients, overall 5-year survival (85% vs. 59%) and survival until death or transplantation (82% vs. 46%) were significantly longer (p < 0.05) after resection than after nonoperative dilation with or without stenting, For cirrhotic patients, survival after liver transplantation was longer than after resection or nonoperative dilation with or without stenting, Five patients developed cholangiocarcinoma, including three (6%) of the nonoperatively managed patients but none of the resected patients. Conclusions In carefully selected noncirrhotic patients with PSC, resection and long-term stenting remains a good option, Patients with cirrhosis should undergo liver transplantation.