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.
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.
- 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
- 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.
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.
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.
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.
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.
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