先天性巨结肠症（Ｈｉｒｓｃｈｓｐｒｕｎｇ’ｓｄｉｓｅａｓｅ，ＨＤ）是一种肠道发育畸形，又称无神经节细胞症。其发病率大约是１／５０００。病因多为结肠远端及直肠缺乏神经节细胞，远端肠管呈痉挛性狭窄；近端肠管扩张、肥厚。ＨＤ根据其发生长短不同分为短段型、长段型 Hirschsprung’s disease (HD) is a form of gut dysplasia, also known as ganglion cell disorder. Its incidence is about 1/5000. Most of the etiology of the distal colon and rectum lack of ganglion cells, distal intestine was spastic stenosis; proximal bowel dilatation, hypertrophy. HD is divided into short-segment and long-segment according to its occurrence