[1]徐伟珏,吕志宝,吕逸清,等.一穴肛精准分型与手术方案决策的临床研究[J].临床小儿外科杂志,2020,19(10):891-896.[doi:10.3969/j.issn.1671-6353.2020.10.006]
　Xu Weijue,Lü Zhibao,Lü Yiqing,et al.Clinical study for precise claaification and surgical treatment of cloaca[J].Journal of Clinical Pediatric Surgery,2020,19(10):891-896.[doi:10.3969/j.issn.1671-6353.2020.10.006]
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一穴肛精准分型与手术方案决策的临床研究

《临床小儿外科杂志》[ISSN:1671-6353/CN:43-1380/R] 卷: 第19卷 期数: 2020年10期 页码: 891-896 栏目: 专题·肛门直肠畸形 出版日期: 2020-10-28
Title:
Clinical study for precise claaification and surgical treatment of cloaca
作者:
徐伟珏1;  吕志宝1;  吕逸清2;  孙俊1;  吴一波1;  陈发玲1;  陈方2;  唐雯娟3
1. 上海市儿童医院/上海交通大学附属儿童医院普外科(上海市, 200062);
2. 上海市儿童医院/上海交通大学附属儿童医院泌尿外科(上海市, 200062);
3. 上海市儿童医院/上海交通大学附属儿童医院影像科(上海市, 200062)
Author(s):
Xu Weijue1;  Lü Zhibao1;  Lü Yiqing2;  Sun Jun1;  Wu Yibo1;  Chen Faling1;  Chen Fang2;  Tang Wenjuan3
1. Department of General Surgery, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai 200062, China;
2. Department of Urology, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai 200062, China;
3. Department of Radiology, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai 200062, China
关键词:
消化系统畸形; 泄殖腔/畸形; 外科手术/方法
Keywords:
Digestive System Abnormalisties; Cloaca/AB; Surgical Procedures;  Operative/MT
分类号:
R726.1;R726.2;R657.1
DOI:
10.3969/j.issn.1671-6353.2020.10.006
摘要:
目的 探讨一穴肛的精准分型与手术方案的关系，以期最小损伤完成肛门及尿道阴道的一次成形术。方法 收集2010年10月至2020年2月上海市儿童医院收治的22例一穴肛患儿的临床资料，分析通过X线造影、MRI、膀胱镜检查了解患儿共同管（Common channel，CC）、尿道、阴道的长度，阴道子宫、直肠盲端汇入位置的变异及选择的手术方法及路径。结果 22例一穴肛患儿中，CC>3 cm 1例，≤3 cm 21例；尿道≥2 cm 12例，<2 cm 10例；阴道≥3 cm 11例，<3 cm 11例；双角子宫双阴道7例，其中严重阴道积液需处理2例；直肠盲端与尿道、阴道汇合于同一点12例，汇入于阴道、尿道汇合点远端3例，汇入于双阴道间隔处1例，汇入于阴道或子宫颈部5例，汇入于膀胱1例。后矢状入路肛门成形术（posterior sagittal anorectoplasty，PSARP）+经腹阴道旋转术完成阴道、尿道成形术1例；PSARP+尿道生殖窦整体游离（Total Urogenital Mobilization，TUM）拖出术或尿道生殖窦部分游离拖出术（Partial urogenital mobilization，PUM）14例；腹腔镜辅助下肛门直肠成形术（laparoscopically assisted anorectoplasty，LAARP）+TUM或PUM 5例；仅行肛门成形术2例。术后随访结果，所有患儿无严重并发症，并按Krickenbeck评价标准评估患儿的排便功能均超过6分，提示排便功能恢复尚可；其中评分>9分，排便功能恢复较好者10例（50%）。结论 一穴肛的精准分型是医生选择手术方案的依据，MRI、造影及术中膀胱镜联合检查有助于精准了解CC、尿道、阴道长度及直肠盲端汇入点，多学科联合制定手术方案并完成手术及随访。
Abstract:
Objective To explore the relationship between the precise classification of cloaca malformation and the surgical repair of complex cloacas with minimal damage.Methods Clinical data were retrospective reviewed for 22 girls with cloaca malformation undergoing operations within the last 9 years.The length of common channel (CC),urethra,vagina and the location of rectal termination were detected through angiography,magnetic resonance imaging (MRI),cystoscopy and corresponding surgical approaches were retrospectively reviewed. Results CC was >3 cm(n=1) and ≤3 cm(n=21); length of urethra ≥2 cm(n=12) and <2 cm(n=10); length of vagina ≥3 cm(n=11) and <3 cm(n=11).Seven cases had bicornuate uterus and duplicate Mullerian systems.Two cases of severe hydrocolpos were treated.Rectal termination was located at the junctions of urethra & vagina (n=13),distal vagina & urethra (n=2),duplicate Mullerian systems (n=1),vagina or cervix (n=5) and bladder (n=1).One patient underwent transabdominal vaginoplasty and urethroplasty through vaginal switch; posterior sagittal anorectoplasty (PASAP) plus total urogenital mobilization/partial urogenital mobilization (TUM/PUM,n=14); laparoscopically assisted anorectoplasty (LAARP) plus TUM/PUM (n=5) and anoplasty only (n=2).There were no serious postoperative complications.Postoperative follow-ups were evaluated by the Krickenbeck standard.Ten children (50%) earned >9 points with excellent defecation function.Above 6 points hinted that decent defecation function.Conclusion Accurate classification of cloaca is essential for selecting optimal surgical approaches.And angiography,MRI and cystoscopy can help to detect the length of CC,urethra and vagina and the /locations of rectal termination,vaginas and bladder.Multi-disciplinary consultations are vital for preoperative planning,surgery and follow-ups.

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备注/Memo

收稿日期:2020-03-09。
通讯作者:吕志宝,Email:lvzb@shchildren.com.cn

更新日期/Last Update: 2020-10-28