A disorder caused by an aggregation of cells (fibrous mass) in the back of the abdomen, which may block the tubes that carry urine from the kidneys to the bladder (ureters).

Alternative Names

Idiopathic retroperitoneal fibrosis; Ormond's disease

Causes, incidence, and risk factors

Retroperitoneal fibrosis is caused by the proliferation of fibrous tissue in the retroperitoneum (the area of the body behind the stomach). Physicians don't know why these masses form. The disorder is rare, usually affecting people age 40 to 60, men are twice as likely to be affected as women. The disorder may cause chronic unilateral obstructive uropathy or chronic bilateral obstructive uropathy as a result of ureteral blockage caused when growth of fibrotic tissue obstructs the ureters. The symptoms are caused by the obstruction of the ureters.

Signs and tests

Treatment

Surgery to remove the mass and free the ureters may be required. In some cases, the ureters will be moved to a different position in the body or wrapped in fat tissue harvested from other areas to prevent recurrence of the fibrosis. Stents or drains placed in the ureter or in the renal pelvis may provide short-term relief of the symptoms until the mass can be removed. Corticosteroid therapy may be of value if surgery is can't be done due to other medical conditions. Some doctors use the drug tamoxifen to treat this condition.

Expectations (prognosis)

Prognosis depends on the extent of the fibrosis and the amount of damage to the kidneys. The kidney damage may be temporary or permanent.

Complications

Calling your health care provider

Prevention

If possible, avoid prolonged use of medications which contain methysergide, which has been shown to cause retroperitoneal fibrosis.