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先天性输尿管中段狭窄

Congenital mid ureteral strictures.

作者信息

Hwang Andrew H, McAleer Irene M, Shapiro Ellen, Miller Oren F, Krous Henry F, Kaplan George W

机构信息

Division of Urology, Childrens Hospital Los Angeles, Los Angeles, California 90027, USA.

出版信息

J Urol. 2005 Nov;174(5):1999-2002. doi: 10.1097/01.ju.0000176462.56473.0c.

Abstract

PURPOSE

Congenital mid ureteral stricture is rare. We report 7 cases, and discuss the differences in preoperative evaluation and surgical management compared to other obstructive entities.

MATERIALS AND METHODS

Medical records and imaging studies of 7 children identified with mid ureteral strictures between 1998 and 2002 were reviewed retrospectively. Five newborns presented with prenatal hydronephrosis, and 2 children presented at age 15 years, one in the course of evaluation of blunt trauma, and one due to pain and abdominal mass. Imaging studies included renal ultrasound, voiding cystourethrography, radionuclide renography and computerized tomography. All patients underwent retrograde pyelography. Pathological examination of each specimen was undertaken at the respective institutions.

RESULTS

Prenatal hydronephrosis was the most common presentation. There were no urinary tract infections. All patients had significant obstruction on the affected side. No patient had vesicoureteral reflux. After imaging but before surgery the urinary obstruction was believed to be at the ureteropelvic junction in 4 patients and the ureterovesical junction in 2, and secondary to posterior urethral valves in 1. At cystoscopy all of the affected ureters had a normally located and normally configured orifice. Retrograde pyelography led to an accurate diagnosis of mid ureteral narrowing in all patients. Six patients underwent ureteroureterostomy, all of whom had satisfactory outcomes. In 1 of these patients contralateral nephrectomy was performed due to nonfunction of the multicystic dysplastic kidney. The remaining patient underwent nephrectomy for ipsilateral end stage kidney disease and hydronephrosis. In this patient the ureters were stenotic and suggested asymmetry in the thickness of the muscular coat, perhaps secondary to extrinsic compression.

CONCLUSIONS

Congenital mid ureteral stricture is rare. Renal ultrasound and radionuclide renography alone do not reliably demonstrate the site of obstruction. Retrograde pyelography at the time of surgical correction of presumed ureteral obstruction is an important adjunct for correctly identifying the site of narrowing in the affected ureteral segment, unless the ureter has been imaged with another modality.

摘要

目的

先天性输尿管中段狭窄较为罕见。我们报告7例病例,并讨论其与其他梗阻性疾病在术前评估和手术治疗方面的差异。

材料与方法

回顾性分析1998年至2002年间确诊为输尿管中段狭窄的7例儿童的病历和影像学检查资料。5例新生儿表现为产前肾积水,2例儿童分别于15岁时就诊,1例因钝性外伤评估过程中发现,1例因疼痛和腹部肿块就诊。影像学检查包括肾脏超声、排尿性膀胱尿道造影、放射性核素肾图和计算机断层扫描。所有患者均接受逆行肾盂造影。各标本在相应机构进行病理检查。

结果

产前肾积水是最常见的表现。无尿路感染。所有患者患侧均有明显梗阻。无患者存在膀胱输尿管反流。影像学检查后但手术前,4例患者的尿路梗阻被认为位于输尿管肾盂连接处,2例位于输尿管膀胱连接处,1例继发于后尿道瓣膜。膀胱镜检查时,所有受累输尿管口位置和形态正常。逆行肾盂造影使所有患者均准确诊断为输尿管中段狭窄。6例患者接受输尿管输尿管吻合术,所有患者预后良好。其中1例患者因多囊性发育不良肾无功能而进行了对侧肾切除术。其余1例患者因同侧终末期肾病和肾积水接受了肾切除术。该患者输尿管狭窄,提示肌层厚度不对称,可能继发于外部压迫。

结论

先天性输尿管中段狭窄较为罕见。单纯肾脏超声和放射性核素肾图不能可靠地显示梗阻部位。在对疑似输尿管梗阻进行手术矫正时,逆行肾盂造影是正确识别受累输尿管段狭窄部位的重要辅助手段,除非输尿管已通过其他方式成像。

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