The etiology of idiopathic retroperitoneal fibrosis is unknown. The condition manifests itself at first by vague abdominal discomfort. Diagnosis is important because a majority of these patients will recover by preserving renal function using ureteral lysis or occasionally by diversion procedures. Occlusion ureterograms are most important in evaluating the disease. Many other diseases can mimic idiopathic retroperitoneal fibrosis and can only be distinguished by generous biopsies taken at operation. One patient with idiopathic retroperitoneal fibrosis, a man aged 72, needed a second operation with intraperitoneal lysis of both ureters before an apparent cure resulted. A second patient, a woman aged 52, obtained a dramatic result following x-ray therapy. The variable and capricious course the disease may run requires frequent follow-up examinations over many years.
Harrow BR, Sloane JA. Idiopathic Retroperitoneal Fibrosis. JAMA. 1962;182(1):38–43. doi:10.1001/jama.1962.03050400040007
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