Surgery for segmental primary sclerosing cholangitis.

Primary sclerosing cholangitis is an uncommon chronic hepatobiliary disorder, and the definitive surgical treatment for symptomatic primary sclerosing cholangitis is liver transplantation. Although some cases with primary sclerosing cholangitis treated with hepaticojejunostomy or partial hepatectomy have been reported, the indications for these procedures and their long-term results have not been well defined or studied. We present three patients with segmental primary sclerosing cholangitis, and discuss the indication of surgical treatment for primary sclerosing cholangitis excluding liver transplantation. Three patients with segmental primary sclerosing cholangitis of the common bile duct and the hepatic ducts were treated by hepaticojejunostomy with partial resection of the common bile duct. We investigated clinical features such as angiography, cholangiography, and surgical treatment outcomes. It was difficult to differentiate primary sclerosing cholangitis from a cholangioma preoperatively, despite the use of cytology, angiography, and cholangiography. Two of the three patients were preoperatively suspected to have cholangioma. Segmental primary sclerosing cholangitis was diagnosed operatively. All patients were treated hepaticojejunostomy and achieved long-term survival. Although liver transplantation is the treatment of choice for primary sclerosing cholangitis, in cases of segmental primary sclerosing cholangitis, local resection of the involved structures may be curative. Resection of a discrete lesion may provide many years of survival with a good quality of life, in some cases obviating the need for liver transplantation.