袋状直肠、乙状结肠综合征

Atonic Baggy Rectum and Sigmoid Syndrome

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:To improve the diagnosis and treatment for the syndrome of atonic bag- gy rectum and sigmoid.Methods:Four cases of atonic baggy rectum and sigmoid were reviewed. All patients were female and had a perineal anoplasty in infancy.Only one had postoperative anal stenosis.All had intractable constipation and soiling,and unresponsive to conservative treatment. A dilated atonic rectum and sigmoid was demonstrated by barium enema.The non-peristasis di- latated rectosigmoid was found demarcated clearly from the normal bowel at operation.Histologi- cal examination revealed ganglion cells in all specimens.Results:Two had normal bowel move- ments after resection of the big bowel,one received a colostomy,and one remained soiling after swenson's resection.Conclusion:this syndrome is due to local dilatation of rectum and sigmoid, this abnormality is usually associated with anorectal and urogenital deformities.If conservative treatment is ineffective,the pathologic bowel should be resected.