Seminars in Pediatric Surgery最新文献

Congenital diaphragmatic hernia (CDH) is a life-threatening birth defect with significant morbidity and mortality. The prenatal management of a pregnancy with a fetus affected with CDH is complex and requires a multi-disciplinary team approach. An improved understanding of prenatal diagnosis and management is essential to developing strategies to optimize outcomes for these patients. In this review, we explore the current knowledge on diagnosis, severity stratification, prognostic prediction, and indications for fetal intervention in the fetus with CDH.

In the complex arena of Congenital Diaphragmatic Hernia (CDH) management, Extracorporeal Life Support (ECLS) provides a strategic window for stabilization and surgical correction, during which time marginal gains in patient stability can tip the scales towards survival. In modern neonatal ECLS, the focus is increasingly on minimizing survivor morbidity, which calls for considerable multidisciplinary expertise to enhance patient outcomes. This review will delve into the most up-to-date literature on the management of CDH in the context of ECLS, providing a comprehensive synthesis of current insights.

Congenital diaphragmatic hernia (CDH) is characterized by a developmental insult which compromises cardiopulmonary embryology and results in a diaphragmatic defect, allowing abdominal organs to herniate into the hemithorax. Among the significant pathophysiologic components of this condition is pulmonary hypertension (PH), alongside pulmonary hypoplasia and cardiac dysfunction. Fetal pulmonary vascular development coincides with lung development, with the pulmonary vasculature evolving alongside lung maturation. However, in CDH, this embryologic development is impaired which, in conjunction with external compression, stifle pulmonary vascular maturation, leading to reduced lung density, increased muscularization of the pulmonary vasculature, abnormal vascular responsiveness, and altered molecular signaling, all contributing to pulmonary arterial hypertension. Understanding CDH-associated PH (CDH-PH) is crucial for development of novel approaches and effective management due to its significant impact on morbidity and mortality. Antenatal and postnatal diagnostic methods aid in CDH risk stratification and, specifically, pulmonary hypertension, including fetal imaging and gas exchange assessments. Management strategies include lung protective ventilation, fluid optimization, pharmacotherapies including pulmonary vasodilators and hemodynamic support, and extracorporeal life support (ECLS) for refractory cases. Longitudinal re-evaluation is an important consideration due to the complexity and dynamic nature of CDH cardiopulmonary physiology. Emerging therapies such as fetal endoscopic tracheal occlusion and pharmacological interventions targeting key CDH pathophysiological mechanisms show promise but require further investigation. The complexity of CDH-PH underscores the importance of a multidisciplinary approach for optimal patient care and improved outcomes.

This review focuses on contemporary mechanical ventilator practices used in the initial management of neonates born with congenital diaphragmatic hernia (CDH). Both conventional and non-conventional ventilation modes in CDH are reviewed. Special emphasis is placed on the rationale for gentle ventilation and the current evidence-based clinical practice guidelines that are recommended for supporting these fragile infants. The interplay between CDH lung hypoplasia and other key cardiopulmonary elements of the disease, namely a reduced pulmonary vascular bed, abnormal pulmonary vascular remodeling, and left ventricular hypoplasia, are discussed. Finally, we provide insights into future avenues for mechanical ventilator research in CDH.

With improvements in initial care for patients with congenital diaphragmatic hernia (CDH), the number of CDH patients with severe disease who are surviving to discharge has increased. This growing population of patients faces a unique set of long-term challenges, multisystem adverse outcomes, and post-intervention complications requiring specialized multidisciplinary follow-up. Early identification and intervention are essential to mitigate the potential morbidity associated with these challenges. This manuscript outlines a general framework for long-term follow-up for the CDH patient, including cardiopulmonary, gastrointestinal, neurodevelopmental, surgical, and quality of life outcomes.

The aim of standardizing care is to enhance patient outcomes and optimize healthcare delivery by minimizing variations in care and ensuring the efficient allocation of healthcare resources. Despite these potential benefits to patients, healthcare providers and the healthcare system, standardization may also disadvantage these groups. With a specific focus on congenital diaphragmatic hernia, this article will review the promise and pitfalls of standardization, as well as a potential path forward that uses standardization to improve outcomes in this rare and complex disease process.

Congenital Diaphragmatic Hernia (CDH) is a complex developmental abnormality characterized by abnormal lung development, a diaphragmatic defect and cardiac dysfunction. Despite significant advances in management of CDH, mortality and morbidity continue to be driven by pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. The etiology of CDH remains unknown, but CDH is presumed to be caused by a combination of genetic susceptibility and external/environmental factors. Current research employs multi-omics technologies to investigate the molecular profile and pathways inherent to CDH. The aim is to discover the underlying pathogenesis, new biomarkers and ultimately novel therapeutic targets. Stem cells and their cargo, non-coding RNAs and agents targeting inflammation and vascular remodeling have produced promising results in preclinical studies using animal models of CDH. Shortcomings in current therapies combined with an improved understanding of the pathogenesis in CDH have given rise to novel promising experimental treatments that are currently being evaluated in clinical trials. This review provides insight into current developments in translational research, ranging from the cellular origins of abnormal cardiopulmonary development in CDH and the identification of novel treatment targets in preclinical CDH models at the bench and their translation to clinical trials at the bedside.

Patients with lymphatic disorders are remarkably complex and require a wide variety of medical and surgical services. Establishing a multidisciplinary program improves the efficiency of the patients’ hospital experience minimizing the compartmentalization of their care. Offering a clear intake process guarantees that patients will be seen promptly by all the required teams. Additionally, having regular multidisciplinary meetings allows all participating teams to learn from each other and gain experience in the care of a population that is extraordinarily heterogeneous. Additionally, establishing a solid program allows for long-term data collection, research and education.

Protein-losing enteropathy (PLE) describes a syndrome of excessive protein loss into the gastrointestinal tract, which may be due to a wide variety of etiologies. For children in whom the protein loss is associated with lymphangiectasia, medical nutrition therapy focused on restricting enteral long-chain triglycerides and thus intestinal chyle production is an integral component of treatment. This approach is based on the principle that reducing intestinal chyle production will concurrently decrease enteric protein losses of lymphatic origin. In patients with ongoing active PLE or those who are on a fat-restricted diet, particularly in infants and young children, supplemental calories may be provided with medium-chain triglycerides (MCT). MCT are absorbed directly into the bloodstream, bypassing intestinal lymphatics and not contributing to intestinal chyle production. Patients with active PLE or who are on dietary fat restriction should be monitored for associated micronutrient deficiencies. In this paper, we seek to formally present recommended nutrition interventions, principles of dietary education and patient counseling, and monitoring parameters in pediatric populations with PLE based on our experience in a busy clinical referral practice focused on this population.

Percutaneous endovascular techniques established in interventional cardiology and radiology are well-suited for managing lymphatic conduction disorders. In this article, we provide a synopsis of technical aspects of these procedures, including access of the thoracic duct, selective lymphatic embolization, and management of thoracic duct obstruction. In aggregate, these techniques have developed into an integral component of multidisciplinary management of these complex diseases.