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Primary sclerosing cholangitis successfully treated by resection of the confluence of the hepatic duct
Author(s) -
Hirai Ichiro,
Ishiyama Shuichi,
Fuse Akira,
Kuzu Hiroshi,
Sakurai Fumiaki,
Kimura Seishi,
Kimura Wataru
Publication year - 2001
Publication title -
journal of hepato‐biliary‐pancreatic surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.63
H-Index - 60
eISSN - 1868-6982
pISSN - 0944-1166
DOI - 10.1007/s005340170042
Subject(s) - medicine , primary sclerosing cholangitis , cholangiography , gastroenterology , jaundice , common hepatic duct , bile duct , percutaneous transhepatic cholangiography , percutaneous , intrahepatic bile ducts , common bile duct , fibrosis , confluence , gallbladder , disease , computer science , programming language
Abstract Primary sclerosing cholangitis (PSC) is a cholestatic disease characterized by chronic inflammatory fibrosis of the extra‐ and intrahepatic bile ducts. Although the prognosis of patients with PSC was believed to be poor, some patients have not experienced the expected rapid clinical progression. A 51‐year‐old man with PSC was initially hospitalized for jaundice. Laboratory data showed low levels of the complement components C3, C4, and CH50. Percutaneous transhepatic biliary drainage was performed. Cholangiography revealed complete obstruction of the common bile duct below the confluence of the cystic duct. The confluence of the hepatic duct was resected and it was reconstructed by hepaticojejunostomy for palliation of the obstructive jaundice. Increased thickness of the walls of the common bile duct, right hepatic bile duct, and gallbladder was observed. Histopathological examination of the resected specimen revealed periductal fibrosis, with an onion‐skin‐like appearance. The patient is currently doing well, approximately 7 years after the surgery, without any signs of PSC recurrence. In this extraordinary patient, the laboratory data for C3, C4, and CH50 showed a complete return to normal levels. The positive results in this patient suggest that resection of the confluence of the hepatic duct may be an effective surgical treatment for noncirrhotic PSC patients who have dominant extrahepatic strictures.