Prevalence and early surgical outcome of congenital diaphragmatic hernia in the Netherlands: a population-based cohort study from the European Pediatric Surgical Audit.

Nadine Maria Teunissen, Horst Daniels, J Marco Schnater, Ivo de Blaauw, René M H Wijnen
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Abstract

Background: Congenital diaphragmatic hernia (CDH) is a rare birth defect with substantial mortality. This study aims to generate a population-based overview of CDH care and outcomes in the Netherlands. Moreover, it assesses interhospital variations between the two Dutch CDH expert centres.

Methods: This study uses data from the Dutch branch of the European Pediatric Surgical Audit, a prospective clinical audit for congenital anomalies. Data of all patients with CDH treated between 2014 and 2021 were included for epidemiological analysis. For comparative analyses, patients presenting after the neonatal period or not treated in the two CDH expert centres were excluded. Identified interhospital variations were assessed using regression analysis.

Results: In the study period, 283 children with CDH were born, resulting in a national prevalence rate of 2.06/10 000 live births. The patient population, treatment and outcomes at 1 year were comparable between the hospitals, except for length of hospital stay. Regression analysis identified the treating hospital as the strongest significant predictor thereof. Other factors associated with longer length of stay include the presence of other malformations, intrathoracic liver position on prenatal ultrasound, extracorporeal membrane oxygenation treatment, patch repair, complicated postoperative course and discharge to home rather than to another care facility.

Conclusion: Outcomes of CDH care throughout the Netherlands are comparable. However, the length of stay differed between the two hospitals, also when adjusting for other covariates. Further qualitative analysis to explain this interhospital variation is indicated. Our findings underscore the potential of clinical auditing as a quality measurement tool in rare conditions.

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荷兰先天性膈疝的发病率和早期手术效果:欧洲儿科手术审计的一项基于人群的队列研究。
背景:先天性膈疝(CDH先天性膈疝(CDH)是一种罕见的出生缺陷,死亡率很高。本研究旨在对荷兰 CDH 的治疗和结果进行基于人群的概述。此外,它还评估了荷兰两个 CDH 专家中心之间的医院间差异:本研究使用了欧洲儿科外科审计荷兰分部的数据,这是一项针对先天性畸形的前瞻性临床审计。2014年至2021年间接受治疗的所有CDH患者的数据都被纳入流行病学分析。在进行比较分析时,排除了新生儿期后就诊或未在两个 CDH 专家中心接受治疗的患者。采用回归分析法对已确定的医院间差异进行评估:在研究期间,共有283名CDH患儿出生,全国患病率为2.06/10,000。除住院时间外,各家医院的患者人数、治疗方法和1年后的治疗效果均相当。回归分析表明,治疗医院是最重要的预测因素。与住院时间延长相关的其他因素包括:存在其他畸形、产前超声检查显示肝脏位于胸腔内、体外膜氧合治疗、补片修复、术后过程复杂以及出院回家而非转至其他医疗机构:结论:荷兰各地的 CDH 护理结果具有可比性。结论:荷兰各地的 CDH 治疗结果具有可比性,但两家医院的住院时间存在差异,调整其他协变量后也是如此。需要进一步进行定性分析,以解释医院间的这种差异。我们的研究结果凸显了临床审计作为罕见病症质量测量工具的潜力。
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来源期刊
CiteScore
9.00
自引率
4.50%
发文量
90
审稿时长
6-12 weeks
期刊介绍: Archives of Disease in Childhood is an international peer review journal that aims to keep paediatricians and others up to date with advances in the diagnosis and treatment of childhood diseases as well as advocacy issues such as child protection. It focuses on all aspects of child health and disease from the perinatal period (in the Fetal and Neonatal edition) through to adolescence. ADC includes original research reports, commentaries, reviews of clinical and policy issues, and evidence reports. Areas covered include: community child health, public health, epidemiology, acute paediatrics, advocacy, and ethics.
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