无明确产前诊断的新生儿先天性膈疝的临床诊治体会

Clinical diagnoses and treatments of congenital diaphragmatic hernia of neonates without a prenatal diagnosis

目的总结产前未明确诊断的新生儿先天性膈疝(congenital diaphragmatic hernia,CDH)病例的临床特点、治疗方法和预后情况,分析非母胎诊治中心对新生儿CDH的临床诊治经验和体会。方法收集郑州大学附属儿童医院2010年1月至2018年12月急诊收治的136例新生儿CDH病例资料,5例术前因家属原因未接受治疗,未纳入研究,余131例患儿纳入研究。131例患儿均无产前诊断,其中男77例,女54例,出生时胎龄(38.3±1.5)周,范围32.0~41.0周,出生体重(3102.8±473.6)g,范围2000.0~4120.0 g。左侧膈疝115例,右侧膈疝16例,合并肺动脉高压21例,伴发畸形81例。92例术前需机械通气,39例术前可维持自主呼吸。依据急诊入院时的日龄分为:≤48 h入院组(90例),>48 h入院组(41例)。结果经胸腔镜手术58例,开腹手术71例。共计死亡14例,其中术前死亡2例,术后死亡12例。术前抢救治疗无效死亡2例,接受手术治疗的患儿总计129例,术后存活117例,12例死亡或放弃治疗,接受治疗病例的总存活率为89.3%(117/131)。≤48 h入院组的病死率为15.6%(14/90),高于>48 h入院组的病死率为0(P<0.05)。早产儿、合并肺动脉高压以及严重心脏畸形病例的病死率相对较高(P<0.05),死亡病例的入院pH值、PaO2、术后平均pH值明显低于存活病例,入院PaCO2明显高于存活病例(P<0.05)。结论无产前诊断的新生儿CDH产后发病越早,病死率越高;合并肺动脉高压、早产和严重心脏畸形的CDH新生儿病死率较高。

Objective To summarize the clinical characteristics,treatments and prognoses of congenital diaphagmatic hernia(CDH)in prenatal undiagnosed neonates and to explore the clinical diagnostic and therapeutic experiences of neonatal CDH at a non-tertiary center.Methods From January 2010 to December 2018,a total of 136 CDH neonates were admitted by emergency clinic.Five untreated cases due to special reasons were excluded.No prenatal diagnosis was made.There were 77 boys and 54 girls with a gestational age of(38.3±1.5)(32.0-41.0)weeks and a birth weight of(3102.8±473.6)(2000.0-4120.0)grams.The involved side was left(n=115)and right(n=16).The complications included pulmonary hypertension(n=21)and associated malformations(n=81).The preoperative status was mechanical ventilation(n=92)and spontaneous breathing(n=39).According to admission age,they were divided into two group of admission before and after 48 hours after birth.Results The specific procedures included thoracoscopy(n=58)and laparotomy(n=14).Death occurred preoperatively(n=2)and postoperatively(n=12).Two actively treated cases eventually died preoperatively and 129 cases were operated.Twelve cases died or abandoned treatment at post-operation and 117 cases(89.3%)survived.The mortality rate of≤48 h admission group(14/90,15.6%)was higher than that of>48 h admission group(0)(P<0.05).The mortality rate of premature infants,pulmonary hypertension and severe cardiac malformation were relatively high(P<0.05).And the values of pH and PaO2 at admission,mean postoperative pH in deceased cases were significantly lower than those in survivors.And PaCO2 at admission was significantly higher in deceased cases than that in survivors(P<0.05).Conclusions Prenatally undiagnosed neonatal CDH,especially in severe cases,may present challenges for postpartum treatment.With earlier onset and more severe condition,the higher mortality rate will be.Mortality is higher in CDH with pulmonary hypertension,preterm birth and severe cardiac malformation.