What is the definition of Retroperitoneal Fibrosis?

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

What are the alternative names for Retroperitoneal Fibrosis?

Idiopathic retroperitoneal fibrosis; Ormond's disease

What are the causes for Retroperitoneal Fibrosis?

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

The cause of this problem is mostly unknown. It is most common in people aged 40 to 60. Men are twice as likely to develop the condition as women.

What are the symptoms for Retroperitoneal Fibrosis?

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 blood in the stool (due to death of intestinal tissue)

What are the current treatments for Retroperitoneal Fibrosis?

Corticosteroids are tried first. Some health care providers 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.

What is the outlook (prognosis) for Retroperitoneal Fibrosis?

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.

What are the possible complications for Retroperitoneal Fibrosis?

The disorder may lead to:

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

When should I contact a medical professional for Retroperitoneal Fibrosis?

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

How do I prevent Retroperitoneal Fibrosis?

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.

Male

REFERENCES

Comperat E, Bonsib SM, Cheng L. Renal pelvis and ureter. In: Cheng L, MacLennan GT, Bostwick DG, eds. Urologic Surgical Pathology. 4th ed. Philadelphia, PA: Elsevier; 2020:chap 3.

Nakada SY, Best SL. Management of upper urinary tract obstruction. In: Wein AJ, Kavoussi LR, Partin AW, Peters, CA, eds. Campbell-Walsh Urology. 11th ed. Philadelphia, PA: Elsevier; 2016:chap 49.

O'Connor OJ, Maher MM. The urinary tract: overview of anatomy, techniques and radiation issues. In: Adam A, Dixon AK, Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging. 6th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2015:chap 35.

Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 137.

Turnage RH, Mizell J, Badgwell B. Abdominal wall, umbilicus, peritoneum, mesenteries, omentum, and retroperitoneum. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. Philadelphia, PA: Elsevier Saunders; 2017:chap 43.

  • Condition: Non-Malignant Refractory Ureterostenosis
  • Journal: BMC urology
  • Treatment Used: Resonance and Allium Ureteral Stents
  • Number of Patients: 33
  • Published —
This study evaluated the safety and effectiveness of Resonance and Allium metallic stents in the treatment of patients with non-malignant refractory ureterostenosis (narrow ureter).
  • Condition: Woman with Contracted Bladder and Ormond's Disease
  • Journal: Urologiia (Moscow, Russia : 1999)
  • Treatment Used: Total Replacement of Both Ureters and Urinary Bladder by Ileal Conduit
  • Number of Patients: 1
  • Published —
This case report describes a woman diagnosed with contracted bladder and Ormond's disease treated with total replacement of the both ureters and urinary bladder by ileal conduit.