先天性膈疝三十年的预后:来自英国外科中心的报告。

IF 2.1 4区 医学 Q1 PEDIATRICS
Acta Paediatrica Pub Date : 2025-09-02 DOI:10.1111/apa.70295
Wan Teng Lee, Paul D Losty
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引用次数: 0

摘要

目的:先天性膈疝(CDH)与肺发育不全、肺动脉高压和高死亡率相关。报告了英国某中心30年的经验。方法:对1990年2月至2021年11月在多学科诊所就诊的CDH新生儿的医疗记录进行检查。记录生存和健康结果。结果:220例CDH新生儿中,左侧畸形177例(80%)。91例(41%)患者需要隔膜贴片,42例(19%)患者额外需要腹壁假体。使用的材料包括Gore-Tex生物Surgisis贴片,均有早期复发。膈补片与眼底重叠显著相关(p 0.005)。总生存率为85%-(90%原发缺陷生存vs 79%补丁)(p 0.035)。比较几十年,2010年后的CDH研究队列是一个病情较重的患者组,包括更多的心脏畸形指数病例(69%对28%)。结论:尽管在30年的报告中,CDH的存活率为85%,但这家英国大学外科中心的CDH管理人员发现,混合严重表型的患者越来越复杂。未来的挑战仍有待解决,以提高最复杂的弱势患者的生存率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Outcomes for Congenital Diaphragmatic Hernia in Three Decades: A Report From a UK Surgical Centre.

Aims: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia, pulmonary hypertension and high mortality. Three decades experience from a UK centre is reported.

Methods: Medical records of CDH newborns between February 1990 and November 2021 and attending a multidisciplinary clinic were examined. Survival and health outcomes are recorded.

Results: Of 220 CDH newborns, left-sided defects were 177 (80%) cases. Diaphragm patch was required in 91 (41%) patients and 42 (19%) additionally required abdominal wall prosthesis. Materials deployed included Gore-Tex biological Surgisis patches all had early recurrences. Diaphragmatic patch was significantly associated with fundoplication (p 0.005). Overall survival rate was 85%-(90% survival primary defect vs. 79% patch) (p 0.035). Comparing decades, the post-2010 CDH study cohort was a sicker patient group comprising more index cases with cardiac malformations (69% vs. 28%, p < 0.001) requiring greater cardiovascular inotrope support (61% vs. 25%, p < 0.001) and ECMO (15% vs. 5%, p 0.023).

Conclusion: While a modestly excellent 85% survival rate is reported over three decades, CDH management at this UK university surgical centre witnessed a growing complexity of patients with mixed severity phenotype(s). Future challenges remain to be solved to improve survival for the most complex vulnerable patients.

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来源期刊
Acta Paediatrica
Acta Paediatrica 医学-小儿科
CiteScore
6.50
自引率
5.30%
发文量
384
审稿时长
2-4 weeks
期刊介绍: Acta Paediatrica is a peer-reviewed monthly journal at the forefront of international pediatric research. It covers both clinical and experimental research in all areas of pediatrics including: neonatal medicine developmental medicine adolescent medicine child health and environment psychosomatic pediatrics child health in developing countries
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