Objective To investigate clinical manifestations, accessory examinations and treatment of the total 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 recovered 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 after 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 received intestinal fistula and gave up treatment later, because 50 cm of terminal ileum to the whole colon was small in surgical 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. Sequential surgery is more safe than the one stage operation. But the trend is lesions of the bowel resection and lleorectal anastomosis in one stage, because it can decrease the operation times and reduce the economic burden of the family.%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.
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