Primary sclerosing cholangitis: the role of extrahepatic biliary resection.

PSC is an idiopathic chronic cholestatic disease of the liver that has a variable clinical course. Patients should be managed based on the degree of liver dysfunction, as well as the degree of biliary obstruction and symptoms. Endoscopic biliary dilations provides symptomatic and biochemical relief with minimal morbidity and mortality. Endoscopic procedures, however, result in shorter overall and transplant-free survival than resection and may not decrease the incidence of cholangiocarcinoma. Dominant strictures that fail endoscopic management may harbor an underlying cholangiocarcinoma and surgical therapy should not be delayed. While transplantation is the option of choice in PSC patients with cirrhosis, extrahepatic biliary resection including the bifureation is a good therapeutic option in noncirrhotic patients. Resection of the extrahepatic biliary tree with the hepatic duct bifurcation should be considered in patients with a dominant extrahepatic stricture and preserved liver function. In the appropriately selected patient, extrahepatic biliary resection affords patients with survival rates equivalent to that of transplantation. In fact, extrahepatic biliary resection can potentially significantly delay or avoid transplantation. Extrahepatic biliary resection is a good durable option for noncirrhotic patients with PSC and a dominant extrahepatic stricture.