Extensive primary retroperitoneal fibrosis (Ormond’s disease) with common bile duct and ureteral obstruction: A rare case report

INTRODUCTION: Idiopathic retroperitoneal fibrosis (Ormond’s disease) may involve the perirenal tissue, mesentery and biliary system in extremely uncommon situations in addition to classical compression of retroperitoneal structures particularly the ureters. PRESENTATION OF CASE: We report the case of a 60 year’s old man with clinical manifestation of obstructive jaundice, due to the common bile duct narrowing caused by a primary retroperitoneal fibrosis. Magnetic resonance cholangiopancreatography (MRCP) showed the presence of intrahepatic bile duct obstruction, suggesting the diagnosis of a hilar cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography (ERCP) showed strictures in the proximal common bile duct. A biliary endoprosthesis was inserted at ERCP. Histological investigations as well as CT-scan were compatible with retroperitoneal fibrosis. DISCUSSION: The main clinical presentation of the Ormond’s disease is compression of the ureters, and less commonly blood vessels and nerves. Our patient presented with obstructive jaundice, due to the common bile duct (CBD) compression, which was unusual. The first description of the extrahepatic biliary obstruction secondary to retroperitoneal fibrosis was made in 1964 and since then only 13 cases have been reported in the medical literature. CONCLUSION: Retroperitoneal fibrosis can cause compression of the CBD and therefore mimic a cholangiocarcinoma. Patients can be successfully managed with long-term CBD stent placement.