IS Case 264: Ormond's disease (retroperitoneal fibrosis)

Parul Patel, MD


Imaging Sciences URMC 2009
Publication Date: 2009-06-26

History

A 55-year-old male with a significant history for atherosclerotic disease presented with bilateral hydronephrosis.

Findings

Contrast enhanced CT of the abdomen demonstrated a soft tissue mass surrounding the abdominal aorta with entrapment of both ureters. Intravenous pyelogram demonstrates medial deviation of both ureters. There is also narrowing of both ureters in the mid portion and bilateral hydronephrosis.

Diagnosis

Ormond's disease (retroperitoneal fibrosis)

Discussion

Retroperitoneal fibrosis is a disease in which fibro-inflammatory tissue surrounds the abdominal aorta and iliac arteries. This fibro-inflammatory tissue then extends into the retroperitoneum and entraps the ureters and other nearby structures. In about two-thirds of cases of retroperitoneal fibrosis, the etiology is idiopathic. This disease however can also be secondary to the use of certain drugs, previous surgeries, radiation therapy, infections, or cancer. The idiopathic form of the disease is thought to be caused by a local inflammatory reaction to antigens in the atherosclerotic plaques of the aorta. This is the likely cause in our patient who has an extensive history of atherosclerotic disease.

Retroperitoneal fibrosis affects men two to three times more commonly than woman with a mean age of presentation at 50-60 years of age. Although there are no set diagnostic criteria for this disease, imaging studies are essential to the diagnosis and CT and MRI are the imaging modalities of choice. On CT, one will usually see a soft tissue mass surrounding the abdominal aorta and iliac arteries with possible encasement of the ureters and inferior vena cava. Intravenous pyelograms usually demonstrate a triad of findings which include medial deviation of the ureters with extrinsic compression of the ureters and hydronephrosis. Raised concentrations of acute phase reactants in the blood also raise the possibility of retroperitoneal fibrosis in a patient with these imaging findings. Although the pathogenesis of this disease is not clearly defined, the main mechanism involved is thought to be periaortitis with advanced aortic atherosclerosis, medial thinning and significant adventitial and periaortic inflammation and fibrosis.

Clinically, patients present with localized and systemic manifestations of the disease. Side, back or abdominal pain are the most common localized symptoms of the disease. The pain is usually described as dull and constant, however if the ureters are involved, it may be described more as a colic pain. Hematuria, polyuria and urinary tract infections (UTI) are also reported, and our patient was treated for a UTI prior to the diagnosis.

References

  1. Amis ES Jr. Retroperitoneal fibrosis. AJR Am J Roentgenol. 1991 Aug;157(2):321-9. PMID: 1853816 [PMID: 1853816]
  2. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006 Jan 21;367(9506):241-51. PMID: 16427494 [PMID: 16427494]

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