Stenosis of the common bile duct due to Ormondʹs 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 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 Eight months later, azathioprine was withdrawn and prednisolone was tapered continuously to a dose less than 10 mg/day.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 Ormondʹs disease was responsible for the tumor that had to be resected.