全结肠无神经节细胞症21例临床分析  

Clinical analysis of 21 cases of total colonic aganglionosis.

作　　者：曾甜 李新宁 石群峰 罗树友 苏乃伟 莫丹 

机构地区：[1]广西儿童医院小儿外科,广西南宁530003

出　　处：《海南医学》2013年第9期1299-1300,共2页Hainan Medical Journal

基　　金：广西科技厅自然科学基金(编号:桂科自0991181)

摘　　要：目的研究全结肠无神经节细胞症(TCA)患儿的临床表现、辅助检查和治疗,提高患儿生存率。方法回顾性分析21例TCA患儿的临床资料、手术方式及预后。本组21例,其中男16例,女5例;年龄4d^5个月。21例48h内均未自主排出胎粪,主要症状为腹胀、呕吐。均行剖腹探查。结果 16例一期根治术的患儿术后12d扩肛,顺利出院,随访1个月~1年,1例偶有粪污,余排便可,发育正常;4例回肠造瘘术患儿,其中2例3个月至半年后回院关瘘,行巨结肠根治术,随访1个月~1年,术后患儿恢复良好,排便3~5次/d,生长发育正常。1例因经济原因至今未回院关瘘。1例行回肠造瘘,现已2个月,患儿发育正常,恢复良好,待关瘘;1例探查示回肠末端50cm至全结肠细小,行肠造瘘,后放弃治疗。结论 TCA发病早,病情较重,结合钡灌肠及术中多点肠管冰冻活检为早期确诊方法。分期手术较安全,但趋向于一期行病变肠管切除,并回肠直肠吻合术,不仅减少了对患儿手术打击次数,而且降低了患儿的家庭负担。Objective To investigate clinical manifestations, accessory examinations and treatment of the to- tal colonic aganglionosis （TCA）, and to improve children＇s survival rate. Methods A total of 21 patients with TCA were studied, including 16 males and 5 females, aged from 4 days to five months. The clinical data, surgical methods and prognosis were analyzed retrospectively. None of them were voluntary defecation within 48 hours. The main symptoms were abdominal distention, vomiting. All the patients received exploratory laparotomy. Results Sixteen patients received primary radical operation, and were cured with anal dilatation at 12 days after surgery. The follow-up period ranged from 1 month to 1 year. One patient suffered from incontinence of loose stool after 1 year. 15 patients re- covered excretive function and had a normal development. Four patients were dealt with ileum tubal fistulation, two of which received operation to close the colostomy and to perform radical operation on congenital megacolon defense af- ter three months to half a year. The results were satisfactory, with the frequency of defecation between 3 times and 5 times per day, normal growth and development during the follow-up （1 month to 1 year）. One patient has not returned and closed the colostomy so far due to economic reasons. One patient was dealt with tube fistulization via cristal ileum two months ago, who is waiting for closing the colostomy with anormal growth and development. One patient re- ceived intestinal fistula and gave up ~＇eatment later, because 50 crn of terminal ileum to the whole colon was small in surgi- cal exploration. Conclusion TCA occurs early and does heavy harm to the child. The method for the early diagnosis of TCA is barium enema and the intraoperative rapid frozen biopsy from multiple regions of intestinal in combination. Se- quential surgery is more safe than the one stage operation. But the trend is lesions of the bowel resection and lleorectal anas- tomosis in one stage, because it can decrease th

关 键 词：全结肠型巨结肠 外科手术 预后 

分 类 号：R574.62[医药卫生—消化系统]