Epidemiology, clinical features and treatment of idiopathic retroperitoneal fibrosis: our experience

Retroperitoneal fibrosis (RF) is an uncommon disease with an estimated incidence of 1 case on 200.000 inhabitants. Idiopathic RF (2/3 of cases) must be distinguished from secondary RF, caused by hexogen substances assumptions, radiotherapy and various diseases. The treatment of RF is not yet univocally defined. From 1984 to 2002 we observed 15 patients (1 case with idiopathic retroperitoneal fibrosis) i.e. 27 ureteral units (1 patient was previously nephrectomized and 2 showed a monolateral ureteral involvement). The mean age was 55 and the male:female ratio was 0.66:1. The commonest presenting symptoms were acute renal failure or renal colic. 14 patients needed preoperatory urinary drainage by nephrostomy or ureteral stenting. 6 patients were treated with steroids or tamoxifen without response so that all the 15 patients underwent surgery. 6 patients were lost to follow-up after 8 months while the other 9 underwent clinical and radiological controls for a mean time of 84 months (range 12-168) with evidence of normal canalization or steady dilatation of the excretory system for 8 of them. 1 patient developed a relapsing ureteral stenosis after ureterolysis and intraperitonealisation and needed ureterectomy with ileal interposition. In a case with monolateral presentation a controlateral stenosis was evidenced 5 years later. According to literature data, the surgical technique that ensured us the best results was ureterolysis with omental wrapping of the ureter. In our experience, medical treatments gave poor outcomes. However, in consideration of the low incidence of RF, perspective and multicentric studies are necessary to establish which is the most suitable treatment for each patient. Follow-up must be extended in patients with monolateral presentation to precociously reveal a possible contralateral involvement.