What Is Retroperitoneal Fibrosis?
Retroperitoneal fibrosis is a rare condition that’s also known as
Ormond’s disease. It occurs when excess fibrous tissue develops in the space
behind your stomach and intestine called the retroperitoneal area. Fibrosis is
the growth of excess connective tissue, which causes a mass to form. This often
causes compression and blockage of the ureters, which are the tubes that carry
urine from your kidneys to your bladder.
Tissue masses can block one or both of your ureters. When urine
backs up in the ureters, harmful materials can build up in your blood and
kidney damage can result. The disease can cause kidney failure if it isn’t
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.
Symptoms of Retroperitoneal Fibrosis
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. Symptoms that occur in the early stages of this condition include:
- dull pain in the abdomen or back that may be
hard to pinpoint
- pain on one side between your upper abdomen and
- leg pain
- discoloration in one or both legs
- swelling of one leg
- intense abdominal pain with bleeding or
Other symptoms may arise as the disease progresses, but certain
symptoms can occur at any stage. They include:
- severe abdominal or back pain
- appetite loss
- weight loss
- nausea or vomiting
- an inability to urinate
- reduced urine production
- impaired limb movement
- an inability to think clearly
- a low level of red blood cells, which is called
- kidney failure
You should see your doctor if you have reduced urine output with
abdominal or lower back pain. These can be symptoms of kidney damage.
Causes and Risk Factors of Retroperitoneal
According to the National
Organization for Rare Diseases, the exact cause of this condition is unknown
in about two-thirds of cases.
Age and gender are the greatest risk factors for the disease.
According to the National
Center for Biotechnology Information, 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.
According to Johns
Hopkins University, the disorder is associated with a specific condition in
10 to 25% of cases. These can include:
- actinomycosis, which is a bacterial infection
- histoplasmosis, which is a fungal infection
- recent trauma of the abdomen or pelvis
- abdominal or pelvic tumors
The disorder can also be associated with:
- recent surgery on the abdomen or pelvis
- the use of cancer treatments involving external
- certain medications to treat migraines and high
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.
Diagnosing Retroperitoneal Fibrosis
An accurate diagnosis requires the use of CT or MRI scans of your
Additional tests used to confirm the diagnosis include:
- blood tests to measure kidney function, anemia,
- an X-ray of the kidneys and ureters, which is
called an intravenous pyelogram
- an ultrasound of the kidneys
- a biopsy to check for cancer cells
Treatment for Retroperitoneal Fibrosis
Treatment varies depending on the severity and location of the
fibrosis. If you’re diagnosed in the early stages of the condition, you may be
prescribed anti-inflammatory medications, corticosteroids, or immunosuppressants.
If you’re diagnosed after fibrosis has blocked one or both of
your ureters, your doctor will need to clear the obstruction. This is done by
draining the urine with a stent, or drainage tube, inserted through your back
and into your 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 the blockage from happening again
The goals of treatment are to remove the blockage, repair the affected
ureter, and prevent it from happening again. For many people, treatment
requires both medication and internal intervention.
Long-Term Outlook for Retroperitoneal
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’s a 90 percent
chance of long-term success.
However, in cases where the kidneys are severely affected, damage
can be permanent, leading to the need for a kidney transplant.
How to Prevent Retroperitoneal Fibrosis
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 high blood pressure and medications to treat migraines
called ergotamines. Ask your doctor about the possible side effects of these
types of drugs and whether alternatives are available.