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    Retroperitoneal fibrosis

    Idiopathic retroperitoneal fibrosis; Ormond's disease

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

    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 - 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)

    Exams and Tests

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

    Other tests that can help diagnose this condition include:

    • BUN and creatinine blood tests
    • Intravenous pyelogram (IVP) - not as commonly used
    • Kidney ultrasound
    • MRI of the abdomen
    • CAT scan of the abdomen andretroperitoneum

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

    Treatment

    Corticosteroids are tried first. Some doctors alsoprescribe 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 systemcan beprescribed.

    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:

    • Ongoing blockage of the the tubes leading from the kidney on one or both sides
    • Chronic kidney failure

    When to Contact a Medical Professional

    Call your health care provider if youhave 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.

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          Tests for Retroperitoneal fibrosis

            Review Date: 5/7/2013

            Reviewed By: 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.

            The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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