患有CDH的儿童患漏斗胸畸形和进行性胸部不对称的风险很高:CDH幸存者的胸壁畸形。

IF 2.4 2区 医学 Q1 PEDIATRICS Journal of pediatric surgery Pub Date : 2025-01-01 DOI:10.1016/j.jpedsurg.2024.162057
Carolin Riemer , Luisa Gräfin Lambsdorff , Nina Hutflesz , Christoph Mohr , Meike Weis , Christel Weiss , Richard Martel , Michaela Klinke Petrowsky , Katrin Zahn , Thomas Schaible , Michael Boettcher , Julia Elrod
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引用次数: 0

摘要

背景:先天性膈疝(CDH)幸存者发生肌肉骨骼异常的风险增加。本前瞻性长期队列研究探讨了大量CDH患者不同胸壁畸形的特征、预测因素和动态变化。方法:纳入2010年至2023年在曼海姆大学医院诊断为CDH并接受治疗的所有儿童。根据740次CT或MRI扫描评估胸部形态,并使用mann - whitney - u检验、Fisher精确检验和Spearman相关进行统计分析。p值结果:在1216例CDH患儿中,548例在随访期间接受了胸部横断面成像检查。平均Haller指数为2.62±0.55,34.09%的CDH患者存在漏斗胸畸形。45.90%的胸腔不对称倾向于右半胸,并表现出明显的年龄相关进展。胸壁畸形严重程度与产前诊断、肝疝、低肺头比和胎儿相对肺体积、ECMO和吸入一氧化氮、膈补片闭合和剖腹手术有显著相关。结论:CDH患儿胸壁畸形发生率增加,包括胸侧畸形和不对称,并有可能出现明显的年龄相关进展。使用基于MRI或CT扫描的标准化参数测量胸部形态应作为标准化随访计划的重要组成部分。需要严格的随机对照干预试验来评估某些疗法是否能改变这些胸壁畸形的发展轨迹。
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Children With CDH are at High Risk for Pectus Excavatum Deformity and Progressive Thoracic Asymmetry

Background

Congenital diaphragmatic hernia (CDH) survivors exhibit an increased risk for developing musculoskeletal anomalies. This prospective long-term cohort study investigates the characteristics, predictors and dynamic changes of different chest wall deformities in a large cohort of CDH patients.

Methods

All children diagnosed with CDH and treated at the University Hospital Mannheim from 2010 to 2023 were included. Thoracic morphology was evaluated based on 740 CT or MRI scans and statistical analysis was performed using the Mann-Whitney-U-Test, Fisher's Exact Test and Spearman's correlation. P-values<0.05 were considered statistically significant.

Results

Out of 1216 children with CDH, 548 received cross-sectional imaging examinations of the chest within the follow-up program. Mean Haller Index was 2.62 ± 0.55 and pectus excavatum deformity was present in 34.09 % of CDH patients. Thoracic asymmetry in favor of the right hemithorax was present in 45.90 % and showed a pronounced age-related progression. The severity of chest wall deformities showed a significant correlation with prenatal diagnosis, liver herniation, lower lung to head ratio and fetal relative lung volume, use of ECMO and inhaled nitric oxide, diaphragmatic patch closure and laparotomy.

Conclusion

Children with CDH show an increased incidence of chest wall deformities, including pectus deformity and asymmetry with the potential for significant age-related progression. Measurement of thoracic morphology using standardized parameters based on MRI or CT scans should be implemented as an essential component of standardized follow-up programs. Rigorous randomized, controlled intervention trials are required to evaluate whether certain therapies can alter the trajectory of these chest wall deformities.
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来源期刊
CiteScore
1.10
自引率
12.50%
发文量
569
审稿时长
38 days
期刊介绍: The journal presents original contributions as well as a complete international abstracts section and other special departments to provide the most current source of information and references in pediatric surgery. The journal is based on the need to improve the surgical care of infants and children, not only through advances in physiology, pathology and surgical techniques, but also by attention to the unique emotional and physical needs of the young patient.
期刊最新文献
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