Idiopathic Retroperitoneal Fibrosis

At NYU Langone Medical Center, significant work has been done in the evaluation and treatment of idiopathic retroperitoneal fibrosis. We have developed a multidisciplinary team that includes rheumatologist Dr. Bruce Solitar and urologic surgeon Dr. Michael Stifelman. Our approach also incorporates advanced imaging techniques such as PET and MRI, as well as laboratory evaluation, often including a tissue biopsy.

Definition

Retroperitoneal fibrosis is a rare autoimmune, inflammatory disorder that affects the area behind the stomach and intestines. In this disorder, a mass of fibrous tissue develops around the aorta and often blocks the ureters (the tubes that carry urine from the kidneys to the bladder). Over time, obstruction of the ureters can cause buildup of toxic chemicals in the blood and kidney failure. Kidney damage may be temporary or permanent if not recognized and repaired quickly.

Causes

The causes of retroperitoneal fibrosis are not certain. Some studies have linked retroperitoneal fibrosis with long-term use of the drug methysergide (found in some in migraine medications). At NYU Langone, we have found that a deep biopsy of this tissue is required to diagnose this disease and rule out an underlying malignancy.

Risk factors

Retroperitoneal fibrosis is most common in individuals from 50 to 70 years of age. Men are more likely to develop the condition than women.

Symptoms

Early symptoms include:

Pain in the abdomen
Pain in the legs
Swelling and/or change in color of one leg
Back pain

Later symptoms include:

Decreased urine output
No urine output (anuria)
Nausea or vomiting
Changes in thinking
Severe abdominal pain

Diagnosis of retroperitoneal fibrosis

A diagnosis of retroperitoneal fibrosis almost always requires a CT scan or MRI. At NYU Langone, we make use of specialized MRI protocols and PET scans to diagnose and stage the disease. In addition, we have found that a biopsy of the mass is required to make the diagnosis and to rule out the possibility of cancer.

Treatment of retroperitoneal fibrosis

Treatment of retroperitoneal fibrosis depends on the amount of ureteral involvement and the individual patient’s response to medical therapy.

Medical treatment of retroperitoneal fibrosis

Typically, the first line of treatment for retroperitoneal fibrosis is an anti-inflammatory medication known as a corticosteroid. Immunosuppressive medicines may also be prescribed. Dr. Bruce Solitair, an NYU rheumatologist with a special interest in retroperitoneal fibrosis, creates an individualized regimen of medical therapy for each patient based on the results of blood tests, imaging studies and pathology results. Patients are followed regularly and medications adjusted based on follow-up imaging studies and blood tests.

Surgical treatment of retroperitoneal fibrosis

If medication does not help alleviate the condition, surgery may be necessary to free and protect the obstructed ureters. In this operation, the ureter is first freed from the fibrotic tissue. Then, a piece of the omentum (fat that overlies the intestine) is wrapped around the ureter. The omentum acts as a ‘buffer’ to protect the ureter from being overgrown again with fibrotic tissue.

Surgery may be done by an open, laparoscopic or robotic approach. In the traditional ‘open’ surgery to treat retroperitoneal fibrosis, a two- to three-foot incision is needed, stretching from the pubic bone to the chest bone. The open surgical approach requires an extended hospital stay and a 6- to 8-week convalescence period.

At NYU Langone Medical Center, Dr. Michael Stifelman and his team have pioneered the use of robotic surgery for the treatment of retroperitoneal fibrosis. This operation produces the same results as open surgery but requires only four quarter-inch incisions in the abdomen wall, meaning less trauma to the body and a shorter recovery period. Dr. Stifelman was the first to publish on robotic ureterolysis with omental wrapping and has lectured throughout the world on this topic.