Tralce Luigi, Antonelli Alessandro, Dotti Paolo, Cunico Sergio Cosciani
Cattedra di Urologia, Università degli Studi di Brescia, Brescia, Italy.
Arch Ital Urol Androl. 2004 Sep;76(3):135-9.
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.
腹膜后纤维化（RF）是一种罕见疾病，估计发病率为每20万居民中有1例。特发性RF（占病例的2/3）必须与由六氯化合物摄入、放射治疗和各种疾病引起的继发性RF区分开来。RF的治疗尚未明确界定。1984年至2002年，我们观察了15例患者（1例特发性腹膜后纤维化），即27个输尿管单位（1例患者先前已行肾切除术，2例表现为单侧输尿管受累）。平均年龄为55岁，男女比例为0.66:1。最常见的症状是急性肾衰竭或肾绞痛。14例患者术前需要通过肾造瘘术或输尿管支架置入进行尿液引流。6例患者接受类固醇或他莫昔芬治疗但无反应，因此所有15例患者均接受了手术。6例患者在8个月后失访，另外9例接受了平均84个月（范围12 - 168个月）的临床和放射学检查，其中8例排泄系统显示正常管道形成或持续扩张。1例患者在输尿管松解术和腹膜内固定术后出现复发性输尿管狭窄，需要行输尿管切除术并置入回肠。在1例单侧表现的病例中，5年后发现对侧狭窄。根据文献数据，能确保我们获得最佳结果的手术技术是输尿管松解术并使用网膜包裹输尿管。根据我们的经验，药物治疗效果不佳。然而，考虑到RF的发病率较低，有必要进行前瞻性多中心研究以确定最适合每位患者的治疗方法。对于单侧表现的患者，必须延长随访时间以尽早发现可能的对侧受累情况。
