A case of extrahepatic bile duct resection with central bisectionectomy in patient with early onset localized primary sclerosing cholangitis

Abstract

Liver transplantation (LT) improves the prognosis of primary sclerosing cholangitis (PSC), but this procedure is limited in Japan. We experienced a case of early onset localized PSC, in which favorable QOL was obtained after bile duct resection (BDR) including hepatectomy. A 17-year-old male presented with jaundice. Bile duct stricture with a beaded appearance was found between the right anterior hepatic duct and extrahepatic bile duct, leading to diagnosis of PSC. Endoscopic treatment was difficult for the stricture, and therefore PTBD tubes were placed. Given the localized stricture, young age, difficulty with endoscopic treatment, and decreased QOL using the drainage tubes, we performed central bisectionectomy, extrahepatic BDR, and cholangiojejunostomy. In cases of localized PSC that may be improved by BDR including hepatectomy, surgery is an option as conservative treatment prior to LT.