先天性巨结肠症(hirschsprung′s disease,HD)又称先天性无神经节细胞症,其临床表现主要为顽固性便秘腹胀。在病理学上,HD是一种由于肠壁黏膜下层神经丛和肌间神经丛中神经节细胞缺失所导致的先天性肠道阻塞性疾病。HD在男性儿童中更常见,80%～85%的患者无神经节细胞,病变局限于直肠和乙状结肠,10%的患者病变累及更近端的结肠,5%～8%的全结肠HD患者病变可累及小肠的不同水平。除了污染、有害食品添加剂、宫内病毒感染等可能病因外,近 Hirschsprung’s disease (HD), also known as congenital ganglion cell disease, its main clinical manifestations of refractory constipation bloating. Pathologically, HD is a congenital intestinal obstruction caused by a loss of ganglion cells in the submucosal plexus and myenteric plexus of the intestinal wall. HD is more common in male children, with 80% to 85% of patients having no ganglion cells, localized in the rectum and sigmoid colon, in 10% of patients involving the more proximal colon, and in 5% to 8% of patients with total colon HD May affect the different levels of the small intestine. In addition to pollution, harmful food additives, intrauterine virus infection and other possible causes