RETROPERITONEAL FIBROSIS (ORMOND’S DISEASE)

Abstract

Retroperitoneal fibrosis – is a nonspecific non-purulent inflammation of a fibroadipose tissue that causes symptoms owing to a gradual compression of tubular structures of retroperitoneal spaces. There are primary (idiopathic) and secondary retroperitoneal fibroses (RPF) are distinguished. In the development of idiopathic RPF the leading part belongs to autoimmune mechanisms. The secondary RPF is a consequence of various causes with medication among them. Intravenous urography is traditionally used to confirm the diagnosis of retroperitoneal fibrosis. The triad showing the presence of retroperitoneal fibrosis includes: 1) hydronephrosis with dilated gyrose upper segment of the ureter, 2) medial deviation of the ureter and 3) external compression of the ureter. Idiopathic retroperitoneal fibrosis is often curable when treated with corticosteroids and/or with additional immunosuppressive agents. At the same time for patients, especially for those with diffuse disease, surgical intervention is often required in order to release ureters and other structures with the aim to reduce obstruction.