A case of retroperitoneal fibrosis responding to steroid therapy]

A 73-year-old man presented with acute renal failure due to bilateral hydronephroses. Urography and CT scan revealed stenosis and medial deviation of the bilateral ureters surrounded by a soft tissue mass at the level of the 4-5th lumber vertebrae. Under a diagnosis of retroperitoneal fibrosis, bilateral nephrostomies were temporarily placed and prednisolone administration was initiated at a dose of 20 mg/day. Ureteral passage markedly improved within 2 weeks in association with a decrease in size of the retroperitoneal soft tissue mass. The steroid therapy was continued for 4 months with decreasing dosing schedule. The patient has been doing well without any signs of recurrence for 3 months after the cessation of the steroid therapy.     

A case of retroperitoneal fibrosis demonstrating a remarkable response to steroids

We present a case of idiopathic retroperitoneal fibrosis that demonstrated a remarkable response to steroids. The patient, a 73-year-old man, complained of left flank pain and weight loss. Erythrocyte sedimentation rate was elevated and both CRP and antinuclear factor were positive. DIP showed left hydronephrosis, which proved to be due to the stenosis of the left ureter at the level of L5 by retrograde pyelography. Then abdominal computed tomography revealed a large retroperitoneal mass with the density of soft tissue in which the left ureter was involved. Based upon the above findings, we made the diagnosis of idiopathic retroperitoneal fibrosis. After confirming histological diagnosis by biopsy from the mass, we performed left ureterolysis. Since then, he has been treated with steroid administration. Now we can recognize a remarkable reduction of the mass in abdominal CT and improvement of the left renal function in DIP. We reviewed the association of autoimmune disease with idiopathic retroperitoneal fibrosis and recent therapies for this disease.     

A case of idiopathic retroperitoneal fibrosis accompanied by asynchronous bilateral urinoma

The patient was a 68-year-old female with the complaint of left flank pain. On examination, hydronephrosis of the left kidney and the urinoma of left retroperitoneal space were disclosed by abdominal computed tomographic (CT) scan. The urinoma disappeared spontaneously, but 4 months later right hydronephrosis and the urinoma of ipsilateral retroperitoneum emerged. Enhanced CT scan revealed aneurysm of bilateral common iliac arteries and perianeurysmal fibrotic mass. After steroid therapy under the diagnosis of idiopathic retroperitoneal fibrosis (IRPF), the fibrotic mass reduced in size and the urinoma disappeared. We should be aware that urinomas, which are thought to be caused by sudden obstruction of the upper urinary tract, could be brought about by IRPF.     

Idiopathic retroperitoneal fibrosis and ankylosing spondylitis. A new case report

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases.     

Spondyloarthropathy and retroperitoneal fibrosis: a case report

Retroperitoneal fibrosis is an uncommon inflammatory condition in which the retroperitoneal tissue is transformed into a tight sheet of fibrous tissue. It can occur in association with various diseases, including rheumatic conditions. We report a new case in a 66-year-old man with spondyloarthropathy. Nine similar cases have been reported. Axial manifestations predominated, and half the patients carried the HLA-B27 antigen. The relation between retroperitoneal fibrosis and spondyloarthropathy remains controversial.     

Idiopathic retroperitoneal fibrosis and common bile duct stenosis. A case report

The ordinary manifestations of idiopathic retroperitoneal fibrosis (IRF) with involvement of the ureters and urinary obstruction are well known. Quite a number of publications have made it clear that this disease process can often also affect organ systems other than the urinary tract--from the trachea and mediastinum to the rectum. The present case report calls attention to the fact that IRF may also be a surgically treatable cause of obstructive icterus.     

Idiopathic retroperitoneal fibrosis: the case for nonsurgical treatment

OBJECTIVE: To review a 10-year experience of medical treatment for idiopathic retroperitoneal fibrosis (RPF), a rare condition of unknown causes, which may obstruct the ureter and for which treatment includes ureterolysis and relief of obstruction, and medical treatment with steroids with or without immunosuppressive medication. PATIENTS AND METHODS: Of 31 patients with RPF treated between 1996 and 2004, 28 had idiopathic disease. Ureteric stents were used to relieve the obstruction in all patients, 12 had previous nephrostomies, 16 were given steroids and only one was treated with ureterolysis. RESULTS: In all, 28 patients were followed for a mean of 51.17 months; the symptoms were relieved in all cases. Renal function, where impaired, improved dramatically in all patients. Fifteen patients (54%) are free of stents and medication after a mean (range) follow-up of 60.9 (24-110) months. CONCLUSION: Idiopathic RPF responds well to conservative management consisting of relief of urinary tract obstruction and steroids.     

腹膜后纤维化行腹腔镜输尿管松解腹腔内间置术1例报道

目的 探讨腹膜后纤维化所致肾积水腹腔镜治疗的可行性.方法 报告1例因腹膜后纤维化所致左肾积水行腹腔镜治疗的诊治过程和结果,结合文献复习,探讨采用腹腔镜方法治疗腹膜后纤维化所致肾积水的可行性.55岁女性,间断左下肢浮肿,伴恶心、呕吐5个月,化验检查提示血肌酐和尿素氮进行性上升,血肌酐最高达503μmol/L.CT提示右肾萎缩,左输尿管下段与髂血管相交处狭窄,腹主动脉前与髂内外血管周围软组织影包绕,其上输尿管扩张.术前予以左输尿管双J管置入,手术取右侧半卧位,分别采用脐下1 cm、4 cm和左侧腹外斜肌外侧相同水平2点穿刺置入穿刺套管,腹腔镜自脐下1 cm处套管置人,术中见后腹膜呈板状,灰白色,打开后腹膜,见左输尿管巾段与髂血管周围粘连严重,超声刀仔细分离长度约9 cm,分离松解输尿管与周围组织问粘连,上下各达无粘连处,将此段输尿管放入腹腔内,关闭后腹膜.结果 术后恢复好,5 d切凵拆线,术后1月拔除双J管,拔管后3 h出现腰痛、发热和少尿,予以解痉、镇痛和抗菌治疗2 d后逐渐好转,尿量正常,多次复查肾功能正常,B超提示左肾积水程度逐渐减轻.3个月后复查B超提示左肾积水程度较术前显著减轻,肾功能维持正常.结论 腹膜后纤维化行腹腔镜输尿管松解、腹腔内间置术具有微创、病人痛苦小、术后恢复快的优点.但因有关此种治疗的报告例数少、随访时间较短,今后还需要更多病例和长时间的随访来进行验证其治疗效果.     

Idiopathic pelvic fibrosis: A variant of retroperitoneal fibrosis?

The management of 2 cases of a rare condition previously labelled idiopathic pelvic fibrosis is described. Two cases reported previously are reviewed. The unusual histologic and radiologic features are emphasized, and the differences between this condition and idiopathic retroperitoneal fibrosis are summarized. Etiologic features, including the possible role of previous pelvic infection, drug-induced hypersensitivity and autoimmunity, are discussed. We believe that disease originating in the vas may be the source of this unusual fibrotic process.     

Idiopathic retroperitoneal fibrosis

We treated a patient with idiopathic retroperitoneal fibrosis accompanied by right ureteral constriction. Pyelography, ureterography, and abdominal CT scan were pertinent diagnostics. Close collaboration between the surgeon and the urologist is required when attempting to treat such patients.