目的:为了提高对袋状直肠、乙状结肠综合征的诊断和治疗水平。方法:4例袋状直肠、乙状结肠综合征患儿均为女性,合并先天性肛门闭锁,婴儿期行会阴肛门成型术。术后4例均进行性排便困难,充盈性大便失禁,经保守疗法无效。钡灌肠可见直肠、乙状结肠扩张呈囊袋状。手术发现扩张肠管无蠕动,与正常肠管分界明显,切除病变肠管。组织学检查肠壁肌层增生、肥厚,神经节细胞正常。结果:术后2例患儿排便正常,无污粪及大便潴留,1例尚未关瘘,1例大便次数多,有污粪。结论:袋状直肠、乙状结肠综合征是直肠、乙状结肠局限性高度扩张,合并有瘘的肛门闭锁和(或)同时合并泌尿生殖系畸形的一种疾病。保守治疗无效,必须手术切除扩张肠管。 Objective: In order to improve the diagnosis and treatment of pouch rectum and sigmoid syndrome. Methods: All 4 children with pouch rectum and sigmoid syndrome were female with congenital anal atresia and perineal anoplasty during infancy. Postoperative four cases were progressive defecation, filling stool incontinence, conservative treatment ineffective. Barium enema visible rectum, sigmoid colon was dilated bag. Surgical discovery of no expansion of intestinal peristalsis, and clear boundaries between normal bowel, removal of diseased bowel. Histological examination of intestinal wall muscle hyperplasia, hypertrophy, ganglion cells normal. Results: Two cases of postoperative children with normal bowel movements, no stool and retention of stool, one case has not closed fistula, one case of stool frequency and stool. Conclusions: Pouch rectum and sigmoid colon syndrome are a type of diseases in which the rectum and sigmoid colon are highly localized, anal atresia with fistula and / or genitourinary malformations combined. Conservative treatment ineffective, surgical resection of the bowel must be removed.