Surg Today (2006) 36:638–641 DOI 10.1007/s00595-006-3218-5 Intrahepatic Segmental Primary Sclerosing Cholangitis: Report of a Case Taku Matsumoto 1 , Tetsuo Ajiki 1 , Ippei Matsumoto 1 , Masahiro Tominaga 2 , Hiroshige Hori 1 , Yoshiyasu Mita 1 , Tsunenori Fujita 1 , Yasuhiro Fujino 1 , Yasuyuki Suzuki 1 , Yonson Ku 2 , and Yoshikazu Kuroda 1 Departments of 1 Gastroenterological Surgery and 2 Liver Surgery and Transplantation, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan results in stenosis of only one part of the intrahepatic bile duct. 2 We report a case of intrahepatic segmental PSC, which was diagnosed preoperatively as intra- hepatic cholangiocarcinoma. Case Report A 67-year-old woman presented to a local hospital after she began to experience epigastric discomfort. Abdomi- nal ultrasonography showed localized dilatation of the intrahepatic bile duct and she was referred to our hospital for further investigations. Laboratory data on admission showed a slight elevation of the serum hepatobiliary enzymes. Asparatate aminotransferase (AST, 50 IU/l), alanine aminotranseferase (ALT, 77 IU/ l), alkaline phosphatase (ALP, 392 IU/l), and leucine aminopeptidase (LAP, 95 IU/l) were elevated, but serum total bilirubin, carcinoembryonic antigen (CEA), and carbohydrate antigen (CA) 19-9 were not elevated. Abdominal ultrasonography and computed tomogra- phy (CT) scans both showed dilatation of the intrahe- patic bile duct in the left lobe, but we did not detect any tumoral density on these studies. Endoscopic retro- grade cholangiography (ERC) showed complete steno- sis of the main branch of the left intrahepatic bile duct without irregularity of the wall (Fig. 1). Cytology of bile collected during the ERC was negative for malignancy. Abdominal angiography did not show a tumor stain and there was no encasement of the hepatic artery or portal vein. The patient also underwent CT during arterial portography (CTAP), done after a 30-s delay from when 90 ml contrast material was injected through a 5-F catheter in the superior mesenteric artery via a femoral approach at a rate of 2.5 ml/s. The CTAP showed a blood flow defect in the left lower lateral segment (S3) (Fig. 2a), but the same segment was strongly contrasted on CT during hepatic arteriography (CTHA), done after a 10-s delay from when 15 ml contrast material was Abstract A 67-year-old woman was referred to our hospital for investigation of epigastric discomfort. Computed tomo- graphy (CT) showed dilatation of the intrahepatic bile duct in the left lobe of the liver, endoscopic cholangiog- raphy 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, with- out any evidence of malignancy. Since these findings were compatible with sclerosing cholangitis and the pa- tient 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 di- agnostic procedure for segmental intrahepatic bile duct stenosis. Key words Segmental primary sclerosing cholangitis · Intrahepatic bile duct · Operation Introduction Primary sclerosing cholangitis (PSC) is a cryptogenic bile static inflammatory disease characterized by fibrosing inflammatory destruction of the intra- or extrahepatic bile duct, or both. 1 Bile duct stenosis in PSC is generally diffuse and manifold, and it rarely Reprint requests to: T. Ajiki Received: July 28, 2005 / Accepted: January 17, 2006