Retroperitoneal fibrosis is a rare condition in which the tubes that carry urine from the kidneys to the bladder, called ureters, are blocked by masses located in the back of your abdominal cavity. This space, located behind your stomach and intestines, is called the retroperitoneal area. The disorder causes inflammation and fibrosis. Fibrosis is the growth of excess connective tissue, which causes a mass to form.
Tissue masses can block one or both of the ureters. When urine backs up in the ureters, harmful materials can build up in your blood and kidney damage can result. Without treatment, the disease can cause kidney failure.
The condition typically starts with inflammation and fibrosis of the abdominal aorta. The abdominal aorta is the large artery that brings blood from your heart to the areas below your kidneys. As the disease progresses, it affects the arteries that carry blood to your legs and kidneys. Pain, leg swelling, and a reduction in kidney function can occur.
The condition is also known as Ormond’s disease.
According to the National Organization for Rare Diseases (NORD), the exact cause of this condition is unknown in about two-thirds of cases (NORD, 2008).
Age and gender are the greatest risk factors for the disease. According to the National Center for Biotechnology Information (NCBI), it occurs most often between the ages of 40 and 60. However, it can develop at any age. The condition occurs twice as often in men as in women (NCBI, 2011).
Research from Johns Hopkins suggests that among a smaller group of people—roughly 10 to 25 percent of patients—the disorder is associated with the following factors:
- actinomycosis (a bacterial infection)
- histoplasmosis (a fungal infection)
- cancer treatments involving external beam radiation
- recent surgery on the abdomen or pelvis
- recent trauma to the abdomen or pelvis
- use of certain medications to treat migraines and high blood pressure
This disorder results in decreased blood flow from the aorta to the lower part of your body. Initially, your body reacts to the reduced blood flow.
The following symptoms occur in the early stages of this condition:
- dull pain in the abdomen or back that may be hard to pinpoint
- pain on one side between your upper abdomen and back
- leg pain
- discoloration in one or both legs
- swelling of one leg
- intense abdominal pain with hemorrhaging (bleeding)
Other symptoms may arise as the disease progresses; however these symptoms can occur at any stage. They include:
- severe abdominal or back pain
- appetite loss
- weight loss
- nausea and/or vomiting
- inability to urinate
- reduced urine production
- impaired limb movement
- inability to think clearly
- anemia (low level of red blood cells)
- kidney failure
Contact your physician if you have reduced urine output with abdominal or low back pain, which can indicate kidney damage.
The complications associated with this disease vary. The size and location of the excess tissue growth can cause damage to various areas served by the abdominal aorta.
If this condition goes untreated, the most serious problems result from swelling and blockage of the ureters. This may result in chronic kidney failure and long-term blockage of the ureters, which can cause urine backup and kidney swelling.
An accurate diagnosis requires the use of computed tomography (CT) or magnetic resonance imaging (MRI) scans to examine your abdomen.
Additional tests used to confirm the diagnosis include:
- blood tests to measure kidney function, anemia, and inflammation
- intravenous pyelogram (IVP): an X-ray of the kidneys and ureters
- ultrasound of the kidneys
- biopsy to check for cancer cells
Treatment varies depending on the severity and location of the fibrosis. If you are diagnosed in the early stages of the condition, the following medications typically are prescribed:
- anti-inflammatory medications or corticosteroids
If diagnosis occurs after fibrosis has blocked one or both ureters, the obstruction must be cleared. This is done by draining the urine with a stent, or drainage tube, inserted through your back and into the kidney. A stent may also be run from your bladder through the ureter into the kidney.
In some cases, surgery may be required. It may be used to:
- free the affected ureter from the fibrosis
- wrap the affected ureter in fat tissue from the intestines to protect it from fibrosis regrowth
- reposition the affected ureter away from the inflammation to prevent recurrence
The goals of treatment are to remove the blockage, repair the affected ureter, and prevent recurrence. For many people, treatment requires both medication and internal intervention.
If the condition is diagnosed and treated at an early stage, the long-term outlook for patients can be very good. When kidney damage is minimal and surgery is successful, there is a 90 percent chance of long-term success (Amis, 1991).
However, in cases where the kidneys are severely affected, damage can be permanent, leading to the need for a kidney transplant.
Since the majority of cases cannot be linked to any specific cause, prevention may not be possible. However, the condition is associated with the use of some medications to treat migraines and high blood pressure. Ask your doctor about the possible side effects of these types of drugs and whether alternatives are available.