Stenosis of the common bile duct due to Ormond's disease: case report and review of the literature

In a 46-year-old man endoscopic retrograde cholangiopancreatography and computed tomography scan showed a stenosis of the common bile duct by a hypodense mass highly suggestive of a Klatskin tumor. Histologic examination of the resected tumor revealed only non-specific inflammatory, fibrotic tissue...

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Bibliographic Details
Published in:Journal of hepatology 1999-07, Vol.31 (1), p.156-159
Main Authors: Dejaco, Clemens, Ferenci, Peter, Schober, Ewald, Kaserer, Klaus, Függer, Reinhold, Novacek, Gottfried, Gangl, Alfred
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cholangiopancreatography, Endoscopic Retrograde
Cholestasis, Extrahepatic - diagnosis
Cholestasis, Extrahepatic - etiology
Cholestasis, Extrahepatic - pathology
Common Bile Duct - diagnostic imaging
Common Bile Duct - pathology
Common bile duct stenosis
Gastroenterology. Liver. Pancreas. Abdomen
Humans
Immunosuppressive therapy
Klatskin tumor
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Middle Aged
Ormond's disease
Other diseases. Semiology
Retroperitoneal Fibrosis - complications
Retroperitoneal Fibrosis - diagnosis
Retroperitoneal Fibrosis - diagnostic imaging
Tomography, X-Ray Computed
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Summary:In a 46-year-old man endoscopic retrograde cholangiopancreatography and computed tomography scan showed a stenosis of the common bile duct by a hypodense mass highly suggestive of a Klatskin tumor. Histologic examination of the resected tumor revealed only non-specific inflammatory, fibrotic tissue without any evidence of malignancy. Three months later, the patient presented with hydronephrosis of the left kidney. Computed tomography scan showed a retroperitoneal mass with encasement of the left ureter. A percutaneous nephrostomy was performed and immunosuppressive therapy with prednisolone and azathioprine was initiated. Under this medication, almost complete regression of the pelvic mass and reopening of the ureter were observed within 3 weeks. Eight months later, azathioprine was withdrawn and prednisolone was tapered continuously to a dose less than 10 mg/day. After a follow-up of 2 years, the patient is still well. Although the histologic findings were nonspecific, further evaluation of this case suggests that Ormond's disease was responsible for the tumor that had to be resected.
ISSN:0168-8278
1600-0641
DOI:10.1016/S0168-8278(99)80176-7