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目的:结合文献分析回肠J型储袋肛管吻合术（ileal pouch anal anastomosis, IPAA）治疗全结肠型巨结肠（total colonic aganglionosis, TCA）的方案和预后。方法:分析首都医科大学附属北京儿童医院普外科2017月6月至2020年6月采用IPAA治疗的3例TCA患儿的临床资料。3例患儿均胎便排出延迟，继而便秘、呕吐和腹胀。例1生后9个月外院行阑尾切除术，术中未行冰冻活检，术后未明确诊断，1岁9个月行“肠壁多点冰冻活检+回肠造瘘术”时诊断TCA，造瘘口排成形软便后于3岁2个月行IPAA。例2、例3分别于生后3 d、26 d外院行“回肠造瘘术”时诊断TCA，造瘘口排成形软便后于2岁4个月、2岁2个月行“肠壁多点冰冻活检+IPAA+保护性回肠造瘘术”。检索Pubmed、Web of Science、Medline、Cochrane Library、中国知网和万方数据库截至2020年6月1日关于IPAA治疗TCA的文献，英文检索词为“total colonic aganglionosis”与“pouch”，中文检索词为“全结肠型巨结肠”与“储袋”或“贮袋”，对IPAA方案和预后进行系统评价。结果:本组例1IPAA术后1年2个月，排便频率为3次/d，排便控制可，污粪频率每周少于1次；例2关瘘术后1个月，排便频率为3~4次/d，排便控制可，污粪频率每周少于1次；例3关瘘术后6个月，排便频率为2次/d，排便控制可，污粪频率每周少于1次。共检索到文献27篇，符合纳入标准2篇，共14例患儿。11例IPAA术前行回肠造瘘的中位年龄为17 d，行IPAA时中位年龄为9个月，7例IPAA术后行保护性回肠造瘘，平均3.7个月关瘘。最常见的术后并发症为肠梗阻（50.0%，4/8）和小肠结肠炎（62.5%，5/8）。末次术后半年，平均排便频率≥7次/d；末次术后超过2年，平均排便频率≤4次/d，无排便失禁。结论:分期IPAA治疗TCA患儿能获得良好预后，近期预后与术前造瘘和根治手术年龄相关，需根据造瘘口排便性状选择合适根治手术时机。“,”Objective:To evaluate the operative timing and prognosis of ileal pouch anal anastomosis (IPAA) for total colonic aganglionosis (TCA) based upon institutional experiences with a systematic literature review.Methods:From June 2017 to June 2020, 3 TCA children undergoing IPAA were retrospectively reviewed. There were constipation, vomiting, abdominal distension and a delayed passage of meconium. Case 1 of an indefinite diagnosis underwent appendicitis resection at external hospital at Month 9 without a frozen biopsy. Then TCA was diagnosed at Month 21 after “intraoperative frozen section examination + ileostomy” and IPAA was performed at Month 38 with formed stool in fistula. During ileostomy, cases 2 and 3 received a definitive diagnosis of TCA at Day 3 and 26 post-birth at external hospitals and underwent “ intraoperative frozen section examination + IPAA+ protective ileostomy” at the ages of 2.3 and 2.2 years respectively. Ileostomy was closed in 2 cases at Month 12 and 6 respectively. Prior to June 2020, publications of IPAA for TCA were retrieved from the databases of PubMed, Web of Science, Medline, Cochrane library, CNKI and Wangfang. The procedures and prognosis were systematically analyzed.Results:All children had less than 4 bowel movements within 24h with no fecal incontinence. And occasional soiling and perianal redness occurred at Month 14 post-IPAA. A total of 27 publications were retrieved. Two papers fulfilled the inclusion criteria and 14 patients were enrolled. Among them, 11 underwent ileostomy pre-IPAA with a median age of 17 days. The median age of IPAA was 9 months. Seven children underwent protective ileostomy at the same time with an interval time of 3.7 months before fistula closure. The most common postoperative complications were obstruction (4/8, 50.0%) and enterocolitis (5/8, 62.5% ) . The average defecation frequency was at least 7 times at Month 6 after the last operation. The average defecation frequency declined to less than 4 times at Month 24 after the last operation and there was no defecation incontinence.Conclusions:IPAA may achieve an excellent prognosis for TCA patients. And short-term prognosis is correlated with age of preoperative fistula and radical operation. Timing of radical operation should be selected according to the characteristic of stool in fistula.