A Rare Case of Idiopathic Retroperitoneal Fibrosis Involving Obstruction of the Mesenteric Arteries, Duodenum, Common Bile Duct, and Inferior Vena Cava

Abstract

Idiopathic retroperitoneal fibrosis (IRF), usually affects the ureter, although the biliary tree, duodenum and vasculature mayalso be susceptible. This case report describes a 64-year-old man with IRF, who presented painless watery diarrhea, radiological features of obstructive jaundice and duodenal obstruction, and ultimately an obstruction of the inferior vena cava. We employed tamoxifen for his treatment, but the disease progressed and the patient died of multiple organ failure two years after the onset. While the cause of IRF in this patient was obscure, we suspected his painless watery diarrhea indicated chronic ischemia of the small bowel, and the findings of an abdominal CTscan were extremely valuable in indicating IRF. (Internal Medicine 42: 812-817, 2003)

18F-Fluorodeoxyglucose Positron Emission Tomography in a Case of Retroperitoneal Fibrosis

“…In addition, although histologically benign, it may also present with symptoms related to encasement of various abdominal structures, including the urinary tract, 14,18) mesenteric arteries, 19) bile duct, and inferior vena cava. 20) Pain in the lower abdomen, as observed in the present case, is one of the most common presenting symptoms. Various drugs, such as azathioprine, cyclophosphamide, tamoxifen, and corticosteroids may cause regression of the retroperitoneal fibrotic mass, 12) although there have been no prospective randomized trials due to its low prevalence.…”

Obstructive jaundice due to autoimmune cholangiopathy

“…The ureter is the most vulnerable organ, although the biliary tree, as in our case, duodenum and blood vessels can also be affected 6. When the bile duct is involved, cholestasis develops 7.…”

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

“…The main mechanism is supposed to be an autoimmune disorder due to the presence of antibodies against ceroid, a complex of oxidized lipid and protein [4,5] . On the other hand, malignant tumors and proliferations can occur in 8% of cases [6,7] . The main clinical presentation of the Ormond’s disease is often the ureters’s compression, as well as blood vessels and nerves.…”