田广超 苑思彤 王登辉 崔梦杰 楚向阳 范应中

[摘要] 对我院小儿外科2021年2月收治的1例小儿肾外肾盏并先天性输尿管中远段重度狭窄患儿的临床资料进行回顾性分析，并对国内外相关文献进行复习。本例患儿为7月龄男性婴儿，母乳喂养，孕龄26周孕检时发现右肾发育异常，无典型阳性临床表现，经术前CT尿路造影、逆行性膀胱尿道造影、肾核素扫描等检查及术中探查明确诊断，并经手术治疗后病愈出院。阅读国内外文献，偶有单纯肾外肾盏或先天性输尿管长段重度狭窄或闭锁的案例报道，均行手术治愈，但無二者合并的个例报道，二者均为罕见的泌尿系统先天发育畸形，二者合并故更加罕见，采取适宜的个体化手术方案并及时尽早行手术治疗是取得良好预后的重要保证。

[关键词] 肾外肾盏;先天性;小儿;输尿管长段重度狭窄;输尿管长段闭锁

[中图分类号] R726.9          [文献标识码] C          [文章编号] 1673-9701（2021）36-0154-04

Analysis on a case of pediatric extrarenal calyce with congenital severe stenosis of the middle and distal ureter and literature review

TIAN Guangchao   YUAN Sitong   WANG Denghui   CUI Mengjie   CHU Xiangyang   FAN Yingzhong

Department of Pediatric Surgery，the First Affiliated Hospital of Zhengzhou University，Zhengzhou  450052，China

[Abstract] To retrospectively analyze the clinical data of a child with extrarenal calyce complicated by congenital severe stenosis of the middle and distal ureter admitted to our pediatric surgery department in February 2021，and to review the relevant literatures at home and abroad. This case was a 7-month-old male infant，breastfed，with abnormal development of the right kidney detected by pregnancy test at 26 weeks of gestation without typical positive clinical manifestations. The diagnosis was confirmed by preoperative CT urography（CTU），retrograde cystourethrography，renal nuclear scanning and intraoperative exploration，and the child was discharged after surgical treatment and cured. In both the domestic and international literatures，there are occasional reports on only extrarenal calyce or congenital severe stenosis or atresia of the distal ureter，all of which were cured by surgery. However，there is no case report on the combination of the two，both of which are rare congenital and developmental malformations of the urinary system，and the combination of the two is even rarer. Therefore，the adoption of an appropriate individualized surgical plan and timely and early surgical treatment is an important guarantee of a good prognosis.

[Key words] Extrarenal calyce;Congenital;Pediatric;Severe stenosis of the distal ureter;Atresia of the distal ureter

肾外肾盏及先天性输尿管长段重度狭窄或闭锁均为泌尿系统罕见的先天发育畸形，二者发病原因均未被明确证实，多数学者认为其与肾脏胚胎发育时期及肾脏迁移时期的异常有关[1-2]。既往报道多为散发病例且均为单纯肾外肾盏或先天性输尿管长段重度狭窄或闭锁，未见二者合并的个例报道。此二类患者多在婴幼儿时期体检发现，或年龄稍大儿童或成人因腹部肿物发现。早期无典型临床症状，若不及早发现并行手术治疗，多引起患侧肾脏发育不全，进而导致肾功能减退、肾萎缩、肾性高血压等一系列病程变化。……