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.

Alternative Names

Idiopathic retroperitoneal fibrosis; Ormond's disease

Causes, incidence, and risk factors

Retroperitoneal fibrosis is a rare disorder caused by an excess of fibrous tissue in the area just behind the stomach. Doctors don't know why these masses form. It is most common in people aged 40 - 60, and men are twice as likely to develop them 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 the obstruction of the ureters, the tubes that carry urine from the kidneys to the bladder.

Symptoms

Early symptoms:

• Dull pain in the abdomen that 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:

• Decreased urine output
• 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

• Serum BUN and creatinine showing elevated levels
• Kidney ultrasound showing hydronephrosis (distention of the kidney pelvis because of fluid accumulation) and a mass
• Excretory urography showing compression and deviation of the ureters
• Abdominal CT scan (the best test to reveal the retroperitoneal mass)
• MRI (compares well to the CT scan)
• Biopsy showing either retroperitoneal fibrosis or a cancerous tumor

Treatment

Powerful anti-inflammatory medicines called corticosteroids are tried first. Some doctors also use a drug called tamoxifen to treat this condition.

If corticosteroid treatment doesn't work, a biopsy should be done to confirm the diagnosis. If confirmed, other medicines to suppress the immune system are usually prescribed.

When medicine does not work, surgery and stents (draining tubes) are needed.

Support Groups

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.

Images

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