摘要：
Objective To explore the clinical profiles,multidisciplinary treatment (MDT) and prognosis of neonatal congenital severe diaphragmatic hernia.Methods A total of 42 neonatal severe congenital diaphragmatic hernia cases were reviewed from September 2007 to April 2017.They were divided into MDT group (n =23) and non-MDT group (n =19).Gender,age,birth weight,gestational age,prenatal diagnosis,Apgar score,hepatic position,defect size and prognosis were reviewed.And t-test,rank-sum test and analysis of variance were performed.Results Prenatal diagnosis (17 ％ vs 4 ％,P =0.011) and 1 min Apgar score [(6.2 ± 1.7) vs (7.2 ± 1.4),P =0.044] had great prognostic impacts.However,lung-to-head ratio,hepatic position and defect position showed no significant statistical differences.As compared to non-MDT group,the average 1min Apgar score [(6.2 ± 1.7) vs (7.2 ± 1.4),P=0.044],ratio of prenatal diagnosis (91.3％ vs 57.9％,P=0.030),ratio of transfer (92.3％ vs 47.3％,P =0.002),gestational age of prenatal diagnosis [(26.4 ± 5.5) vs (31.6 ± 7.2),P =0.020],proportion of endoscopic surgery (13 ∶ 5 vs 2 ∶ 11,P =0.003),ventilator support time [(6.8 ± 2.7) vs (4.1 ± 2.3) days,P =0.024] had significant statistical differences.However,birth weight,gestational age,operation age,length of operation stay,survival rate and recurrence rate had no intergroup difference.Conclusions The prognosis of neonatal severe diaphragmatic hernia is correlated with prenatal diagnosis and 1 minute Apgar score after birth.And MDT is feasible and effective for severe diaphragmatic hernia.As a non-standard intervention method,fetoscopy is feasible and widely accepted after birth.%目的 分析近10年经治的新生儿重症膈疝患儿围生期临床资料、相关治疗和预后情况,探讨新生儿重症膈疝预后相关因素及多学科综合治疗.方法 回顾性分析首都儿科研究所附属儿童医院2007年9月至2017年4月收治的42例新生儿重症膈疝患儿资料.根据是否引入多学科综合治疗模式(MDT)分为非MDT组和MDT组,其中MDT组23例,非MDT组19例.对性别、年龄、出生体重、胎龄、产前诊断、肝脏位置、Apgar评分、膈肌缺损大小及预后情况进行对比较研究.统计学处理采用t检验、秩和检验和方差分析.结果 早期产前诊断(＜25周)较晚期产前诊断(≥25周)病例存活率(17％比4％,P=0.011)明显降低,生后1min Apgar评分越低者存活率越低(P=0.029),以上差异均有统计学意义,然而肺/头比(LHR)值、肝脏位置、膈肌缺损大小对患儿预后无影响.相较于非MDT组,MDT组平均1 min Apgar评分[(6.2±1.7)比(7.2±1.4),P=0.044]明显变小,产前诊断比率(91.3％比57.9％,P=0.030)、转运比率(92.3％比47.3％,P=0.002)明显增多,产前诊断胎龄[(26.4±5.5)周比(31.6±7.2)周,P=0.020]减小,腔镜手术占比明显增加(13∶5和2∶11,P=0.003),术后平均呼吸机使用时间[(6.8±2.7)d和(4.1±2.3)d,P=0.024]延长,以上差异均具有统计学意义,两组的性别比、缺损位置、出生胎龄、出生体重、术前死亡或放弃、手术年龄、平均住院时间、存活及复发率差异无统计学意义.结论 新生儿重症膈疝预后与产前诊断胎龄及生后1 minApgar评分相关,MDT模式治疗重症膈疝是可行有效的,胎儿镜并非标准干预手段,产后手术治疗可行性高、接受度广.
