Surgical alleviation of ureteric obstruction in idiopathic retroperitoneal fibrosis. An analysis of 9 cases

Nine patients with retroperitoneal fibrosis were reviewed. All patients had idiopathic disease, bilateral in eight patients. An IVP on admission was normal in only one of 17 kidneys with delayed excretion in seven. The upper urinary tract was dilated in eight kidneys and no excretion of contrast medium was seen in four; another four kidneys were not examined by IVP because of uremia. Surgical alleviation of ureteric obstruction was carried out by ureterolysis alone (2 ureters), ureterolysis and lateral displacement of the ureter (2 ureters), ureterolysis and neoimplantation into the bladder (2 ureters), ureterolysis and intraperitonealization (2 ureters), and ureterolysis and omental wrap (7 ureters). After surgery, an IVP showed normal excretion in six of the seven kidneys with delayed excretion preoperatively. Two of the four preoperatively nonfunctioning kidneys also showed normal excretion at IVP. Of the four kidneys not preoperatively examined by IVP because of uremia two showed a normal excretion on postoperative IVP. Two ureters developed recurrent obstruction and required additional surgery whereas 10 ureters remained unobstructed in the follow-up period of 26-89 months. Our experience suggests that complete and extensive ureterolysis combined with omental wrapping gives the best results on a long-term basis.     

原发性腹膜后纤维化19例报告

目的 提高原发性腹膜后纤维化患者临床治疗效果.方法 原发性腹膜后纤维化患者19例.临床表现腰腹部疼痛、恶心、食欲不振,乏力、体重下降、尿量减少,体检发现双肾积水肾区叩击痛等.影像学检查表现为腹膜后软组织肿块,沿腹主动脉前方及两侧分布,包绕输尿管和下腔静脉.行开放手术9例,其中双输尿管松解加腹腔间置术7例,松解失败行肾造瘘术者2例;经膀胱镜或输尿管镜置入双J管引流10例.辅以泼尼松口服治疗17例,60 mg/d连续应用6周,每周减5 mg,至维持量10 mg/d至少应用6个月,总疗程＞12个月.结果 19例患者获随访17例,失访2例.随访时间5～81个月.行输尿管松解并腹腔间置术获随访者6例,肾积水减轻或基本消失,SCr降至正常水平或稳定在轻度升高的代偿状态.行肾造瘘术2例,分别于术后3、7个月反复发生尿路感染,1例需要血液透析治疗.行双J管引流者9例,患者症状消失,肾积水消失或明显减轻,SCr值平均(103±48)μmol/L;拔除双J管后3例复发再次置入双J管,2例再拔管后症状无复发,1例仍保留双J管.结论 输尿管松解腹腔间置是治疗原发性腹膜后纤维化的有效手段;双J管引流、口服泼尼松方法简单、创伤小、近期疗效较满意,远期效果有待进一步观察.     

Treatment of idiopathic retroperitoneal fibrosis]

Idiopathic retroperitoneal fibrosis is a rare disease and a delay in diagnosis may cause renal failure. Medical treatment i.e. corticotherapy or more recently, tamoxifene has been used successfully. This approach is recommended in patients either with moderate obstruction of the upper urinary, risk of major surgery or in cases of recurrence after surgical treatment. Ureterolysis using conventional surgery or laparoscopy remains the treatment of choice. This procedure should be considered in patients with neoplasic fibrosis, corticoresistant fibrosis or in cases of peri-aneurysm fibrosis.     

Mediastinal fibrosis in a patient with idiopathic retroperitoneal fibrosis

Combined idiopathic retroperitoneal-mediastinal fibrosis is rare. We report a case of mediastinal fibrosis that followed the onset of retroperitoneal fibrosis by six years. A 61-year-old asymptomatic woman was diagnosed with idiopathic mediastinal fibrosis in December of 2006 after discovering a 1.4 cm thick prevascular mass encasing the aortic arch. In August of 2001 the patient had been diagnosed with retroperitoneal fibrosis, which was successfully treated surgically. An axillary thoracotomy found dense adhesions that fixed the arch of the aorta to the adjacent lung. Mediastinal biopsies were consistent with idiopathic fibrosis. We describe the imaging of this case and briefly review the literature.     

Renal autotransplantation for idiopathic retroperitoneal fibrosis

Extensive loss of the ureter is a potential complication of surgery performed to relieve ureteral obstruction caused by retroperitoneal fibrosis. The authors describe the twelfth case managed successfully by renal autotransplantation and review the literature.     

Medical therapy in idiopathic retroperitoneal fibrosis.

Except for its linkage with the ergot derivatives retroperitoneal fibrosis is a disease of unknown etiology. Two cases in which the traditional surgical approach was used but with the subsequent use of steroids are presented. The third patient was treated with steroids alone. The use of steroids alone is suggested for a limited number of poor risk patients when certain criteria are met.     

Laparoscopic ureterolysis for idiopathic retroperitoneal fibrosis

Three patients with idiopathic retroperitoneal fibrosis underwent laparoscopic ureterolysis. Two patients were bilateral cases which were performed successfully as a one stage procedure. Another patient, who was unsuccessfully treated, had a long ureteral stricture. Laparoscopic ureterolysis may be a useful alternative to open surgical management especially in bilateral cases, except for patients with a long ureteral stricture.     

Innovative surgical management of idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis is a rare entity usually treated with exploratory laparotomy, deep biopsies of the fibrotic process and uretrolysis. Innovative surgical management occasionally is required for ureteral obstruction. We report the use of dismembered pyeloplasty, autorenal transplantation and bilateral psoas hitch ureteral reimplantation for the management of ureteral obstruction associated with idiopathic retroperitoneal fibrosis.     

Treatment of idiopathic retroperitoneal fibrosis by immunosuppression

Idiopathic retroperitoneal fibrosis is exceedingly uncommon in childhood and its etiology is uncertain. Support for an immunological basis for the disease is given by a report of a 14-year-old girl with severe retroperitoneal fibrosis causing progressive azotemia in whom azathioprine and prednisolone were used successfully. This case supports the efficacy of immunotherapy in the treatment of idiopathic retroperitoneal fibrosis.     

Peritoneal flap ureteropexy for idiopathic retroperitoneal fibrosis

Eight patients had 13 ureters treated by peritoneal flap ureteropexy. There were no significant post-operative complications. Eleven ureters were functioning normally after an average follow-up of 28 months. The operation is suggested as a method of choice where omental wrapping is not possible.