我国内地地区肛门直肠畸形诊疗现状调查

Status survey on diagnosis and treatment of anorectal malformations in China's Mainland

目的总结我国内地地区先天性肛门直肠畸形(anorectal malformation,ARM)诊断与治疗的现状。方法使用问卷网设计ARM诊治调查问卷,包括ARM分型标准的选择、术前检查、手术方式的选择、腹腔镜的应用、术后排便功能的评估等,通过微信向国内小儿外科专家发送问卷,问卷完成后数据自动上传至问卷网统计分析。结果共收集问卷374份,有221份问卷纳入分析。每年收治ARM例数:5.9%(13/221)医院收治100例以上,15.8%(35/221)收治51~100例,40.7%(90/221)收治11~50例,37.6%(83/221)收治10例以下。术前检查:95.5%(211/221)医院做腹部倒立侧位X线片,71.5%(158/221)做泌尿系超声,68.8%(152/221)做心脏彩色多普勒超声,43.0%(95/221)做骶尾部MRI。术前分型:46.6%(103/221)医院选择Wingspread分型,12.7%(28/221)选择Krickenbeck分型,40.7%(90/221)选择Wingspread分型与Krikenbeck分型相结合。对于ARM合并直肠前列腺部瘘的患儿,选择一期肛门成形不做结肠造瘘的占14.0%(31/221),选择分期手术的占84.2%(186/221)。对于高位ARM的患儿,19.5%(43/221)医院每年开展超过10例腹腔镜辅助下肛门成形术,43.0%(95/221)每年不超过10例,37.6%(83/221)未开展腹腔镜。术后排便功能评估方法:28.1%(62/221)医院使用Kelly评分法,23.5%(52/221)使用李正6分法,12.7%(28/221)使用Wingspread评分法,19.5%(43/221)使用Peña评分法,28.5%(63/221)不进行临床评分。结论目前ARM术前检查及伴发畸形筛查不完善,临床分型方法尚无统一标准,中高位ARM多采用分期手术,腹腔镜辅助下肛门成形术的使用主要集中在大型儿科诊疗中心,ARM术后排便功能的评估需要统一标准。

Objective To summarize the current situation of diagnosing and treating anorectal malformations(ARM)in China's Mainland.Methods The questionnaire network was utilized for designing the ARM diagnosis and treatment questionnaire,including selection of ARM classification criteria,preoperative examination,surgical staging,application of laparoscopy and evaluation of postoperative defecation function,etc.The questionnaire was distributed to domestic experts of pediatric surgery via Wechat.After the questionnaire was completed,the relevant data were automatically uploaded to the questionnaire network for statistical processing.Results Among a total of 374 questionnaires,221 were valid.The average number of ARM cases:only 5.9%(13/221)treated>100 cases of ARM per year,15.8%(35/221)treated 51-100 cases per year,40.7%(90/221)treated 11-50 cases per year and 37.6%(83/221)treated<10 cases per year.Preoperative examination:95.5%(211/221)underwent abdominal inverted lateral radiography,71.5%(158/221)urinary ultrasound,68.8%(152/221)cardiac Doppler ultrasound and 43.0%(95/221)sacrococcygeal magnetic resonance imaging(MRI).Classification method of ARM:46.6%(103/221)adopted traditional Wingspread classification method,12.7%(28/221)hospitals opted for Krickenbeck classification method and 40.7%(90/221)combined them.For ARM plus rectoprostal fistula:14.0%(31/221)chose one-stage anoplasty without colostomy and 84.2%(186/221)staging operation.For high ARM:19.5%(43/221)had>10 cases of laparoscopic-assisted anorectoplasty per year,43.0%(95/221)<10 cases per year and 37.6%(83/221)none.Postoperative defecation function evaluation:Kelly score was used at 28.1%(62/221)hospitals,Li Zheng 6-score at 23.5%(52/221),Wingspread score at 12.7%(28/221),Peña score at 19.5%(43/221)and no clinical score at 28.5%(63/221).Conclusions Preoperative examination and screening of concomitant malformation of ARM are not consummate.There is no unified standard for clinical classification.Most medium/high position of ARM are operated by stages.Laparoscopic technology is generally applied at large pediatric treatment centers.Evaluation standard for functions of postoperative defecation should be unified.