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What Is Retroperitoneal Fibrosis?

Medically Reviewed by Dan Brennan, MD on June 23, 2021

Retroperitoneal fibrosis occurs when tissue grows on or around your abdomen but not within your peritoneum (the membrane surrounding your organs of digestion). It can affect veins and arteries, among other pathways in your body. 

Retroperitoneal fibrosis is also referred to as Ormond’s disease. The abnormal, growing tissue may or may not be cancerous.‌

This condition develops slowly with time and most often affects middle-aged men, though it is rare even for them. It occurs behind (hence: retro) the peritoneum. The fibrous tissue can then block the ureters (tubes that go from your kidneys into your bladder) and other organs in the abdomen.

Causes

Some medical experts have suggested that the state of your immune system has something to do with the formation of this condition.‌

Other possible causes of retroperitoneal fibrosis include:

  • Cigarette smoking
  • Trauma
  • Surgery
  • Exposure to asbestos
  • Infections
  • Tumors (neoplasms)
  • Radiotherapy
  • Medications
  • Use of cocaine and other illegal substances‌

Retroperitoneal fibrosis rarely occurs to more than one individual in the same family. Whether you can inherit the condition is not clear. Most often, its occurrences are random.

Symptoms of Retroperitoneal Fibrosis

Different people may experience different symptoms. Symptoms depend on how much the growth has grown or spread and where it's located. The condition may get worse with time.‌

During the first days, you may experience a dull abdominal pain that increases every day. Your lower limbs may start to change color (because of poor blood circulation), and one lower limb may swell.‌

Other symptoms of retroperitoneal fibrosis, some more common than others, include:

  • Nausea and vomiting
  • High body temperature (fever)
  • Pain in the scrotum
  • Feeling thirstier than usual
  • Feeling generally unwell
  • Back pain
  • Weight loss
  • Urination issues — difficulty, lower volume, blood in urine, excess urination, or a constant need to urinate at night

When should you call your doctor? Tell your doctor if you have pain in the flank or lower abdomen. Seek their help if you start urinating much less than usual.

Diagnosis

Your doctor may take your medical history and do a physical examination. They may require tests to confirm retroperitoneal fibrosis and not some other disorder that involves dull belly pain. These tests may include:

  • Blood tests
  • Ultrasound of the kidney
  • Urine tests
  • MRI (magnetic resonance imaging) of the abdomen
  • X-ray(s) 
  • CT (computed tomography) scan of the abdomen
  • Contrast examination
  • Biopsy and other exploratory surgical procedures

Another test that could be used to confirm a retroperitoneal fibrosis diagnosis is the intravenous pyelogram (IVP). This special type of X-ray is not, however, commonly used today.

Treatment

Treatment for retroperitoneal fibrosis greatly relies on what caused your condition, how severe it is, and how much damage it has done so far. Most damage happens when there is a complication. Treatment methods that may work include:

  • Anti-inflammatory prescriptions, such as corticosteroids
  • Treating the situation that brought about the retroperitoneal fibrosis 
  • Removing the growth surgically
  • Draining the kidneys (done using a tube through your skin)
  • Managing any complications that may occur
  • Medications that keep your immune system working as it should‌

How does treatment progress? This also depends on how severe your condition was when you started treatment and how long you had retroperitoneal fibrosis. Healing of the kidneys begins by the second week. The condition rarely gets to renal (kidney) failure, which would require a kidney transplant.‌

Most cases of retroperitoneal fibrosis heal well.  However, even after treatment, you should get checked for the condition on a regular basis. 

Retroperitoneal fibrosis has been seen to recur in some cases, even years after treatment. Ureter obstruction may recur in about half of all the people who had surgery.

Also, people with noncancerous (benign) retroperitoneal fibrosis have a better survival chance than those with cancerous (malignant) retroperitoneal fibrosis. Malignancy may even lead to death. ‌ 

Complications of Retroperitoneal Fibrosis

If left untreated for long, retroperitoneal fibrosis may develop into complications that can be hard to manage. Some of these complications include:

Other, rarer complications include:

  • Blood clot formation 
  • High blood pressure (hypertension)
  • Swelling of the scrotum

Prevention

To avoid getting retroperitoneal fibrosis, don't use medication containing methysergide for long. This drug is used as a treatment medication for migraine headaches. However, researchers have found that the drug may lead to retroperitoneal fibrosis.‌

If you suspect that what you're feeling may be retroperitoneal fibrosis, you should consult with a professional as fast as possible. Early detection is vital to at least prevent complications and for medical treatment to work. 

WebMD Medical Reference

Sources

SOURCES: ‌

BetterHealth Channel: “Retroperitoneal fibrosis.”‌

Genetic and Rare Diseases Information Center: “Retroperitoneal fibrosis.”‌

Medline Plus: “Retroperitoneal Fibrosis.”‌

Mount Sinai: “Retroperitoneal fibrosis.”    

RöFo: Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin: ‌"Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging."

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