先天性巨结肠合并先天性肥厚性幽门狭窄1例并文献分析  

Hirschsprung Disease Combined with Congenital Hypertrophic Pyloric Stenosis:One Case Report and Literature Review

作　　者：王国辉 张书景 牛波波 牛会忠 WANG Guo-hui;ZHANG Shu-jing;NIU Bo-bo;NIU Hui-zhong(Department of General Surgery, Affiliated Children's Hospital of Hebei Medical University, Shijiazhuang 050031, Hebei, China;Maternal and Child Health Care Family Planning Service Center of Yuhua, Shijiazhuang 050030, Hebei, China)

机构地区：[1]河北医科大学附属河北省儿童医院普外一科,河北石家庄050031 [2]石家庄市裕华区妇幼保健计划生育服务中心,河北石家庄050030

出　　处：《中国现代手术学杂志》2022年第1期20-24,共5页Chinese Journal of Modern Operative Surgery

摘　　要：目的总结先天性巨结肠合并先天性肥厚性幽门狭窄的诊治经验。方法回顾性分析2019年12月收治的1例先天性巨结肠合并先天性肥厚性幽门狭窄新生儿的临床资料,并结合相关文献分析。患儿男性,出生22天,因腹胀、呕吐入院,钡灌肠示先天性巨结肠,超声检查示先天性肥厚性幽门狭窄。充分准备后Ⅰ期行腹腔镜下幽门环肌切开术,待患儿营养状态改善后Ⅱ期行腹腔镜下巨结肠根治术。结果术中冰冻证实为巨结肠,共切除肠管长约55 cm。术后病理示远端肠壁充血,部分浆肌层消失,黏膜下及肌间见粗大神经纤维,未见神经节细胞;近端肠壁肌层肥厚,肌间见神经节细胞,部分发育不良。术后患儿恢复良好,随访1年无不良反应,饮食正常,排便1~2次/d,性状未见异常,无污裤、便秘发生。结论营养干预治疗可促进先天性巨结肠患儿肠功能的恢复,降低并发症发生率;对于先天性巨结肠合并先天性肥厚性幽门狭窄患儿,Ⅰ期先行腹腔镜下幽门环肌切开术可解决患儿进食问题,改善营养状况,从而保证Ⅱ期行腹腔镜巨结肠根治术的安全。Objective To summarize the treatment of Hirschsprung disease(HD)combined with congenital hypertrophic pyloric stenosis(CHPS).Methods The clinical data of one newborn with HD and CHPS were analyzed retrospectively.The case was admitted due to abdominal distension and vomiting.HD was diagnosed by barium enema and CHPS by ultrasonography.Staging surgery was performed,including laparoscopic pyloromyotomy in StageⅠafter adequate preparation,and laparoscopic radical megacolon resection in StageⅡafter improvement of nutritional status.The relevant literatures were reviewed.Results Megacolon was confirmed by intraoperative pathology.The total length of resection segment colon was about 55 cm.Postoperative pathology showed hyperemia of distal intestinal wall,disappearance of partial seromuscular layer,thick nerve fibers in submucosa and between muscles and no ganglion cells,and intestinal muscular hypertrophy of proximal intestinal wall,intermuscular ganglion cells and some dysplasia.The children recovered well after operation,and no adverse reactions were observed during the follow-up of one year.The diet was normal,and the defecation was 1 to 2 times once a day without abnormal stool traits,dirty pants and constipation.ConclusionsNutritional support can promote the recovery of intestinal function and reduce complications in HD children.The treatment of staging surgical intervention is a sensible choice for HD combined with CHPS.Laparoscopic pyloromyotomy of stageⅠcan resolve the problem of feeding and improve nutritional status,and ensure the safety of stageⅡlaparoscopic radical megacolon resection.

关 键 词：先天性巨结肠 先天性肥厚性幽门狭窄 腹腔镜手术 幽门环肌切开术 巨结肠根治术 营养支持 

分 类 号：R726.1[医药卫生—儿科]