Retroperitoneal fibrosis

  • Definition
    • 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
    • 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 to 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 mental status caused by kidney failure and build-up of toxic chemicals in the blood
      • Severe abdominal pain with hemorrhaging (due to death of intestinal tissue)
  • Exams 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 does not 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.

  • Outlook (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.

  • Possible Complications
    • The disorder may lead to:

  • When to Contact a Medical Professional
    • 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
    • Hsu THS, Nakada SY. Management of upper urinary tract obstruction. In: Wein AJ, ed. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 41.

      O'Connor OJ, Maher MM. The urinary tract. In: Adam A, Dixon AK, Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging. 6th ed. New York, NY: Churchill Livingstone; 2015:chap 35.

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

      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: Elsevier Saunders; 2012:chap 45.