标题：全结肠无神经节细胞症21例临床分析

      作者：曾 甜，李新宁，石群峰，罗树友，苏乃伟，莫 丹
    (广西儿童医院小儿外科，广西 南宁 530003)

      卷次： 2013年24卷9期

      【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
4 d~5个月。21例 48 h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12 d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50 cm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。

      【关键词】 全结肠型巨结肠；外科手术；预后

      【中图分类号】 R574.62 【文献标识码】 A 【文章编号】 1003—6350（2013）09—1299—02


Clinical analysis of 21 cases of total colonic aganglionosis.
ZENG Tian, LI Xin-ning, SHI Qun-feng, LUO Shu-you,
SU Nai-wei, MO Dan. Department of Pediatric Surgery, Guangxi Children's Hospital, Nanning 530003, Guangxi, CHINA

【Abstract】 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 treatment later, because 50 cm 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 the operation times and reduce the economic burden of the family.