Korean J Gastroenterol.
1998 Nov;32(5):669-673.
Primary Sclerosing Cholangitis Associated with Idiopathic Retroperitoneal Fibrosis
- Affiliations
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- 1Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
- 2Department of Radiology, Seoul National University College of Medicine, Seoul, Korea.
- 3Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
- 4Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
Abstract
- A 61-year-old man was admitted due to jnundice which developed 15 days ago. One year and 10 months ago, left hydronephrosis and mild dilatation of right posterior intrahepatic duct (IHD) were observed on computerized tomography (CT), but no specific management was tried. The patient showed obstructive jaundice and renal insufficiency. Endoscopic retrograde cholangiopancreatography (ERCP) and CT showed bilateral hydronephrosis, both IHD dilatation and paraaortic fibrotic mass suggesting retroperitoneal fibrosis. Percutaneous transhepatic cholangiography and choledochoscopy suggested common hepatic duct (CHD) obstruction caused by the turnor mass, but its biopsy revealed chronic nonspecific inflarnmation with epithelial proliferation. At laparotorny, CHD wall including orifice of right IHD was thickened at 2 cm length and hydronephrosis and retroperitoneal indurated area were confimed. A hilar bile duct resection with Roux-en-Y hepaticojejunostomy was perfonned. Light microscopy demonstrated the extensive deposits of collagen, fibrocytes and mixed inflammatory cells on the bile duct wall, which are consistent with the features of sclerosing cholangitis. To our knowledge, this is the first case report of sclerosing cholangitis associated with idiopathic retroperitoneal fibrosis in Korea.
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