【摘要】 先天性巨结肠(Hirschsprung’s disease,HD)是肠肌间神经丛神经节细胞的缺如导致肠段丧失功能,故又称先天性无神经节细胞症,是常见的先天性消化道畸形,发病率紧次于直肠肛门畸形,居先天性消化道畸形第二位,发病率为1/2000-1/5000,男女比3-4:1,婴儿及儿童期的HD病史典型,较易诊断,而新生儿HD临床表现变化多端,发病轻重不同,大多数在出生后1周内发生急性肠梗阻;>90%有胎粪性便秘,24-48 h胎粪未能排出,伴发并发症各异,临床上容易忽视而造成误诊,常规治疗是以手术治疗为主,本文拟对新生儿HD诊断方法与治疗进展予以综述。更多还原

【Abstract】 Hirschsprung’s disease(HD) has function loss syptom of intestinal segment for lacking intestinal myenteric plexus ganglion cells, all called congenital aganglionosis, which is a common congenital digestive tract anomaly, with the second incidence following anorectal malformation. HD has incidence of 1/2000-15000, male to female ratio is 3-4:1. HD has typical history at infant and childhood and is easy to diagnose, while HD neonates have varied clinical manifestations, with different severity. Most of them have acute intestinal obstruction within one week after birth;>90% have meconium constipation, meconium fails to be discharged within 24-48 h, with different complications, which are easily ignored and misdiagnosed clinically. Routine treatment is mainly surgical treatment. The article reviews diagnosis method and treatment advance of HD in neonates.更多还原