先天性巨结肠症(Hirschsprung’s disease,HSCR)又称肠无神经节细胞症(agangliono-sis),是一种神经嵴细胞源性疾病和多基因遗传病。其主要病因是患儿在胚胎发育过程中神经嵴细胞迁移障碍,肠神经发育出现停顿,肠壁肌间神经丛的神经节细胞缺失,以致受累肠段异常收缩,其近端结肠代 Hirschsprung’s disease (HSCR), also known as agangliono-sis, is a neural crest-derived and polygenic disease. The main reason is that during the process of embryonic development in children with neural crest cell migration disorders, intestinal development of pause, myenteric plexus of the ganglion cells missing, resulting in an abnormal contraction of bowel involvement, the proximal colon generation