青春期泄殖腔畸形治疗体会  

Experience in the treatment of adolescent cloacal malformation

作　　者：季春宜[1] 尹强[1] 袁妙贤[1] 陈立健[1] 刘朝阳[1] Ji Chunyi;Yin Qiang;Yuan Miaoxian;Chen Lijian;Liu Chaoyang(Department of Pediatric General Surgery,Hunan Children's Hospital,Changsha 410007,China)

机构地区：[1]湖南省儿童医院普外一科,长沙410007

出　　处：《中华小儿外科杂志》2020年第11期1021-1026,共6页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨青春期泄殖腔畸形患儿手术重建方式。方法选择2018年2月至2019年11月湖南省儿童医院收治的3例青春期泄殖腔畸形女患儿的临床资料为研究对象,采用回顾性研究方法分析3例患儿手术前的处理、重建方式及术后恢复情况。结果3例泄殖腔畸形患儿术前均行膀胱镜检查,精确共同通道度长度分别为3、5及4 cm。患儿A行泄殖腔整体下移阴道及尿道成形、膀胱造瘘术;患儿B先行膀胱镜阴道穿刺置管引流,半月后行尿道成形、小阴唇转移皮瓣代阴道成形术;患儿C行膀胱肌瓣转移代尿道成形、阴道成形术。3例患儿分别随访1年6个月、10个月及6个月,均无反复泌尿生殖系统感染,无阴道积液及狭窄。术后患儿A、B能自主控尿;李正肛门功能评分分别为3分及4分。患儿C自主控尿能力欠佳,仍伴大部分尿失禁;李正肛门功能评分为1分。结论对于肛门成形术后青春期泄殖腔畸形患儿,术前均需进行膀胱镜检查并结合影像学分析,精确测量共同通道长度及明确其他系统畸形,多学科合作并个体化选择合适的重建材料及手术方式,缩短手术时间并减少手术创伤。手术的最终目的是保持排尿排便可控、阴道通畅甚至性功能及生育功能恢复,提高患儿远期生活质量。Objective To explore the surgical reconstruction of adolescent cloacal malformation.Methods From February 2018 to November 2019,clinical data were analyzed for three female adolescents of cloacal malformation(girls A,B&C).Preoperative evaluations,surgical reconstructions and postoperative recovery were analyzed.Results During cystoscopy,the length of common channel was 3,5 and 4 cm respectively.In girl A,vaginoplasty,urethroplasty and cystostomy were performed with cloaca moving down.While in girl B,transvaginal puncture and drainage were followed by urethroplasty and vaginoplasty with transfer flap of labia minora at half a month.Girl C underwent urethroplasty with bladder muscle flap plus vaginoplasty.Girls A and B regained self-control and Li Zheng's anal function scores were 3 and 4 respectively.Self-control was poor with partial incontinence in Girl C with a Li Zheng's anal function score of 1.Conclusions Cystoscopy and imaging analysis may be employed for accurately measuring the length of common channel and identifying other system deformities.Operative essentials are individualized selecting of appropriate reconstruction materials and operative approaches,shortening operative duration and minimizing surgical trauma.However,the ultimate goal of operation is to achieve continence,sexual performance,even reproductive function and improve long-term quality-of-life.

关 键 词：泄殖腔畸形 青春期 功能重建 

分 类 号：R726.5[医药卫生—儿科]