先天性袋状结肠的临床认识和诊治进展

Clinical cognition and recent advances in the diagnosis and treatment of congenital pouch colon

先天性袋状结肠是一种较为罕见的先天性畸形,表现为结肠缩短并呈明显的袋状扩张,同时多合并肛门直肠畸形,袋状扩张结肠一般通过瘘管与泌尿生殖道相连。先天性袋状结肠临床特征典型,诊断较为容易,但其临床处理及手术方式等存在争议,大多考虑先行造瘘进行分期手术,并根据剩余结肠的长度决定是否予以结肠成形。

As an extremely rare malformation, congenital pouch colon is characterized by shortened colon, markedly dilated pouch and anorectal malformations. In boys, pouch usually terminates in a colovesical fistula;in girls, terminal fistula opens into urethra or vestibule. Clinical presentations are typical and and its diagnosis is easily made by a large gas shadow or air-fluid level on radiograph. Its clinical managements and surgical options have remained controversial. Staged repair is performed after colostomy or ileostomy. Yet tubularization of dilated segment is dependent on the length of normal colon.