新生儿先天性膈疝11例尸检分析

Congenital diaphragmatic hernia in neonatal autopsies:A report of 11 cases

目的:研究新生儿先天性膈疝(CDH)的死亡原因及病理变化特点。方法:应用大体观察,各重要器官作组织切片后镜检,结合临床资料对1080例死亡新生儿进行尸检分析。结果:检出 CDH11例(1%)。其中1987～1996年内约300例中检出9例。左8例,右3例,均为巨型Bochdalek 疝。疝入器官多寡不一。患侧伴或不伴有对侧肺明显受压,萎陷,心纵隔移位5例。各重要脏器严重淤血。此外,伴有心血管畸形5例,左肺上下叶倒置1例,肝左叶扭转伴胆囊缺如1例,双侧并指并趾畸形1例。结论:近年来新生儿 CDH 检出率有所增加,不容忽视。多发生于左侧。多伴有其它器官畸形,但未发现明显遗传倾向。肺受压萎缩及心纵隔移位为主要死亡原因。

Objective:To discuss the cause of death and the pathological changes of congenital diaphragmatic hernia(CDH).Methods:Autopsies were performed on 1080 neonatal cadavers. Results:Eleven cases of CDH were found.Nine of the 11 cases were found in recent 300 autopsies (from 1987 to 1996).In all the 11 cases a large Bochdalek hernia(left 8 and right 3)was found. Herniated organs were different in each cadaver,but in all cadavers the lungs of the affected side were distinctly compressed and underdeveloped with or without involving the opposite side.Five cases had deviation of mediastinum.All important organs were seriously congested.In addition,5 cases were accompanied with cardiovascular deformity,1 case with transposition of left upper and lower pulmonary lobes,1 case with torsion of left hepatic lobe and gallbladder agenesis,and 1 case with ploy-dactylion.Conclusions:The increasing incidence of CDH in neonatal cadavers in re- cent years must be aware of.CDH mainly occours in left side.Most cases accompany with other deformities,but the hereditary trend has not been found.Serious compression of lungs and devia- tion of cardiomediastinum are the main factors causing death.