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.