小儿巨结肠同源病中的结肠解剖畸形与肠神经系统病变  

Anatomicalcolonic malformation with enteric nervous system abnormalities in allied disorders of Hirschsprung's disease

作　　者：孙晓毅[1] 余东海 李智 朱天琦 王文静 Sun Xiaoyi;Yu Donghai;Li Zhi;Zhu Tianqi;Wang Wenjing(Department of Pediatric Surgery,Affiliated Tongji Hospital,Tongji Medical College,Huazhong University of Science&Technology,Wuhan 430030,China)

机构地区：[1]华中科技大学附属同济医院小儿外科,武汉430030

出　　处：《中华小儿外科杂志》2020年第11期1001-1004,共4页Chinese Journal of Pediatric Surgery

基　　金：湖北省自然科学基金(2010CDB09602)。

摘　　要：目的探讨巨结肠同源病(Allied disorders of Hischsprung's disease,AHD)患儿中出现结肠冗长和屈曲等解剖畸形的诊断治疗和肠神经元病变的相关性问题。方法回顾性分析2014年12月至2018年12月间经手术治疗的25例AHD伴结肠解剖畸形患儿的临床资料。其中,男15例,女10例,年龄为(8.65±4.10)岁。25例患儿均有顽固性便秘,且症状随年龄增加以及社会活动增加逐渐加重,需长期药物助排便,病程超过3年,保守治疗无效。所有患儿术前均通过肠系造影检查和72 h结肠传输试验而确诊。手术方式为结肠次全切除术22例,左半结肠切除术3例。结果术前胆碱酯酶组织化学检查阳性3例,阴性22例;所有患儿均存在肛门直肠抑制反射。92%(23/25)的患儿结肠传输试验72 h的标记物排出率为0。术后病理学检查诊断为肠神经元发育不良症14例,节细胞减少症11例。术后随访时间>1年,23例患儿便秘症状消失,行左半结肠切除术3例中2例复发后再手术治愈。结论结肠解剖畸形是严重便秘的一个重要原因,结肠传输时间延迟与便秘程度呈正相关。畸形肠道的肠神经元病变在结肠壁广泛分布,病变范围与结肠传输试验所提示的动力受损肠管范围高度相符,提示手术切除范围和病理学检查的重点部位。Objective To explore the long and flexed anatomical colonic deformities in children with allied disorders of Hirschsprung's disease and examine its diagnosis and treatment as well as the pathological changes of enteric nervous system.Methods From December 2014 to December 2018,15 boys and 10 girls with an average age of 8.65±4.10 years were operated.Obstinate constipation gradually worsened with advancing age and more social activities.The course of disease was over 3 years.Long-term laxative medications and conservative measures were both ineffective.Anorectal manometry,rectal mucosa biopsy,enterography and 72h colonic transit test were performed.The operative approach was subtotal colectomy(n=22)and left colectomy(n=3).Results The preoperative AChE staining was positive(n=3)and negative(n=22).Anorectal inhibitory reflex was present in all 25 cases.In colonic transit experiment,92%of cases had zero marker excretion at 72h.Pathological diagnosis was hypoganglionosis(HG,n=11)and intestinal neuronal dysplasia(IND,n=14).During the postoperative follow-up period of over 1 year,the symptoms of constipation disappeared(n=23)and became cured after reoperation for recurrence(n=2).Conclusions Anatomical colonic malformation is an important cause of constipation.Delayed transit time is positively correlated with the severity of constipation.Nervous system lesions are widely distributed in abnormal colonic walls.The extent of lesions is highly consistent with the extent of dynamically damaged colon.And the result of transmission test may dictate resection range and target regions of pathological examination of colon.

关 键 词：结肠 畸形 结肠传输 巨结肠同源病 

分 类 号：R726.5[医药卫生—儿科]