Idiopathic retroperitoneal fibrosis

Alternative names
Retroperitoneal fibrosis; Ormond’s disease

Definition
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

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

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  
  • 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

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)
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

     
  • chronic bilateral obstructive uropathy  
  • chronic unilateral obstructive uropathy  
  • chronic renal failure

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.

Johns Hopkins patient information

Last revised: December 6, 2012
by Simon D. Mitin, M.D.

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