Retroperitoneal fibrosis is a rare condition where inflammation and fibrous tissue develops around the blood vessels and ureters in the retroperitoneum. Imaging with CT and MRI can show the abnormal tissue around these structures. Complications can involve blockages of the ureters and blood vessels.
What is retroperitoneal fibrosis?
Retroperitoneal fibrosis leads to development of inflammation and scar like tissue (fibrosis) in the retroperitoneum. The retroperitoneum is the space behind your abdominal cavity.
Retroperitoneal fibrosis involves the blood vessels (aorta and ivc) and ureters. Retroperitoneal fibrosis can be idiopathic (unknown cause) or occur as a result of medications, infection, radiation treatment, surgery and cancer.
Symptoms of retroperitoneal fibrosis
The symptoms are non specific and can include malaise, fever, weight loss, flank pain and low urine production.
Symptoms of blood vessel involvement can include clots in veins, lower extremity swelling, and poor arterial flow to the legs and intestines.
Some patients can have high blood pressure from involvement of the renal arteries.
How is retroperitoneal fibrosis diagnosed?
Retroperitoneal fibrosis is often diagnosed on CT and MRI as a mass in the retroperitoneum at the level of the fourth and fifth lumbar vertebra. The mass often involves the blood vessels and ureters.
Tissue biopsy may be needed to make the diagnosis and exclude other possibilities like cancer which can look similar.
Imaging findings that are classic for the condition may forego a biopsy. Imaging findings which are atypical may prompt a biopsy.
Blood tests may be abnormal but do not help make a definitive diagnosis. Blood tests for the kidneys may reveal some degree of dysfunction because of ureteral involvement.
What does retroperitoneal fibrosis look like on imaging?
Retroperitoneal fibrosis will be a mass that involves the retroperitoneum at the level of the lower lumbar spine. The mass will often surround the aorta, inferior vena cava and ureters. The ureters will often be deviated medially. The kidneys may be obstructed and have hydronephrosis. CT may also show features will are more suggestive of infection or cancer.
What else can look like retroperitoneal fibrosis in radiology?
Cancer like lymphoma can mimic retroperitoneal fibrosis. Certain infections like tuberculosis can also mimic the condition.
Idiopathic retroperitoneal fibrosis (Ormond’s disease)
Majority of cases of retroperitoneal fibrosis are idiopathic. This is called Ormond’s disease. This means there is no identifiable cause for the disorder.
There are many secondary causes which can include medications (nsaids and beta blockers), cancer, prior radiation therapy, and inflammatory conditions like pancreatitis.
Malignant retroperitoneal fibrosis
Cancers like lymphoma, carcinoid and sarcomas can be secondary causes of retroperitoneal fibrosis.
What causes retroperitoneal fibrosis?
Primary cause of retroperitoneal fibrosis or Ormond’s disease is not known. It is thought to be an autoimmune disease which causes inflammation and fibrosis around large vessels.
Numerous secondary causes have been implicated. Some of these include many medications, infections like tuberculosis, cancers, radiation therapy, and prior surgery.
Is retroperitoneal fibrosis dangerous?
It can be dangerous because it can cause kidney failure, blockages of blood vessels and mimic other conditions like cancer and infections.
Retroperitoneal fibrosis treatment
Treatment can be medical and surgical. An underlying cause for the condition should be sought. Medical treatment often consists of steroid treatment. This can last for as long as 2 yrs but helps with symptoms and causes the retroperitoneal mass to regress. Other immunosuppressants have been used as well.
Surgical treatments have been used when medical therapy is ineffective and to obtain a definitive diagnosis when cancer is suspected. Decompressing the obstructed kidneys is often an urgent matter.
What type of doctor treats retroperitoneal fibrosis?
A team of doctors is best to address this rare disorder. This can include internists, surgeons, radiologists and pathologists.
Retroperitoneal fibrosis: summary
Retroperitoneal fibrosis is a rare disorder characterized by inflammation and fibrosis in the retroperitoneum. The disorder can be idiopathic (Ormond’s disease) or secondary to medications, infections, and other conditions.
Retroperitoneal fibrosis is often diagnosed with imaging and confirmed with biopsy. Treatment consists of finding an underlying cause if possible, steroids, and surgery for treatment resistant cases.