先天性肛门直肠畸形是常见的消化道畸形 ,是世界卫生组织常规监测的先天性畸形之一。其发生率约为 1/ 5 0 0 0～1/ 15 0 0 ,有家族发病史者约占 1%～ 9%。目前认为 ,肛门直肠畸形的发生是遗传因素和环境因素共同作用的结果。流行病学和动物实验表明 ,遗传因素在其发病过程中发 Congenital anorectal malformations are common gastrointestinal malformations and are one of the congenital malformations routinely monitored by the World Health Organization. The incidence of about 1/500 ~ 1/15 0 0, with a history of family history of about 1% to 9%. Currently, the occurrence of anorectal malformations is the result of both genetic and environmental factors. Epidemiological and animal experiments show that genetic factors in the pathogenesis of hair