全结肠型无神经节细胞症31例回顾性分析  

Clinical analysis of 31 cases of Total colonic aganglionosis

作　　者：陈浩 何文琦 牛臻 梁木 宋晓峰[1] Chen Hao;He Wenqi;Niu Zhen;Liang Mu;Song Xiaofeng(Department of General SurgicalWard 2,Children's Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,China International Science and Technology Cooperation base of Child development and Critical Disorders,Chongqing Key Laboratory of Pe)

机构地区：[1]重庆医科大学附属儿童医院胃肠新生儿外科,儿童发育疾病研究教育部重点实验室,儿科发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,重庆400014

出　　处：《当代医学》2018年第12期15-17,共3页Contemporary Medicine

摘　　要：目的探讨全结肠型无神经节细胞症(total colonic aganglionosis,TCA)临床特点和治疗方案选择。方法回顾性分析本院胃肠新生儿外科2010年1月~2017年4月收治的31例TCA患儿临床资料,本组患儿均行病理检查。结果本组31例患儿:13例行Ⅰ、Ⅱ期分期手术,术后随访8例患儿排便功能较好,5例失访;2例行Ⅰ期根治术,随访1例排便功能可,1例失访;2例患儿分别于外院或本院行Ⅰ期或Ⅱ期手术,随访排便功能佳;5例行回肠造瘘术,其中1例因肾功能严重损害,家属放弃治疗。9例患儿家属放弃治疗。结论 TCA起病早,病情重。结合临床表现、钡剂灌肠造影、直肠黏膜活检或术中冰冻、术后病检可以确诊。Ⅰ期根治术适合诊断明确或一般状况良好的患儿,分期手术适合大多数患儿。本组病例中放弃治疗9例,1例患儿因术后合并肾功能严重损害死亡,其余患儿均获得良好的效果。Objective To investigate the clinical features and treatment options of total colonic aganglionosis(TCA).Methods Case data of 31 children with TCA in the General Surgical Ward 2 Department of our hospital between January 2010 and April 2017 were analyzed.Every patients have pathological examination.Results In the 31 children,13 children received stageⅠandⅡstaging surgery in our hospital,and then were discharged successfully.Among them,8 children showed good defecation and 5 children were lost in the 3-month-5-year follow-up.Two children underwent radical surgery(stageⅠ)in our hospital and then were discharged successfully;in the 7-month-2-year follow-up,1 child showed good defecation(BFS score,18)and another was lost.One child was treated with ileostomy in another hospital and radical colectomy for Hirschsprung's disease in stageⅡin our hospital,presenting good defecation after surgery.One child was subjected to ileostomy in our hospital and radical colectomy for Hirschsprung's disease in stageⅡin another hospital.Five children only received ileostomy in our hospital;among them 1 child's family members gave up treatment due to severe renal dysfunction after surgery.Nine children's family members gave up treatment after postoperative confirmation of TCA.Conclusion The onset of TCA is early and serious.Combined with clinical manifestations,barium enema,rectal mucosa biopsy or intraoperative frozen biopsy,postoperative pathological examination,TCA can be diagnosed.StageⅠradical surgery is suitable for children who have definite preoperative diagnosis or good general conditions,Staging surgery is suitable for most TCA children.In our cases,9 children gave up treatment,1 child died due to severe renal dysfunction after surgery,and the rest children showed good efficacy.

关 键 词：全结肠型无神经节细胞症 肠无神经节细胞症 外科手术 预后 

分 类 号：R726.5[医药卫生—儿科]