Retroperitoneal fibrosis

• Alternative Names

  Idiopathic retroperitoneal fibrosis; Ormond's disease

• Causes, incidence, and risk factors

  Retroperitoneal fibrosis is a rare disorder that occurs when extra fibrous tissue forms in the area behind the stomach and intestines. The excess tissue forms a mass (or masses) that can block the tubes that carry urine from the kidney to the bladder. Doctors don't know why these masses form. 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 method to reveal a retroperitoneal mass.

  Other tests that can help diagnose this condition include:

  • BUN and creatinine blood tests
  • Intravenous pyelogram (IVP)
  • Kidney ultrasound
  • MRI of the abdomen

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

• 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.

• 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

  The disorder may lead to:

  • Chronic bilateral obstructive uropathy
  • Chronic kidney failure
  • Chronic unilateral obstructive uropathy
• 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 long-term use of medications that contain methysergide, which has been shown to cause retroperitoneal fibrosis. Methysergide is sometimes used to treat migraine headaches.

• References

  Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 21-27 Jan 2006;367(9506);241-251.

  Turnage RH, Richardson KA, Li BD, McDonald JC. Abdominal wall, umbilicus, peritoneum, mesenteries, omentum, and retroperitoneum. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2008:chap 43.

  Pais VM, Strandhoy JW, Assimos DG. Pathophysiology of urinary tract obstruction. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 37.

  Hsu THS, Streem SB, Nakada SY. Management of upper urinary tract obstruction. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 38.

Review Date: 2/9/2009

Reviewed By: Linda Vorvick, MD, Family Physician, Seattle Site Coordinator, Lecturer, Pathophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Louis S. Liou, MD, PhD, Assistant Professor of Urology, Department of Surgery, Boston University School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.