Retroperitoneal fibrosis is a condition characterized by the proliferation of fibrosis in the retroperitoneum. It is also known as Ormond’s disease and usually presents with the presence of flank pain. The condition is named after John Kelso Ormond, a urologist who published information regarding retroperitoneal obstruction.
Retroperitoneal fibrosis may either be benign or malignant and has the tendency to metastasize. It may lead to renal failure if the disease progresses towards the kidney and other surrounding structures. It is a collagen vascular disorder affecting urine flow from the kidneys to the urinary bladder by an obstruction in the ureters.
Causes Of Retroperitoneal Fibrosis
Retroperitoneal fibrosis may be classified as primary or secondary based on the presenting signs associated with the condition.
- In primary retroperitoneal fibrosis, the cause is idiopathic or unknown. However, it has been linked to certain autoimmune conditions as an immunosuppression response.
- Secondary retroperitoneal fibrosis has been associated with certain conditions like malignancy and infections such as actinomycosis, histoplasmosis and tuberculosis as well as medications including methysergide, hydralazine, beta-blockers, ergotomines, pergolide and bromocriptine. Exposure to external beam radiation therapy, a form of treatment used in cancer, has also been associated with the disease. Trauma or surgery to the abdomen or pelvis may also predispose a patient to the condition. Around 10-25% of the cases usually have an identifiable secondary condition.
Signs and Symptoms
- Early signs and symptoms of retroperitoneal fibrosis are usually nonspecific but would usually include abdominal or lumbar discomfort or dullness. Severe pain may be possible in the abdomen, lower bank and flank areas. The decrease in blood flow would cause pain sensation and a change in the color of the legs.
- A late symptom would include a decrease in the urine out until such time as anuria occurs. This is usually indicative of renal or urethral involvement. Nausea, vomiting and mental confusion may occur possibly caused by renal failure and build-up of toxins in the blood. Severe abdominal pain may also present due to hemorrhage brought about by the death of intestinal tissues. Men may experience testicular swelling while women may present with endometriosis secondary to anatomic distortion.
The diagnosis for retroperitoneal fibrosis is usually delayed because of the absence of clinical manifestation which usually prompts a patient to be checked by a physician.
- Radiographic examination is usually the first step in establishing the diagnosis of retroperitoneal fibrosis. Computed tomography (CT scan) and magnetic resonance imaging (MRI) may also be used. Some physicians consider abdominal CT scan as the best method to reveal retroperitoneal fibrosis.
- Blood samples may be used to determine the serum creatinine levels and serum blood urea nitrogen (BUN). These blood tests are necessary to determine and evaluate kidney function.
- Intravenous pyelogram may also be indicated to evaluate blood in the urine as well as presence of kidney stones and tumors. This, however, would utilize an iodine-contrast dye which some patients may be allergic to.
- Kidney ultrasound is also necessary to diagnose and monitor tumor growth.
- Biopsy of the retroperitoneal fibrosis may also be done to eliminate the possibility of tumor malignancies.
- Complete blood count is also necessary to check for the presence of anemia especially if hemorrhage occurs as well as identify markers of inflammation in the blood.
Treatment for Retroperitoneal Fibrosis
The best form of treatment for retroperitoneal fibrosis is not known. The treatment plan would usually depend on the size and type of mass involved as well as the involvement of other surrounding tissues.
- Surgery with omental wrapping may be generally indicated for patients with severe urinary obstruction.
- If there is a blockage of urine caused by the inflammation and fibrosis in the ureters, a tube or stent may be placed in the kidneys through the back to allow the flow of urine.
- For cases premeditated by an autoimmune response, immunosuppressive therapy may be indicated. This may be done through the use of glucocorticoids. Tamoxifen has been shown efficacy in treating retroperitoneal fibrosis although the mechanism of its action is still not fully known.
The prognosis for retroperitoneal fibrosis would depend on the extent of the fibrosis as well as the invasion and damage to other surrounding organs especially to the kidneys. The extent of kidney damage may either be temporary or permanent.
When to contact the doctor?
It is therefore important to have a physical examination done by a physician as soon as flank pain is experienced as well as a decrease in the urinary output or anuria is noted.
Retroperitoneal fibrosis is a condition that would require prompt treatment at the earliest possible time to have a good prognosis as well as to prevent damage to other organs especially the kidneys. Other complications like chronic kidney failure and chronic obstructive uropathy may be prevented if retroperitoneal fibrosis is diagnosed and treated early.