先天性膈疝的诊治进展

摘要

Congenital diaphragmatic hernia (CDH) continues to be a critical condition in neonatal surgery.Complicated with hypoplastic lungs,it has an extremely high mortality.Currently its underlying cause has remained elusive.It is probably correlated with an abnormal signaling pathway of retinoid acid.Most patients may be detected prenatally.The prognosis of CDH may be predicted by the results of imaging examination.As a major treatment for CDH,surgery is composed of open surgery and minimally invasive surgery (MIS).The arrival of small videoscopic instrumentation allows the management of neonatal CDH by pediatric surgeons trained in MIS procedures.%先天性膈疝是新生儿危重症之一,因其合并严重肺发育不良,导致其病死率极高.目前病因及发病机制尚未明确,可能与胚胎期维甲酸通路异常有关.多数患儿可以产前发现,并能依据影像学检查结果对患儿的预后进行评估.外科手术是抢救和治疗先天性膈疝的必要手段,目前主要分为开放和腔镜手术.随着微创技术的发展,越来越多的临床中心开始开展腔镜手术.