Meng Chuyi,Feng Zhong,Wang Ying,et al.Analysis of clinical outcomes for infants with congenital diaphragmatic hernia under a multidisciplinary diagnostic and treatment approach[J].Journal of Clinical Pediatric Surgery,2023,22(08):706-712.[doi:10.3760/cma.j.cn101785-202304028-002]
多学科诊疗模式下先天性膈疝患儿的临床转归
- Title:
- Analysis of clinical outcomes for infants with congenital diaphragmatic hernia under a multidisciplinary diagnostic and treatment approach
- Keywords:
- Hernias; Diaphragmatic; Congenital; Multidisciplinary Treatment; Extracorporeal Membrane Oxygenation; Treatment Outcome; Comparative Study
- 摘要:
- 目的 探讨多学科诊疗模式下先天性膈疝（congenital diaphragmatic hernia，CDH）的临床转归以及预后相关因素。方法 本研究为回顾性研究。以2016年1月至2022年8月首都儿科研究所附属儿童医院收治的103例产前诊断为CDH的患儿为研究对象，以出生24 h内出现体外膜肺氧合（extracorporeal membrane oxygenation，ECMO）使用指征为分组标准，将患儿分为ECMO达标组和非ECMO达标组，其中ECMO达标组39例，非ECMO达标组64例。分析两组患儿临床预后、超声心动图表现，以及ECMO达标组患儿死亡风险因素。结果 103例患儿总病死率为29.1%（95%CI：20.6%~38.9%）。ECMO达标组病死率为53.9%（95%CI：37.2%~69.9%），高于非ECMO达标组的14.1%（95%CI：6.6%~25.0%）），该组存活患儿有创呼吸机使用时间为336.5（160.5，578.5） h，住院时长为37.5（22.8，51.3） d。非ECMO达标组有创呼吸机使用时间为177.0（121.0，311.0） h、住院时长为25（19，34） d。与非ECMO达标组相比，ECMO达标组右侧膈疝占比更多，肝脏疝入发生率更高，膈肌缺损程度更大（P<0.05），早期肺动脉高压更严重（P<0.01），右心增大（69.7%比27.3%，P<0.01）以及左心功能不全（18.2%比0，P<0.01）的发生率更高。多因素回归分析结果显示，诊断胎龄<25周、动脉二氧化碳分压（arterial partial pressure of carbon dioxide，PaCO2）偏高、氧合指数（oxygenation index，OI）偏大是ECMO达标组CDH患儿死亡的独立危险因素（P<0.05）。结论 在CDH患儿诊治中，采取产前-产时-产后一体化诊疗模式，优化通气管理和血管活性药物的精准个性化使用，可获得与ECMO中心相仿的治疗效果。建议ECMO在CDH患儿中的使用更加谨慎。患儿诊断胎龄早及生后早期氧合功能差与死亡结局密切相关。
- Abstract:
- Objective To explore the clinical outcomes and prognostic factors associated with infants of congenital diaphragmatic hernia (CDH) under multidisciplinary treatments.Methods The relevant clinical data were collected from 103 cases of prenatally diagnosed CDH treated from January 2016 to August 2022.Based upon whether or not fulfilling the criteria for using extracorporeal membrane oxygenation (ECMO) within 24h post-birth, they were assigned into two groups of ECMO-indicated (n=39) and non-ECMO-indicated (n=64).Clinical outcomes, echocardiographic findings and independent risk factors were examined for mortality in ECMO-indicated group.Results The overall discharge mortality rate was 29.1%(95%CI:20.6%-38.9%).And mortality rate in ECMO-indicated group was higher than that in non-indicated group[53.9%(95%CI:37.2%-69.9%) vs.14.1%(95%CI:6.6%-25%)].The median duration of invasive ventilator use in survivors was 336.5(160.5, 578.5) hours with a median hospital stay of 37.5(22.8, 51.3) days.In contrast, 177.0(121.0-311.0) hours and 25(19, 34) days in non-indicated group respectively.Proportion of right-sided diaphragmatic hernia, occurrence of hepatic hernia and extent of diaphragmatic defect were higher in ECMO-indicated group (P<0.05).Early pulmonary hypertension of ECMO-indicated group was more severe(P<0.01) with higher incidences of right heart enlargement (69.7% vs.27.3%, P<0.01) and left ventricular dysfunction (18.2% vs.0, P<0.01).Multivariate regression analysis revealed that for ECMO-indicated group, a diagnostic gestational age of 25 weeks, PaCO2 and oxygenation index (OI) are independent morality risk factors (P<0.05).Conclusion Despite non-using ECMO, our center achieved a mortality rate comparable to that of international high-volume treatment centers for high-risk CDH patients through strengthening an integrated management model before, during and after childbirth.ECMO-indicated CDH patients have more severe pulmonary arterial hypertension and a higher incidence of heart dysfunction.In addition, gestational age at diagnosis <25 weeks, PCO2 and OI were independent risk factors for mortality in this cohort of patients.
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备注/Memo
收稿日期:2023-04-11。
基金项目:2022年度慢性病防治与健康教育科研项目（BJMB0012022028001）；北京市自然科学基金资助项目（7224321）；北京市儿科学科协同发展中心儿科重点专项（XTZD20180305）
通讯作者:马立霜,Email:malishuang2006@126.com