Intrahepatic cholangiojejunostomy as a palliative procedure in primary sclerosing cholangitis

J. R. Goldenring and C. E. Cahow
Department of Surgery, Yale University School of Medicine, New Haven, Conn 06510.

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.