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.
参考文献/References:
[1] Paoletti M, Raffler G, Gaffi MS, et al.Prevalence and risk factors for congenital diaphragmatic hernia:a global view[J].J Pediatr Surg, 2020, 55(11):2297-2307.DOI:10.1016/j.jpedsurg.2020.06.022.
[2] Wynn J, Krishnan U, Aspelund G, et al.Outcomes of congenital diaphragmatic hernia in the modern era of management[J].J Pediatr, 2013, 163(1):114-119.e1.DOI:10.1016/j.jpeds.2012.12.036.
[3] Morini F, Lally PA, Lally KP, et al.The congenital diaphragmatic hernia study group registry[J].Eur J Pediatr Surg, 2015, 25(6):488-496.DOI:10.1055/s-0035-1569151.
[4] Deprest J, Flake A.How should fetal surgery for congenital diaphragmatic hernia be implemented in the post-TOTAL trial era:a discussion[J].Prenat Diagn, 2022, 42(3):301-309.DOI:10.1002/pd.6091.
[5] 赵云龙, 马立霜, 蒋宇林, 等.新生儿膈疝产时宫外治疗的临床经验及预后相关因素[J].临床小儿外科杂志, 2021, 20(9):808-813.DOI:10.12260/lcxewkzz.2021.09.002. Zhao YL, Ma LS, Jiang YL, et al.Clinical experience of ex-utero intrapartum treatment and prognostic factors of neonatal diaphragmatic hernia[J].J Clin Ped Sur, 2021, 20(9):808-813.DOI:10.12260/lcxewkzz.2021.09.002.
[6] 王莹, 马立霜, 刘超, 等.产前诊断先天性膈疝的转运救治机制与临床实证分析[J].临床小儿外科杂志, 2022, 21(1):58-62.DOI:10.3760/cma.j.cn.101785-202107031-011. Wang Y, Ma LS, Liu C, et al.Transfer and treatment mechanism and practical effect analysis of prenatally diagnosed congenital diaphragmatic hernia[J].J Clin Ped Sur, 2022, 21(1):58-62.DOI:10.3760/cma.j.cn.101785-202107031-011.
[7] Snoek KG, Reiss IKM, Greenough A, et al.Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe:the CDH Euro consortium consensus-2015 update[J].Neonatology, 2016, 110(1):66-74.DOI:10.1159/000444210.
[8] Horn-Oudshoorn EJJ, Vermeulen MJ, Knol R, et al.The oxygen saturation index as early predictor of outcomes in congenital diaphragmatic hernia[J].Neonatology, 2023, 120(1):63-70.DOI:10.1159/000527407.
[9] Ito M, Terui K, Nagata K, et al.Clinical guidelines for the treatment of congenital diaphragmatic hernia[J].Pediatr Int, 2021, 63(4):371-390.DOI:10.1111/ped.14473.
[10] Hoffman SB, Massaro AN, Gingalewski C, et al.Survival in congenital diaphragmatic hernia:use of predictive equations in the ECMO population[J].Neonatology, 2011, 99(4):258-265.DOI:10.1159/000319064.
[11] 魏延栋, 马立霜, 王莹, 等.胸腔镜手术治疗先天性膈疝的经验及技术要点分析[J].临床小儿外科杂志, 2021, 20(9):819-824.DOI:10.12260/lcxewkzz.2021.09.004. Wei YD, Ma LS, Wang Y, et al.Key technical essentials and experiences during thoracoscopy for congenital diaphragmatic hernia[J].J Clin Ped Sur, 2021, 20(9):819-824.DOI:10.12260/lcxewkzz.2021.09.004.
[12] 李海振, 马立霜, 王莹, 等.新生儿先天性膈疝预后相关因素分析[J].临床小儿外科杂志, 2022, 21(5):458-462.DOI:10.3760/cma.j.cn101785-202012069-011. Li HZ, Ma LS, Wang Y, et al.Analysis of prognostic factors of neonatal congenital diaphragmatic hernia[J].J Clin Ped Sur, 2022, 21(5):458-462.DOI:10.3760/cma.j.cn101785-202012069-011.
[13] Snoek KG, Greenough A, van Rosmalen J, et al.Congenital diaphragmatic hernia:10-year evaluation of survival, extracorporeal membrane oxygenation, and foetoscopic endotracheal occlusion in four high-volume centres[J].Neonatology, 2018, 113(1):63-68.DOI:10.1159/000480451.
[14] Snyder AN, Cheng T, Burjonrappa S.A nationwide database analysis of demographics and outcomes related to Extracorporeal Membrane Oxygenation (ECMO) in congenital diaphragmatic hernia[J].Pediatr Surg Int, 2021, 37(11):1505-1513.DOI:10.1007/s00383-021-04979-y.
[15] Apfeld JC, Kastenberg ZJ, Gibbons AT, et al.Treating center volume and congenital diaphragmatic hernia outcomes in California[J].J Pediatr, 2020, 222:146-153.e1.DOI:10.1016/j.jpeds.2020.03.028.
[16] Jancelewicz T, Langham MR Jr, Brindle ME, et al.Survival benefit associated with the use of extracorporeal life support for neonates with congenital diaphragmatic hernia[J].Ann Surg, 2022, 275(1):e256-e263.DOI:10.1097/SLA.0000000000003928.
[17] Khachane Y, Halliday R, Thomas G, et al.Outcomes for infants with congenital diaphragmatic hernia:a single campus review with low extracorporeal membrane oxygenation utilisation[J].J Paediatr Child Health, 2022, 58(1):90-96.DOI:10.1111/jpc.15659.
[18] Lum LCS, Ramanujam TM, Yik YI, et al.Outcomes of neonatal congenital diaphragmatic hernia in a non-ECMO center in a middle-income country:a retrospective cohort study[J].BMC Pediatr, 2022, 22(1):396.DOI:10.1186/s12887-022-03453-5.
[19] Larsen UL, Jepsen S, Str?m T, et al.Congenital diaphragmatic hernia presenting with symptoms within the first day of life;outcomes from a non-ECMO centre in Denmark[J].BMC Pediatr, 2020, 20(1):196.DOI:10.1186/s12887-020-02072-2.
[20] Yang MJ, Fenton S, Russell K, et al.Left-sided congenital diaphragmatic hernia:can we improve survival while decreasing ECMO?[J].J Perinatol, 2020, 40(6):935-942.DOI:10.1038/s41372-020-0615-3.
[21] Bhombal S, Patel N.Diagnosis and management of pulmonary hypertension in congenital diaphragmatic hernia[J].Semin Fetal Neonatal Med, 2022, 27(4):101383.DOI:10.1016/j.siny.2022.101383.
[22] Ferguson DM, Gupta VS, Lally PA, et al.Early, postnatal pulmonary hypertension severity predicts inpatient outcomes in congenital diaphragmatic hernia[J].Neonatology, 2021, 118(2):147-154.DOI:10.1159/000512966.
[23] Patel N, Massolo AC, Paria A, et al.Early postnatal ventricular dysfunction is associated with disease severity in patients with congenital diaphragmatic hernia[J].J Pediatr, 2018, 203:400-407.e1.DOI:10.1016/j.jpeds.2018.07.062.
[24] Yoneda K, Amari S, Mikami M, et al.Development of mortality prediction models for infants with isolated, left-sided congenital diaphragmatic hernia before and after birth[J].Pediatr Pulmonol, 2023, 58(1):152-160.DOI:10.1002/ppul.26172.
[25] Kadir D, Lilja HE.Risk factors for postoperative mortality in congenital diaphragmatic hernia:a single-centre observational study[J].Pediatr Surg Int, 2017, 33(3):317-323.DOI:10.1007/s00383-016-4032-9.
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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