Retroperitoneal fibrosis

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Male urinary system

Retroperitoneal fibrosis

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 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.

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