婴幼儿先天性巨结肠症的治疗进展

Therapeutic Progress of Hirschsprung′s Disease in Children and Infants

先天性巨结肠症（HD）是一种比较多见的消化道畸形,其发病率与先天性肛门、直肠畸形不相上下,临床表现以便秘为主,病变肠管神经节细胞缺如。手术切除病变肠管是治疗HD的主要方法,近几十年来世界各国儿外工作者进行了大量的病因、病理、组织化学、基因研究及手术技术的创新改进,使HD的病因探讨,诊断方法和手术效果及并发症的降低等方面均有明显的进步和提高。综合近年国内外学者治疗HD的技术现状与进展予以综述。

Hirschsprung＇s disease is a common digestive tract abnormity,it has the similar incurfence rate to that of congenital anus or rectum abnormality. It commonly presents as constipation, absence of ganglion cells in pathological intestinal canal. The major treatment is to surgieally remove the abnormal colon section. Significant advanees have been made in understanding its etiologies and pathology in the last deeades, especially with the explosion of molecular genetic techniques and early diagnosis. And the advancement of treatment strategies for Hirsehsprung＇s disease continues with reduced patient morbidity and improved outcomes. This article describes the techniques and advancements that have occurred recently in the surgical management of this disease.