Retroperitoneal fibrosis

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Computed tomography scan of the abdomen of a 47-year-old woman with a history of fatigue and malaise associated with nausea, vomiting, lower back pain and acute renal failure.

Based on the patient's computed tomography scans, a diagnosis of retroperitoneal fibrosis was confirmed (Figure 1 and Figure 2). Retroperitoneal fibrosis, or Ormond's disease, is a rare, fibro-inflammatory disease with an estimated prevalence of 1–2 per 100 000 and male preponderance.1 It has indolent presentation with early, nonspecific symptoms, including back pain, fatigue, fever, weight loss and malaise. At a later stage, acute renal failure develops because ureteric “wrapping” from ceroid causes flow obstruction and hydronephrosis.2

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Figure 1: Computed tomography scan showing retroperitoneal mass (arrows).

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Figure 2: Axial computed tomography image showing retroperitoneal mass (arrows).

In our patient, hydronephrosis developed in the left kidney (Figure 3). Prednisolone therapy was started and a pigtail ureteric stent was inserted to drain the hydronephrotic kidney. The last follow-up took place 6 months after diagnosis, and the patient was doing well.

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Figure 3: Axial computed tomography scan showing hydronephrosis of the left kidney.

Most cases (~70%) of retroperitoneal fibrosis are idiopathic. In the remainder of cases, asbestos exposure, malignant diseases and lymphomas in the pelvic region, radiation therapy, ureteric rupture and medicines (typically methysergide and ergot alkaloids) have been incriminated as causative factors.

The diagnosis of retroperitoneal fibrosis can be confirmed noninvasively by means of either contrast-enhanced computed tomography or gadolinium-enhanced magnetic resonance angiography. Of note, gadolinium-based imaging should be avoided if the estimated glomerular filtration rate is less than 30mL/min because of concerns about gadolinium-induced nephrogenic systemic fibrosis. Many physicians advocate a computed tomography-guided biopsy to exclude malignant disease.