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Retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare disorder that blocks the tubes (ureters) that carry urine from the kidneys to the bladder.

Alternative Names

Idiopathic retroperitoneal fibrosis; Ormond's disease

Causes, incidence, and risk factors

Retroperitoneal fibrosis occurs when extra fibrous tissue forms in the area behind the stomach and intestines. The tissue forms a mass (or masses) that can block the tubes that carry urine from the kidney to the bladder.

The cause of this problem is not known. It is most common in people aged 40 - 60. Men are twice as likely to develop the condition as women.

Symptoms

Early symptoms:

  • Dull pain in the abdomen that increases with time
  • Pain and change of color in the legs (due to decreased blood flow)
  • Swelling of one leg

Later symptoms:

  • Decreased urine output
  • No urine output (anuria)
  • Nausea, vomiting, changes in thinking caused by kidney failure and build-up of toxic chemicals in the blood
  • Severe abdominal pain with hemorrhaging (due to death of intestinal tissue)

Signs and tests

Abdominal CT scan is the best way to find a retroperitoneal mass.

Other tests that can help diagnose this condition include:

A biopsy of the mass may also be done to rule out cancer.

Treatment

Corticosteroids are tried first. Some doctors also prescribe a drug called tamoxifen.

If corticosteroid treatment doesn't work, a biopsy should be done to confirm the diagnosis. Other medicines to suppress the immune system can be prescribed.

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

Expectations (prognosis)

The outlook will depend on the extent of the problem and the amount of damage to the kidneys.

The kidney damage may be temporary or permanent.

Complications

The disorder may lead to:

Calling your health care provider

Call your health care provider if you have lower abdomen or flank pain and less output of urine.

Prevention

Try to avoid long-term use of medicines that contain methysergide. This drug has been shown to cause retroperitoneal fibrosis. Methysergide is sometimes used to treat migraine headaches.

References

Rottenberg G, Sandhu C. Radiology of the upper urinary tract. In: Adam A, Dixon AK, eds. Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging. 5th ed. New York, NY: Churchill Livingstone; 2008:chap 41.

Turnage RH, Richardson KA, Li BD, McDonald JC. Abdominal wall, umbilicus, peritoneum, mesenteries, omentum, and retroperitoneum In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 45.

Singh I, Strandhopy JW, Assimos DG. Pathophysiology of urinary tract obstruction. In: Wein AJ, ed.Campbell-Walsh Urology. 10th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 40.

Hsu THS, Nakada SY. Management of upper urinary tract obstruction In: Wein AJ, ed.Campbell-Walsh Urology. 10th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 41.

Updated: 5/7/2013

Louis S. Liou, MD, PhD, Chief of Urology, Cambridge Health Alliance, Visiting Assistant Professor of Surgery, Harvard Medical School. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.


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