Retroperitoneal fibrosis

Retroperitoneal fibrosis

Definition:

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.

Symptoms:

Early symptoms:

dull pain in the abdomen which increases with time
swelling of one leg
decreased circulation in the legs leading to pain and discoloration
severe abdominal pain with hemorrhage due to ischemic bowel

Later symptoms:

urine output, decreased
total lack of urine (anuria)
nausea, vomiting, changes in thinking caused by kidney failure and the resulting build up of toxic chemicals in the blood.

Signs and tests:

elevations in serum BUN and creatinine.
A kidney ultrasound may show hydronephrosis (distention of the kidney pelvis because of fluid accumulation) and a mass.
Excretory urography may show compression and deviation of the ureters.
An abdominal CT scan is the best test to reveal the retroperitoneal mass.
MRI offers comparable results to the CT scan.
Biopsy to differentiate between retroperitoneal fibrosis and a cancerous tumor.

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:

Call your health care provider if you experience lower abdomen or flank pain, particularly with decreased urine volume.

Prevention:

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

Review Date: 11/21/2001
Reviewed By: Sarah Pressman Lovinger, M.D., General Internist at Boston Medical Center, Boston, MA. Review provided by VeriMed Healthcare Network.

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