泄殖腔畸形合并其他畸形的临床诊治经验

Experience in the management of rare and complex cloacal malformations

目的探讨泄殖腔畸形患儿合并复杂畸形的手术经验。方法收集2006年6月至2022年12月上海交通大学医学院附属新华医院收治的17例泄殖腔畸形女患儿的临床资料,中位手术年龄为13.2个月,范围为7.2~34.6个月。其中短共同通道者(≤3 cm)13例,长共同通道者(>3 cm)4例。3例合并其他畸形:泄殖腔畸形合并球形结肠及双半子宫1例,泄殖腔畸形合并直肠膀胱瘘及膀胱外阴瘘1例,泄殖腔畸形误诊为直肠阴道瘘行肛门成形术后反复泌尿系统感染1例。通过电话和门诊随访患儿的排便控制功能与泌尿系统并发症,使用Rintala评分评估患儿控便能力。结果17例患儿中,13例短共同通道者患儿采取后矢状入路尿生殖窦整体下拖+肛门成形术,中位手术年龄为13.4个月,中位手术时长185 min。4例长共同通道患儿行腹腔镜/开腹辅助腹骶会阴联合尿生殖窦整体下拖+肛门成形术,中位手术年龄为12.5个月,中位手术时长482 min。术后随访短共同通道患儿的中位Rintala评分为16分,4例患儿出现尿路感染,2例患儿存在尿失禁情况,其余7例未出现泌尿系统并发症。长共同通道组患儿的中位Rintala评分为14分,2例患儿出现尿道感染,1例患儿存在尿失禁情况,其余1例未出现泌尿系统并发症。结论泄殖腔畸形合并其他畸形术前必须进行精准评估,多学科联合制定个体化治疗方案和术后长期管理。

Objective To explore the surgical management experience of children with rare and complex cloacal malformations.Methods Retrospective analysis was conducted for clinical data of children hospitalized with cloacal malformations from June 2006 to December 2022.The average operative age was 13.2(7.2-34.6)months.There were short common channel malformation(≤3 cm,n=13)and long common channel malformation(>3 cm,n=4).Three rare cases were associated with other malformations,including urogenital sinus malformation plus colonic atresia and double uterus(n=1),urogenital sinus malformation plus rectovesical fistula and bladder-perineal fistula(n=1)and urogenital sinus malformation misdiagnosed as rectovaginal fistula undergoing anal reconstruction and followed by repeated urinary tract infections(n=1).Bowel function and urinary system complications were evaluated through telephone follow-ups.The Rintala scoring system was employed for assessing bowel function.Results Among them,13 cases of short common channel malformation underwent posterior sagittal anorectoplasty plus urinogenital sinus mobilization and anal reconstruction.The median operative age was 13.4 months and the median operative duration 185 min.Four cases of long common channel malformation underwent laparoscopic/open-assisted abdominoperineal pull-through plus urinogenital sinus mobilization and anal reconstruction.The median operative age was 12.5 months and the median operative duration 482 min.During postoperative follow-up period of patients with short common channel malformation,the median Rintala score was 16.There were urinary tract infection(n=4),urinary incontinence(n=2)and no urinary system complications(n=7).In long common channel group,the median Rintala score was 14.There were urethral infection(n=2),urinary incontinence(n=2)and no urinary system complications(n=1).Conclusions Accurate preoperative assessment is necessary for cloacal malformation plus other malformations.A multidisciplinary approach should be adopted for formulating individualized treatment plans and ensuring long-term postoperative management.