摘要目的 探讨通过肠镜在巨结肠扩张段放置减压管并保留一段时间,使患儿可以安全接受一期微创根治手术,以避免造瘘手术的可行性及效果.方法 回顾性分析2016年1月至2017年3月经肠镜放置减压管治疗先天性巨结肠症9例的临床资料.其中,男8例,女1例;长段型先天性巨结肠8例,常见型累及降结肠中段1例.置管后如果腹胀和小肠结肠炎缓解,体重增加患儿将再次入院接受腹腔镜辅助巨结肠根治术.结果 全部患儿术前均有腹胀和发育延迟,其中5例伴有小肠结肠炎.放管时患儿年龄11～228 d,中位年龄41 d,中位体重3.8 kg(2.5～8.4 kg),中位肠镜手术时间42.5 min(35～60 min).术后全部患儿腹胀和肠炎缓解,体重增加,进食好,无需静脉营养,未出现严重并发症.有6例患儿已经接受了腹腔镜辅助巨结肠根治术,其中4例行腹腔镜辅助结肠次全切除、升结肠翻转肛管吻合术,1例行腹腔镜辅助全结肠切除、回肠肛管吻合术,1例行腹腔镜辅助巨结肠根治、降结肠肛管吻合术.根治手术中位年龄88.5 d(59～113 d),中位体重5.7 kg(4.6～7.0 kg),中位增加体重2.4 kg(0.6～3.8 kg),中位根治手术时间165 min(120～210 min),失血量5～15 ml,中位切除肠管长度45 cm(40～70 cm).术后无严重并发症,患儿恢复好.结论 结肠镜引导下结肠减压管放置后,腹腔镜辅助一期根治长段型巨结肠可行、有效,但还应进一步扩大使用范围,改进管道设计,增加使用病例数和随访时间,以期得到更客观、充分的评估.

abstractsObjective Long-segment Hirschsprung's disease (L-HD)is still intractable for pediatric surgeons.Enterostomy is often initially applied.If bowel decompression can be achieved endoscopically by placing a tube across transitional zone,some patients may avoid staged or high-risk operation.Methods After rectal mucosa biopsy,9 patients (eight L-HD and one with transitional zone proximal to middle of descending colon)underwent endoscopic decompression tube placement (EDTP ).Hirschsprung 's associated entroco litis (HACE ) and physical condition improved significantly and body weight gained.The patients were then readmitted and underwent laparoscopic assisted placement.Results The median age of patients at EDTP was 41 (11-228)days,the median body weight 3.8 (2.5-8.4)kg and the median endoscopic operative duration 42.5 (35-60)mins. Abdominal distention and HAEC were relieved,body weight was gained and oral feed was well-tolerated in all patients after intubation without a need of total parenteral nutrition.There was no major complication.Six patients underwent TRM-PIAS and laparoscopic assisted pull-through.The definitive pull-through procedure was performed at a median age of 88.5 (59-113)days with a median body weight of 5.7(4.6-7.0)kg.The median operative duration was 165 (120-210)mins.Blood loss was 5 to 15 ml.The median length of resected colon was 45 (40-70 )cm.All of them recovered uneventfully.Conclusions Endoscopic decompression tube placement and one-stage laparoscopy are both safe and effective for L-HD.Tubes should be modified for better anchoring and extensive usage.