Intrahepatic cholangiojejunostomy as a palliative procedure in primary sclerosing cholangitis.

Primary sclerosing cholangitis is an idiopathic disease characterized by progressive diffuse stricture of extrahepatic and intrahepatic bile ducts. Eighteen patients with end-stage symptoms of primary sclerosing cholangitis were evaluated during a 10-year period from 1976 to 1986. Nine patients presented with disease amenable to intrahepatic cholangiojejunostomy. All patients presented with elevated liver function test results, and six of nine patients had a history of ulcerative colitis. The mean survival after intrahepatic cholangiojejunostomy was 3.9 years (range, 4 months to 10 years). Two of three of the patients with biliary cirrhosis died within 1 year after surgery. Four of nine patients remain alive today, with a mean survival of 4.7 years. For patients with end-stage primary sclerosing cholangitis, intrahepatic cholangiojejunostomy provides effective surgical palliation in those without secondary biliary cirrhosis.