产前诊断与生后治疗一体化管理模式诊治胎儿先天性膈疝的研究  

Integrated mode of prenatal diagnosis and postnatal treatment for fetal congenital diaphragmatic hernia at a single center

作　　者：夏波[1,2] 俞钢 陈月清[1] 洪淳 余攀[1] 毛武[1] 余慧雯[1] 刘千里 刘翠芬[1] Xia Bo;Yu Gang;Chen Yueqing;Hong Chun;Yu Pan;Mao Wu;Yu Huiwen;Liu Qianli;Liu Cuifen(Department of Fetal Medicine,Guangdong Women&Children Hospital,Guangzhou 511440,China)

机构地区：[1]广东省妇幼保健院胎儿医学科,广州511440 [2]广州市妇女儿童医疗中心新生儿外科,510623 [3]广州医科大学附属第三医院小儿外科,510150

出　　处：《中华小儿外科杂志》2020年第5期421-425,共5页Chinese Journal of Pediatric Surgery

基　　金：广东省基础与应用基础研究基金(2020A1515010296);广东省科技计划项目(2014A020213021)。

摘　　要：目的总结采用一体化管理模式诊治胎儿先天性膈疝(congenital diaphragmatic hernia,CDH)的流程并探讨其对诊治CDH的影响。方法回顾分析2013年1月至2017年10月广东省妇幼保健院经一体化管理模式诊治的胎儿先天性膈疝的临床资料。277例产前诊断疑似CDH患儿中,排除14例生后手术中确诊为先天性膈膨升及5例生后确诊为胸骨后疝的患儿,确诊CDH的共258例患儿,其中,左侧206例,右侧51例,双侧1例。19例患儿合并其他畸形;产前评估轻度141例,中度62例,重度55例。35例家属选择终止妊娠,余223例CDH患儿中,男143例,女80例,产前诊断胎龄(25.4±6.2)周,出生胎龄(38.8±4.3)周,出生体重(3.25±0.53)kg。结果223例CDH患儿结局中,治愈184例,死亡39例。存活率由2014年的57.50%提高到2017年的81.82%;微创手术率由2014年的0提高到2017年的95.59%,微创术后复发率由2015年的20%降低到2017年的6.15%。39例死亡患儿中,31例(79.5%)合并重度肺动脉高压(其中24例因出生后合并重度肺动脉高压,经积极治疗病情无法纠正,术后死亡,5例为术后胸腔积液诱发肺动脉高压,2例为术后气胸诱发肺动脉高压),4例(10.3%)生后严重窒息导致不可纠正的酸中毒,2例(5.1%)术后下肢血流障碍,1例(3.2%)因术中发现一侧膈肌完全缺损放弃治疗,1例(3.2%)因术后两次复发放弃治疗。结论CDH的产前诊断和出生后一体化管理模式能提高轻度胎儿CDH的整体存活率,降低中重度CDH患儿病死率,是CDH患儿的有效管理模式。Objective To summarize the clinical characteristics of fetal congenital diaphragmatic hernia(FCDH)at a single center so as to explore the outcomes of integrated mode of fetal diagnosis and postnatal treatment for FCDH.Methods Clinical data of 258 hospitalized FCDH children from January 2013 to October 2017 were retrospectively analyzed.Thirty-five cases were chosen for termination of pregnancy(TOP).Among the remaining 223 CDH cases,there were 143 boys and 80 girls with an average prenatal diagnostic gestational age of(25.4±6.2)weeks,an average birth gestational age of(38.8±4.3)weeks and an average birth weight of(3.25±0.53)kg.The involved side was left(n=206),right(n=51)and bilateral(n=1).Nineteen cases were associated with other malformations.Prenatal ultrasonic severity was mild(n=141),moderate(n=62)and severe(n=55).Results The outcomes were curative(n=184)and death(n=39).The survival rate rose to 81.8%in 2017 from 57.7%in 2014;the rate of mini-invasive surgery from 0%in 2014 spiked to 95.6%in 2017,the recurrence rate of mini-invasive surgery decreased from 20%in 2015 to 6.15%in 2017.Among 39 deaths,31 cases(79.5%)had severe pulmonary hypertension(24 postnatal cases non-responding after aggressive treatment,including 5 lethal surgical cases per family requests,5 cases with postoperative pleural effusion and 2 cases with postoperative pneumothorax),4 cases(10.3%)had severe postnatal asphyxia,2 cases(5.1%)had lower blood blockage after surgery,1 case(3.2%)with unilateral complete diaphragm defect gave up treatment and 1 case(3.2%)gave up treatment after twice recurrence.Conclusions Integrated mode of prenatal diagnosis and postnatal treatment is an effective management mode for managing CDH.It may boost the survival rate for mild CDH and reduce the mortality for moderate/severe CDH.

关 键 词：胎儿 疝 横膈 先天性 一体化管理模式 

分 类 号：R714.5[医药卫生—妇产科学]