胎儿镜下气管栓塞术治疗重度先天性膈疝一例并文献复习

Application of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia: a case report and literature review

目的探讨胎儿镜下气管栓塞术(fetoscopic tracheal occlusion,FETO)在先天性膈疝(congenital diaphragmatic hernia,CDH)重度肺发育不良中的应用。方法回顾性分析一例产前诊断左侧CDH并重度肺发育不良的胎儿、应用FETO治疗的围手术期临床资料。同时通过检索PubMed、Springer Link、Google Scolar、中国生物医学文献数据库(CBM)、中国知网(CNKI)、万方和维普数据库,收集并详细分析截至2018年1月前发表的中英文相关文献,复习讨论胎儿CDH的FETO应用指征及术后序贯化管理的重要性。本次检索的中英文关键词为先天性膈疝(congenital diaphragmatic hernia)、胎儿镜下气管栓塞术(fetal endoscopic tracheal occlusion)和随机对照研究(randomized controlled trial)。检索后将综述类文献及对同一作者、医院、研究所、数据库重复报道病例予以剔除。结果患儿于孕中期行产检超声检查时发现左侧膈疝,遂转入本院胎儿医学中心进行详细检查并经多学科团队制定序贯化治疗方案。孕26+3周时胎儿超声检查评估为重度肺发育不良[实测/预测LHR比值(o/e LHR)为18.8%]。按计划于孕29周行FETO,操作过程顺利。FETO后于孕29+5周、30+4周时复查超声提示o/e LHR分别为23.6%、26.1%。孕33周时突发胎膜早破,子宫外产时治疗下取出胎儿气管球囊后予气管插管,转入新生儿外科监护室。患儿于生后11 h予体外膜肺氧合(extracorporeal membrane oxygenation,ECMO),生后5 d在ECMO支持下行左侧膈肌修补术;33 d时因出现肝肾功能衰竭,家长放弃治疗后死亡。检索文献仅发现2篇单中心RCT英文报道,一篇认为FETO未能提高CDH存活率,另一篇则认为FETO可明显提高CDH存活率。结论FETO的管理需要多学科团队配合,可促进胎儿肺发育,对提高重度CDH新生儿期存活率有着积极的意义。

Objective To summarize the experience of fetal endoscopic tracheal occlusion(FETO)for severe fetal congenital diaphragmatic hernia(CDH).Methods Retrospective reviews were conducted for clinical records of a severe fetal CDH undergoing FETO.The relevant reports were retrieved from the databases of PubMed,Springer Link,Google Scholar,CBM,CNKI,WAN-FANGDATA and CQVIP until January 2018.The key searching words included congenital diaphragmatic hernia,fetal endoscopic tracheal occlusion and randomized controlled trial(RCT).Selected criteria and post-FETO management were systematically summarized.Results A fetus with left-side CDH,detected ultrasonically during a second trimester,was transferred into our fetal care center for routine perinatal management.Severe pulmonary hypoplasia with an o/e LHR of 18.8%was considered after fetal ultrasound at 26+3 gestational weeks.FETO was performed at 29 gestational weeks.Serial ultrasonographs hinted at an increase in o/e LHR(23.6%at 29+5 gestational weeks and 26.1%at 30+4 gestational weeks).Unfortunately,premature rupture of membranes occurred at 33 gestational weeks and balloon was removed via ex utero intrapartum therapy.The neonatal boy received extracorporeal membrane oxygenation at Day 1 of life and underwent diaphragmatic defect repair at Day 5.At Day 33,he died of liver and renal failures.Two English literatures of RCT study were retrieved but their results were inconsistent in the improvement of survival rate.Conclusions Management of FETO may be accomplished with a coordination of a multi-disciplinary team.And FETO improves pulmonary hypoplasia and helps to boost neonatal survival.