泄殖腔畸形的个体化评估与手术决策

Individualized evaluations and surgical options of cloacal malformations

目的 探讨泄殖腔畸形术前个体化评估流程及其对手术方式的指导意义。方法 回顾性分析2011年3月至2017年11月接诊的31例泄殖腔畸形患儿的临床数据,阐释在姑息及根治术前进行的首次及二次评估中,通过B型超声、共同通道造影、磁共振及内窥镜检查等手段对泌尿、生殖系统伴发畸形、直肠盲端位置及共同通道长度等重要信息分析的临床意义,及其对泄殖腔畸形分型以及手术方式选择的指导意义。本组全部为女童,首次就诊年龄(280.25±731.01)d,年龄范围0~9岁,中位数为25 d;随访终末患儿年龄(51.91±44.13)个月,范围在11~192个月,中位数41个月。结果 本组确定共同通道小于1 cm的Ⅰ型畸形患儿10例,大于1 cm小于3 cm的低位畸形患儿14例,大于3 cm的高位畸形患儿7例。Ⅰ型畸形患儿中,3例行后矢状入路肛门成形术(posterior sagittal anorectoplasty,PSARP),7例行后矢状入路肛门成形+阴道后壁下移。低位畸形14例患儿全部采取后矢状入路肛门成形+尿生殖窦整体下移术。高位畸形1例仅行后矢状入路肛门成形术,未处理尿生殖窦;其余6例采取腹骶联合直肠阴道替代+肛门成形术。随访9个月至7年,平均38.46个月;31例患儿结肠造口均在术后3~6个月内关闭。1例患儿出现肛门狭窄,再次行后矢状入路肛门成形术治疗;2例患儿因肛门黏膜脱垂或肛周瘢痕形成再次手术;1例患儿因尿失禁行膀胱颈结扎及尿流改道。结论 在个体化的首次、二次评估策略指导下,以共同通道造影、CT、MR及内窥镜检查等为主的评估,可明确疾病分型及并发畸形,有效指导手术方式的选择。

Objective To explore the individualized preoperative evaluations of cloacal malformations and to examine its guiding significance for surgical approaches.Methods A retrospective analysis was conducted for clinical data from 31 girls with cloaca malformations from March 2011 to November 2017.During the first and second assessments prior to palliative and radical operations,clinical significance was analyzed for urinary and reproductive system malformations,rectal position and common channel length by ultrasonography type B,common channel angiography,magnetic resonance(MR)and endoscopy,abnormality type and selecting surgical approaches.Initial visit was(280.25±731.01)days with an age range of(0-9)years(median:25 days);the follow-up period(51.91±44.13)months with a median range of 41(11-192)months.Results Ten patients(type I)with a common canal<1 cm were identified.Those>1 cm and<3 cm were of lower malformation(n=14)and>3 cm high malformation(n=7).The procedures of type I malformation included posterior sagittal anorectoplasty(PSARP)(n=3)and PSARP plus post-vaginal wall mobilization(n=7);all cases with low malformation had PSARP plus urogenital sinus mobilization(n=14).One patient underwent PSARP with untreated urogenital sinus while another 6 girls had rectal vaginal replacement plus angioplasty.During a follow-up period of 38.46(9-84)months,there were perineal wound rupture(n=1)and vaginal/anal stenosis(n=4).The overall prognosis was good after corresponding treatments.Conclusions With the guidance of the first and second evaluation strategies,individualized assessment based upon common canal angiography,CT,MR and endoscopy can identify the associated malformations,complete the individualized assessments of common channels and effectively guide the choice of surgical approaches.