Seminars in Fetal & Neonatal Medicine最新文献

英文中文

Fetal therapies in congenital diaphragmatic hernia: a review of possibilities and practical challenges 胎儿治疗先天性膈疝：可能性和实际挑战的回顾。

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-08-22 DOI: 10.1016/j.siny.2025.101656

Adriana Dekirmendjian , Fabian Doktor , Lina Antounians , Augusto Zani

Congenital diaphragmatic hernia (CDH) is characterized by pulmonary hypoplasia. CDH lungs exhibit an inflammatory signature with impaired growth, maturation, and vascularization, which postnatally result in altered gas exchange and pulmonary hypertension. These pulmonary abnormalities are drivers of poor survival and long-term morbidity. Improving lung development before birth remains a key strategy to enhance outcomes, and several antenatal therapies have emerged to address this need. These include surgical procedures, pharmacologic agents, and regenerative approaches. Fetal Endoluminal Tracheal Occlusion has shown the most robust evidence, improving survival in selected CDH cases. However, concerns about its generalizability, complications, and its limited effect on overall lung development have underscored the need for complementary or alternative strategies. A wide range of pharmacologic agents, some of which are in clinical use while others remain experimental, has been studied in experimental CDH models, such as corticosteroids, anti-inflammatory drugs and vasoactive medications. In parallel, regenerative medicine strategies via the antenatal administration of stem cells or their derivatives (extracellular vesicles) offer promise through multi-targeted mechanisms that address several aspects of pulmonary hypoplasia. Encouraging preclinical results provide a promising basis for advancing these regenerative therapies toward clinical application, with ongoing investigations focused on establishing their safety, efficacy, and feasibility.

先天性膈疝（CDH）以肺发育不全为特征。CDH肺表现出炎症特征，包括生长、成熟和血管化受损，这在出生后导致气体交换改变和肺动脉高压。这些肺部异常是低生存率和长期发病率的驱动因素。在出生前改善肺部发育仍然是提高结果的关键策略，已经出现了几种产前治疗方法来满足这一需求。这些方法包括外科手术、药物制剂和再生方法。胎儿腔内气管闭塞已显示出最有力的证据，可提高某些CDH病例的生存率。然而，对其广泛性、并发症和对整体肺发育的有限影响的担忧强调了补充或替代策略的必要性。已经在实验性CDH模型中研究了各种各样的药物，其中一些已在临床使用，而另一些仍处于实验阶段，例如皮质类固醇、抗炎药和血管活性药物。与此同时，通过产前给药干细胞或其衍生物（细胞外囊泡）的再生医学策略通过多靶向机制解决肺发育不全的几个方面提供了希望。令人鼓舞的临床前结果为推动这些再生疗法走向临床应用提供了有希望的基础，目前正在进行的研究重点是确定其安全性、有效性和可行性。

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引用次数: 0

Transitioning to adulthood in congenital diaphragmatic hernia, how do we design the right services? 过渡到成年期的先天性膈疝，我们如何设计正确的服务？

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-07-10 DOI: 10.1016/j.siny.2025.101653

S. Kamphuis Lieke , M. Schnater Johannes , C.W. Spaander Manon , IJsselstijn Hanneke

For children who have undergone surgical and neonatal intensive care treatment for congenital diaphragmatic hernia (CDH), health care providers’ concerns have shifted from survival rates to the management of associated morbidities. In recent decades, an increasing number of children born with CDH have reached adulthood. Although long-term outcome data remain limited, available evidence suggests that lifelong, multidisciplinary care is necessary across multiple healthcare domains. Moreover, the natural course of many associated morbidities remains poorly understood. This review explores the outcomes of CDH survivors during childhood and young adulthood – periods in which complications often become more evident – and underscores the importance of lifelong care. It also addresses the challenges associated with transitional care programs for adolescents living with chronic health conditions. Finally, the review offers recommendations for designing effective services that support children born with CDH throughout their lifespan.

对于因先天性膈疝（CDH）而接受手术和新生儿重症监护治疗的儿童，卫生保健提供者的关注点已从生存率转移到相关发病率的管理。近几十年来，越来越多出生时患有CDH的儿童已经成年。尽管长期结果数据仍然有限，但现有证据表明，跨多个医疗保健领域的终身多学科护理是必要的。此外，许多相关疾病的自然过程仍然知之甚少。本综述探讨了儿童期和青年期CDH幸存者的预后，这一时期的并发症往往更为明显，并强调了终身护理的重要性。它还解决了与患有慢性疾病的青少年过渡护理方案相关的挑战。最后，本综述为设计有效的服务提供了建议，以支持先天性CDH患儿的整个生命周期。

引用次数: 0

Challenges and controversies in the surgical management of Congenital Diaphragmatic Hernia 先天性膈疝手术治疗的挑战与争议。

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-07-01 DOI: 10.1016/j.siny.2025.101648

Carmen Mesas Burgos , Erin E. Perrone , Katrin Zahn , Holly L. Hedrick

Congenital diaphragmatic hernia (CDH) is a rare yet serious congenital condition marked by a diaphragmatic defect, pulmonary hypoplasia, and often pulmonary hypertension. While surgical repair is essential for survival, many controversies remain regarding its timing, approach, and techniques.

Delayed repair after physiological stabilization is the current standard, though optimal timing varies. Earlier repair within 24–48 h may reduce hospital stay in some cases, while others benefit from extended stabilization. CDH patients on extracorporeal life support (ECLS) pose additional challenges, balancing bleeding risks against potential survival benefits when surgery is done on ECLS.

Open surgical repair remains the gold standard, especially for large defects, with laparotomy preferred over thoracotomy. Minimally invasive surgery (MIS), although associated with better cosmetic and recovery outcomes, has higher recurrence rates and a steep learning curve. MIS is generally suited for smaller defects, though recent experience shows promising outcomes even in complex cases with patch repairs.

Repair technique depends on defect size. Primary repair is preferred for small defects, while larger defects require synthetic or biological patches with emphasis on a tension-free repair for all defects. Synthetic patches offer strength but carry infection risks, whereas biological materials may lack durability. Muscle flap techniques offer an alternative, especially for recurrent or large defects.

Recurrence remains a major concern, especially with large defects and patch repairs. Open reoperation is standard for recurrences, with MIS reserved for select cases. Prevention strategies include tension-free repair, appropriate patch choice, and meticulous technique.

Emerging strategies, such as regenerative medicine, composite patches, and tissue engineering, show promise but are not yet widely available. Multicenter research, individualized care, and standardized reporting are essential to optimize outcomes and guide future innovations in CDH management.

先天性膈疝（CDH）是一种罕见但严重的先天性疾病，以膈缺损、肺发育不全和肺动脉高压为特征。虽然手术修复是生存所必需的，但关于手术的时机、方法和技术仍然存在许多争议。生理稳定后的延迟修复是目前的标准，尽管最佳时间各不相同。在某些情况下，24-48小时内的早期修复可以减少住院时间，而其他情况则受益于延长稳定时间。体外生命支持（ECLS）的CDH患者面临着额外的挑战，在体外生命支持下进行手术时，要平衡出血风险和潜在的生存益处。开放手术修复仍然是金标准，特别是对于大的缺陷，剖腹手术优于开胸手术。微创手术（MIS）虽然具有更好的美容效果和恢复效果，但其复发率较高，学习曲线陡峭。MIS通常适用于较小的缺陷，尽管最近的经验表明，即使在具有补丁修复的复杂情况下，也有很好的结果。修复技术取决于缺陷的大小。初级修复是小缺陷的首选，而较大的缺陷需要合成或生物补片，并强调对所有缺陷进行无张力修复。合成贴片具有强度，但有感染风险，而生物材料可能缺乏耐用性。肌肉瓣技术提供了另一种选择，特别是对于复发性或较大的缺陷。复发仍然是主要的问题，特别是对于大的缺陷和修补。开放性再手术是复发的标准，MIS保留用于选定的病例。预防策略包括无张力修复，适当的补片选择和细致的技术。新兴的策略，如再生医学、复合贴片和组织工程，显示出希望，但尚未广泛应用。多中心研究、个性化护理和标准化报告对于优化结果和指导未来CDH管理创新至关重要。

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引用次数: 0

In search of “optimal care”: where should congenital diaphragmatic hernia be managed? 寻找“最佳护理”：先天性膈疝应该在哪里治疗？

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-07-15 DOI: 10.1016/j.siny.2025.101655

Jessica C. Pollack , Tim Jancelewicz , Yigit S. Guner

Management of congenital diaphragmatic hernia (CDH) varies widely between institutions, and emerging data suggest that these center-specific practices influence patient outcomes. Given the resource-intensive nature of extracorporeal life support (ECLS) and its reliance on multidisciplinary expertise, regional and hospital level factors are likely critical in optimizing outcomes for this high-risk population. This review synthesizes the existing literature on institutional factors contributing to observed differences in outcomes across centers, explores the complexity of center-level variation in CDH management, and describes targeted research and quality improvement initiatives aimed at enhancing outcomes for neonates affected by this condition.

不同机构对先天性膈疝（CDH）的处理差异很大，新出现的数据表明，这些中心特定的做法会影响患者的预后。鉴于体外生命支持（ECLS）的资源密集型性质及其对多学科专业知识的依赖，区域和医院层面的因素可能对优化这一高危人群的结果至关重要。本综述综合了现有文献中关于导致不同中心预后差异的制度因素，探讨了中心水平CDH管理差异的复杂性，并描述了旨在提高受此疾病影响的新生儿预后的有针对性的研究和质量改进举措。

引用次数: 0

Optimizing ECLS strategies in the management of congenital diaphragmatic hernia: Recent advances and best practices 优化ECLS治疗先天性膈疝的策略：最新进展和最佳实践。

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-09-02 DOI: 10.1016/j.siny.2025.101659

Kylie I. Holden , Devin Saljuud , Kevin Johnson , Matthew T. Harting

Infants with congenital diaphragmatic hernia (CDH) face severe pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction, making it one of the most challenging neonatal conditions to manage. Advances in extracorporeal life support (ECLS) have the potential to significantly improve outcomes for the most severe infants with CDH. There has been significant evolution in indications, technology, and clinical strategies which have enhanced its role and improved early stabilization, management, and outcomes. While ECLS remains the foundational management strategy for the most challenging and highest-risk CDH patients, there remains no standard, evidence-based, or widely adopted approach, with variability across centers and ongoing equipoise regarding the optimal timing and criteria, highlighting the need for the development of evidence, further understanding, and refined approaches in CDH. In this review, we highlight recent advances and progressive approaches to ECLS in CDH.

先天性膈疝（CDH）婴儿面临严重的肺发育不全、肺动脉高压和心功能障碍，使其成为最具挑战性的新生儿疾病之一。体外生命支持（ECLS）的进步有可能显著改善最严重CDH婴儿的预后。在适应症、技术和临床策略方面发生了重大变化，增强了其作用，改善了早期稳定、管理和结果。虽然ECLS仍然是最具挑战性和最高风险的CDH患者的基本管理策略，但仍然没有标准的、基于证据的或广泛采用的方法，各中心之间存在差异，关于最佳时间和标准的持续平衡，突出了CDH中证据的发展、进一步理解和改进方法的必要性。在这篇综述中，我们重点介绍了CDH中ECLS的最新进展和进展方法。

引用次数: 0

Congenital Diaphragmatic Hernia: what are the priorities for the next 20 years? 先天性膈疝：未来20年的重点是什么？

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-08-26 DOI: 10.1016/j.siny.2025.101658

David McCulley , Florian Kipfmueller , Neil Patel

Advances in clinical care, biomedical research, and health advocacy have significantly improved survival and quality of life across the lifespan. To sustain and accelerate this progress, it is essential to define clear goals for the future. Congenital diaphragmatic hernia (CDH), one of the most common and severe congenital malformations, serves as a model condition where coordinated efforts in clinical care, research, and advocacy have led to important advances in understanding disease mechanisms and improving outcomes. Reflecting on this progress reveals several key themes, including the value of disease modelling, novel therapeutic strategies, and sustained innovation. Achieving a better future for people with CDH will require strategic prioritization of research, engagement of methodological expertise, addressing practical barriers, and promotion of collaboration. We propose that coordinated global efforts—including for example the creation of an international research platform, leveraging technological advances, and raising the visibility of CDH—will empower clinicians, researchers, and the CDH community to drive continued progress.

临床护理、生物医学研究和健康宣传的进步显著提高了整个生命周期的生存率和生活质量。为了维持和加速这一进展，必须为未来确定明确的目标。先天性膈疝（CDH）是最常见和最严重的先天性畸形之一，作为一种典型的疾病，在临床护理、研究和宣传方面的协调努力导致了对疾病机制的理解和改善结果的重要进展。反思这一进展揭示了几个关键主题，包括疾病建模的价值、新的治疗策略和持续创新。为CDH患者创造更美好的未来，需要在战略上确定研究的优先顺序，利用方法学专业知识，解决实际障碍，促进合作。我们建议协调全球努力，包括例如创建国际研究平台，利用技术进步，提高CDH的可见度，将使临床医生，研究人员和CDH社区能够推动持续的进步。

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引用次数: 0

Introduction to Seminars in Fetal and Neonatal Medicine, Congenital Diaphragmatic Hernia issue 胎儿和新生儿医学研讨会介绍，先天性膈疝问题。

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-09-13 DOI: 10.1016/j.siny.2025.101661

Neil Patel

引用次数: 0

What is the role of echocardiography in newborns with CDH? 超声心动图在新生儿CDH中的作用是什么？

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-08-22 DOI: 10.1016/j.siny.2025.101657

María V. Fraga , Gabriel Altit , Bradley A. Yoder , Shazia Bhombal

Congenital diaphragmatic hernia (CDH) is associated with high neonatal morbidity and mortality, often due to complex cardiovascular physiology and ventricular dysfunction. Echocardiography is a critical tool for assessing cardiac anatomy, ventricular performance, pulmonary hypertension, and shunt dynamics in both the prenatal and postnatal periods. Early echocardiographic evaluation can identify high-risk physiologic phenotypes and guide targeted interventions, yet optimal timing and frequency remain uncertain. This review outlines the role of fetal and neonatal echocardiography in CDH, highlights view-specific imaging strategies, and discusses key physiologic patterns. While early imaging may inform management, it must be interpreted in context to avoid unnecessary interventions. A targeted, phenotype-driven approach to echocardiography can support individualized care. Further research is needed to establish standardized protocols and determine the impact of echocardiographic timing on outcomes in this vulnerable population.

先天性膈疝（CDH）与新生儿高发病率和死亡率相关，通常是由于复杂的心血管生理和心室功能障碍。超声心动图是评估心脏解剖、心室功能、肺动脉高压和产前和产后分流动力学的关键工具。早期超声心动图评估可以识别高危生理表型并指导有针对性的干预，但最佳时机和频率仍不确定。这篇综述概述了胎儿和新生儿超声心动图在CDH中的作用，强调了特定视点的成像策略，并讨论了关键的生理模式。虽然早期成像可以为管理提供信息，但必须结合实际情况进行解释，以避免不必要的干预。一种有针对性的、表型驱动的超声心动图方法可以支持个体化护理。需要进一步的研究来建立标准化的方案，并确定超声心动图时间对这一弱势人群预后的影响。

引用次数: 0

Cardiorespiratory transition in CDH CDH的心肺过渡。

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-07-01 DOI: 10.1016/j.siny.2025.101649

Deepika Sankaran , Satyan Lakshminrusimha , Michelle J. Lim

Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly that occurs due to incomplete closure of the diaphragm followed by herniation of abdominal contents into the chest. Fetal hemodynamics are altered in CDH due to pulmonary alveolar and vascular hypoplasia, low pulmonary venous return and in some cases, hypoplasia or dysfunction of the left heart. CDH is associated with high risk of morbidity and mortality due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn secondary to pulmonary vascular hypoplasia and remodeling. Resuscitation in the delivery room involves optimizing cardiopulmonary transition from fetal to neonatal circulation through gentle ventilation, endotracheal intubation and gastric decompression. Infants with CDH often present with hypoxemic respiratory failure in the immediate postnatal period due to pulmonary hypertension. The understanding of critical cardiopulmonary interactions and the distinguishing features of CDH sub-phenotypes and degree of cardiac involvement may aid in an augmented precision-based approach to invasive ventilation, vasoactive use, and ECMO management. Improved survival is reported after fetal in-utero interventions such as fetoscopic endoluminal tracheal occlusion (FETO) with CDH and severe secondary pulmonary hypoplasia. Clinical outcomes can potentially be improved by optimizing cardiopulmonary transition in the delivery room and cardiopulmonary interactions in the immediate the postnatal period.

先天性膈疝（CDH）是一种罕见的先天性异常，是由于膈肌不完全闭合导致腹部内容物疝入胸部而发生的。胎儿血流动力学改变是由于肺泡和血管发育不全，肺静脉回流低，在某些情况下，左心发育不全或功能障碍。CDH与新生儿继发于肺血管发育不全和重塑的肺发育不全和持续性肺动脉高压导致的高发病率和死亡率相关。产房复苏包括通过温和通气、气管插管和胃减压优化从胎儿到新生儿循环的心肺过渡。新生儿CDH常表现为低氧性呼吸衰竭在直接产后期由于肺动脉高压。了解关键的心肺相互作用和CDH亚表型和心脏受损伤程度的显著特征可能有助于增强基于精确的有创通气、血管活动性使用和ECMO管理方法。据报道，胎儿宫内干预（如胎镜下腔内气管闭塞（FETO）合并CDH和严重继发性肺发育不全）后，生存率有所提高。通过优化产房的心肺过渡和产后心肺相互作用，可以潜在地改善临床结果。

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Nutrition in congenital diaphragmatic hernia: How and when? 先天性膈疝的营养：如何及何时？

IF 2.8 3区医学 Q1 PEDIATRICS

Seminars in Fetal & Neonatal Medicine

Pub Date : 2025-10-01 Epub Date: 2025-07-01 DOI: 10.1016/j.siny.2025.101650

Ulla Lei Larsen , John T. Wren Jr. , Christy Gilcrease , Mark Bremholm Ellebaek , Patrick E. Sloan

Survival for infants with congenital diaphragmatic hernia (CDH) has improved, but they continue to face significant nutritional morbidity. They are at risk of growth failure, malnutrition, gastroesophageal reflux disease (GERD), oral aversion, and the need for nutritional tube feeding support after the initial hospitalization. Malnutrition and poor nutritional outcomes impact neurodevelopment, underlining the importance of addressing these issues. Available evidence in this area is scarce, and controversies remain about the timing and mode of enteral feeding in the peri-operative period, the role of enteral nutrition on extracorporeal membrane oxygenation (ECMO), and nutritional growth targets for infants with CDH.

In this manuscript, we discuss enteral and parenteral targets for infants with CDH, growth targets, the timing and advancement of enteral feeding. In addition, we discuss post-discharge nutritional support, identify gaps in the current literature and address parental concerns around nutrition and growth in infants with CDH.

婴儿先天性膈疝（CDH）的生存率有所提高，但他们仍然面临着显著的营养发病率。他们有生长衰竭、营养不良、胃食管反流病（GERD）、口腔厌恶和初次住院后需要营养管饲支持的风险。营养不良和营养不良影响神经发育，强调了解决这些问题的重要性。这方面的证据很少，围手术期肠内喂养的时机和方式，肠内营养对体外膜氧合（ECMO）的作用，以及CDH婴儿的营养生长目标仍然存在争议。在这篇文章中，我们讨论了婴儿CDH的肠内和肠外目标，生长目标，肠内喂养的时间和进度。此外，我们还讨论了出院后的营养支持，确定了当前文献中的空白，并解决了父母对CDH婴儿营养和生长的担忧。

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