腹膜后纤维化1例并国内文献分析

Retroperitoneal fibrosis: one case report and domestic literature reviews

目的: 分析国内对腹膜后纤维化(RPF)的诊断、治疗状况,以提高对RPF的早期诊断率。方法: 回顾总结了我院1例和国内报道的114例RPF患者的诊断、治疗和预后情况。结果: (1) 该病患者男∶女为1.8∶1,腰背部疼痛80 例(69. 57%),肾积水87 例(75. 65%),肾功能损害60 例(52. 17%),上段输尿管扩张49 例(42.61%),下肢水肿29例(25.22%),高血压26例(22.61%),少尿、无尿24例(20.87%)。(2) CT对诊断腹膜后软组织异常、B超对诊断肾积水和输尿管扩张非常有价值。本组96.52%的患者是依靠剖腹探查、组织学检查确定诊断的。(3) 外科治疗的主要目的是解除输尿管梗阻和防止再狭窄,应用糖皮质激素是内科治疗的主要手段。结论: RPF起病隐匿,临床表现无特异性,需依靠剖腹探查和组织学检查确诊;早期应用糖皮质激素治疗效果较好,免疫抑制剂和他莫西芬的应用尚在探索中;晚期出现器官压迫和梗阻时需手术治疗。

Objective: To retrospect the domestic status of diagnosis and treatment of retroperitoneal fibrosis (RPF). Methods: A total of 115 cases with RPF that included 1 case from our hospital and 114 cases from domestic literature were reviewed for analyzing the clinical manifestations, diagnosis, treatment and prognosis of this disease. Results: The ratio of male patients to female in the group was about 1.8∶1. Frequent clinical symptoms and signs: back and flank pain(69.57%),hydronephrosis(75.65%),deficiency of renal function(52.17%),dilatation of superior segment ureter(39.13%),edema of lower extremities (25.22%) , hypertension(22.62%),and oliguria or anuresis(20.87%). For abnormality in retroperitoneal space, CT was a valuable diagnostic method. Type B ultrasonic was sensitive to diagnose hydronephrosis and dilatation of superior segment ureter. The final diagnosis of 96.52% patients in this group was depended on exploratory laparotomy and pathological section examination. The purpose of surgical treatment was to relief ureterostenoma and to prevent its recurrence. The use of glucocorticoid was main process of medical treatment. Conclusions: Retroperitoneal fibrosis conceals its manifestation on onset stage. Its clinical situation is unspecific. Biopsy is needed to make the final diagnosis. Therapy of glucocorticoid on early stage has better curative effect. It is exploring that whether immunodepressants and tamoxifen are good for treating the disease. Surgical therapy should be resorted in the advanced stage of RPF to remove organ compression or obstruction.