中文摘要：

目的 探讨肾盂输尿管连接处梗阻（ureteropelvic junction obstruction,UPJO）合并非反流性巨输尿管（nonreflux megaureter,NRM）患儿的诊治方法.方法 回顾性分析2005年1月至2015年12月收治的13例UPJO合并NRM患儿的临床资料.男8例,女5例.年龄1.8～14岁,平均3.7岁.排除医源性狭窄、膀胱输尿管反流、输尿管远端狭窄合并反流患儿.术前均行超声、静脉肾盂造影（IVU）、排尿性膀胱尿道造影、CT重建等检查.病变位于左侧8例,右侧5例.术前确诊UPJO合并输尿管膀胱连接处梗阻（ureterovesical junction obstruction,UVJO）6例.13例患儿中,10例行肾盂输尿管成形术（anderson-hynes procedure,AH）,3例确诊UVJO者行输尿管膀胱再植术.结果 13例患儿中术后经肾造瘘管输尿管造影确诊UVJ0 3例,非反流非梗阻性巨输尿管（nonreflux andnonobstruction megaureter,NNM）3例,1例术后3、6个月复查超声和IVU检查示输尿管远端未见狭窄,肾输尿管积水明显减轻,确诊为NNM.10例行AH者中3例术后3个月复查IVU示肾积水加重,行输尿管膀胱再植术治疗后肾输尿管积水逐渐减轻,余7例AH术后肾输尿管积水逐渐减轻未再次手术.3例行输尿管膀胱再植术者中2例术后3个月复查IVU示肾积水加重,行AH治疗后积水情况减轻,余1例术后肾积水逐渐减轻未再行手术.13例术后随访6 ～53个月,平均23.3个月,肾输尿管积水情况均逐渐减轻.结论 UPJO合并NRM的患儿诊断UVJO困难,对于此类患儿可先行AH,术后通过肾造瘘管造影或长期随访诊断是否存在UVJO,在明确远端梗阻致肾输尿管积水加重后再行输尿管膀胱再植术.

英文摘要：

Objective To investigate the experience of diagnosis and management of coexisting ureteropelvic junction obstruction （UPJO） and nonreflux megaureter （NRM）.Methods The retrospective study of UPJO with NRM was based on 10 years information retrieved from January 2005 to December 2015.The data of 13 patients （8 males and 5 females） were available and recorded.Mean age at surgery was 3.7 years old （range 1.8 to 14 years）.The diagnosis and mangement were summarized.Coexisting ureterovesical junction obstruction （UVJO） and vesicoureteral reflux,iatrogenic stricture and vesicoureteral reflux were excluded.Intravenous pyelography,voiding cystourethrography,ultrasound and CT reconstruction were performed before operation.Only six patients had an accurate diagnosis as UPJO with UVJO before surgery.Pyeloplasty was the initial surgical management choice for 10 patients,and ureteroneocystostomy in 3 patients.Results UVJO were diagnosed with pyelography techniques in 3 patients after pyeloplasty,while 4 were diagnosed as nonreflux and nonobstruction megaureter.Of the 10 patients who underwent initial pyeloplasty,additional ureteroneocystostomy was required in 3 and the prognosis was good.Additional pyeloplasty was required in 2 of the 3 patients who initially underwent ureteroneoeystostomy.Mean follow-up time from last operation was 23.3 months （6-53 months）,the overall prognosis was good.Conclusions It is often difficult to correctly diagnose coexisting UPJO and NRM.In patients with UPJO,it is highly recommended nephrostomy radiography after pyeloplasty to evaluate the distal ureterovesical junction.Initial pyeloplasty is always recommended as first-line therapy.Additional ureteroneocystostomy was required when hydroureteropelvic was aggravated.