MedlinePlus Medical Encyclopedia: Retroperitoneal fibrosis

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Retroperitoneal fibrosis

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Illustrations

Male urinary system

Alternative names Return to top

Idiopathic retroperitoneal fibrosis; Ormond's disease

Definition Return to top

Retroperitoneal fibrosis is a disorder in which the tubes that carry urine from the kidneys to the bladder are blocked by a fibrous mass in the back of the abdomen.

Causes, incidence, and risk factors Return to top

Retroperitoneal fibrosis is a rare disorder caused by the proliferation of fibrous tissue in the area of the body behind the stomach. Doctors don't know why the masses form. It is most common in people aged 40-60, and men are twice as likely to be affected as women.

The disorder may cause chronic unilateral obstructive uropathy or chronic bilateral obstructive uropathy, which result when the fibrous mass blocks the ureters.The symptoms are caused by this obstruction of the ureters.

Symptoms Return to top

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 Return to top

elevations in serum BUN and creatinine
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
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 Return to top

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 (drainage tubes) 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 help if surgery can't be done due to other medical conditions.

Some doctors use the drug tamoxifen to treat this condition.

Expectations (prognosis) Return to top

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 Return to top

chronic bilateral obstructive uropathy
chronic unilateral obstructive uropathy
chronic renal failure

Calling your health care provider Return to top

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

Prevention Return to top

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

Update Date: 8/18/2003

Updated by: David Webner, M.D., Sports Medicine Fellow, Crozer-Keystone Family Practice Program, Springfield, PA. Review provided by VeriMed Healthcare Network.

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