新生儿膈疝产时宫外治疗的临床经验及预后相关因素

Clinical experience of ex-utero intrapartum treatment and prognostic factors of neonatal diaphragmatic hernia

目的总结新生儿先天性膈疝产时宫外治疗(Ex utero intrapartum treatment,EXIT)的单中心经验,并分析与患者预后相关的因素。方法对2011年1月1日至2020年12月31日期间在首都儿科研究所附属儿童医院接受治疗的新生儿先天性膈疝进行回顾性研究,患者均接受包括产前筛查、诊断、评估,产时监护及呼吸支持,产后人工转运、手术、随访在内的“产前-产时-产后”多学科综合诊疗模式。对其中22例产前诊断为先天性膈疝、经评估病情较重、出生后呼吸困难的新生儿应用EXIT(为EXIT组,其余患者归入非EXIT组),对患者的临床特征、手术相关参数、临床结局等资料进行比较。结果共95例纳入研究,获随访6个月至10年。EXIT组共22例,其中21例接受手术治疗,存活19例(术后存活率为90.5%);非EXIT组共73例,其中60例接受手术治疗,存活43例(术后存活率为71.7%)。Logistic回归分析显示,行产时宫外治疗[OR=0.104,(95%CI:0.022~0.493)]、产前诊断时间[OR=0.863,(95%CI:0.783~0.952)]与肝脏疝入胸腔[OR=9.657,(95%CI:3.094~30.137)]三个因素为先天性膈疝患者预后状况的独立影响因素。根据ROC曲线结果可知,产前诊断时间最优诊断效能对应的cutoff值为24.5周,即产前诊断孕周小于24.5周的患者出生后存活率显著低于产前诊断孕周大于等于24.5周的患者。结论EXIT安全可行,可有效改善先天性膈疝新生儿患者预后,产时宫外治疗、产前诊断胎龄及肝脏疝入胸腔是新生儿先天性膈疝的预后相关因素。

Objective To conduct a retrospective review of our hospital's single-center experience in the treatment of neonatal congenital diaphragmatic hernia(CDH)and elucidate the relevant prognostic factors.Methods From January 1,2011 to December 31,2020,a retrospective review was performed for 95 CDH neonates.All of them received prenatal-perinatal-postpartum multidisciplinary diagnosis and treatments including prenatal screening,prenatal diagnosis,evaluations,intrapartum monitoring and transfer on respiratory support,surgery and follow-ups.Among them,22 cases with severe disease and postnatal dyspnea received ex utero intrapartum treatment(EXIT).Clinical characteristics,surgical parameters and outcomes were compared.Results Among them,62 survived and 33 died.Twenty-two received EXIT during labor,21 were operated and 19 survived with a postoperative survival rate of 90.5%.There were 73 cases in non-EXIT group,60 cases were operated and 43 cases survived with a postoperative survival rate of 71.7%.Logistic regression analysis showed that EXIT during labor(OR=0.104,[95%CI:0.022-0.493]),gestational age at prenatal diagnosis(OR=0.863,[95%CI:0.783-0.952])and liver herniation into chest(OR=9.657,[95%CI:3.094-30.137])were independent prognostic factors for CDH children.According to the receiver operating characteristic(ROC)curve,the cutoff value of maximizing the diagnostic efficiency of prenatal diagnostic age was 24.5 weeks.The survival rate of children with a gestational age under 24.5 weeks was significantly lower than that of those with a gestational age above 24.5 weeks.Conclusion Both safe and feasible,EXIT may improve the prognosis of neonatal CDH effectively.EXIT,gestational age of prenatal diagnosis and liver herniation into thoracic cavity are independent prognostic factors for CDH neonates.