Intrahepatic segmental primary sclerosing cholangitis: report of a case

A 67-year-old woman was referred to our hospital for investigation of epigastric discomfort. Computed tomography (CT) showed dilatation of the intrahepatic bile duct in the left lobe of the liver, endoscopic cholangiography showed complete stenosis of the left main branch of intrahepatic bile duct, and CT during angiography showed decreased portal blood flow to segment 3 of the liver. Based on these findings, we suspected intrahepatic cholangiocarcinoma and performed left lobectomy of the liver. However, pathological examination revealed fibrosis and infiltration of inflammatory lymphoid cells around the stenotic bile duct and periportal area, without any evidence of malignancy. Since these findings were compatible with sclerosing cholangitis and the patient did not have a disorder that would cause secondary sclerosing cholangitis, the final diagnosis was primary sclerosing cholangitis (PSC). It is difficult to distinguish segmental PSC from cholangiocarcinoma; thus we think surgical resection is an appropriate therapeutic and diagnostic procedure for segmental intrahepatic bile duct stenosis.