Seminars in Pediatric Surgery最新文献

{"title":"Multidisciplinary approach to patients with lymphatic conduction disorders","authors":"Erin Pinto,&nbsp;Christopher Smith,&nbsp;Aaron DeWitt,&nbsp;Pablo Laje,&nbsp;Yoav Dori","doi":"10.1016/j.sempedsurg.2024.151416","DOIUrl":"10.1016/j.sempedsurg.2024.151416","url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151416"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141138305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comprehensive nutrition guidelines and management strategies for enteropathy in children","authors":"Melanie L. Savoca ,&nbsp;Jefferson N. Brownell","doi":"10.1016/j.sempedsurg.2024.151425","DOIUrl":"10.1016/j.sempedsurg.2024.151425","url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151425"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141145674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Percutaneous procedures for central lymphatic conduction disorders","authors":"Abhay Srinivasan ,&nbsp;Christopher L. Smith ,&nbsp;Yoav Dori ,&nbsp;Ganesh Krishnamurthy","doi":"10.1016/j.sempedsurg.2024.151418","DOIUrl":"10.1016/j.sempedsurg.2024.151418","url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151418"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141131116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Preliminary report of a thoracic duct-to-pulmonary vein lymphovenous anastomosis in swine: A novel technique and potential treatment for lymphatic failure","authors":"Benjamin Smood ,&nbsp;Terakawa Katsunari ,&nbsp;Christopher Smith ,&nbsp;Yoav Dori ,&nbsp;Constantine D. Mavroudis ,&nbsp;Sarah Morton ,&nbsp;Anthony Davis ,&nbsp;Jonathan M. Chen ,&nbsp;J. William Gaynor ,&nbsp;Todd Kilbaugh ,&nbsp;Katsuhide Maeda","doi":"10.1016/j.sempedsurg.2024.151427","DOIUrl":"10.1016/j.sempedsurg.2024.151427","url":null,"abstract":"<div><h3>Objective</h3><p>The thoracic duct is the largest lymphatic vessel in the body, and carries fluid and nutrients absorbed in abdominal organs to the central venous circulation. Thoracic duct obstruction can cause significant failure of the lymphatic circulation (i.e., protein-losing enteropathy, plastic bronchitis, etc.). Surgical anastomosis between the thoracic duct and central venous circulation has been used to treat thoracic duct obstruction but cannot provide lymphatic decompression in patients with superior vena cava obstruction or chronically elevated central venous pressures (e.g., right heart failure, single ventricle physiology, etc.). Therefore, this preclinical feasibility study sought to develop a novel and optimal surgical technique for creating a thoracic duct-to-pulmonary vein lymphovenous anastomosis (LVA) in swine that could remain patent and preserve unidirectional lymphatic fluid flow into the systemic venous circulation to provide therapeutic decompression of the lymphatic circulation even at high central venous pressures.</p></div><div><h3>Methods</h3><p>A thoracic duct-to-pulmonary vein LVA was attempted in 10 piglets (median age 80 [IQR 80–83] days; weight 22.5 [IQR 21.4–26.8] kg). After a right thoracotomy, the thoracic duct was mobilized, transected, and anastomosed to the right inferior pulmonary vein. Animals were systemically anticoagulated on post-operative day 1. Lymphangiography was used to evaluate LVA patency up to post-operative day 7.</p></div><div><h3>Results</h3><p>A thoracic duct-to-pulmonary vein LVA was successfully completed in 8/10 (80.0%) piglets, of which 6/8 (75.0%) survived to the intended study endpoint without any complication (median 6 [IQR 4–7] days). Initially, 2/10 (20.0%) LVAs were aborted intraoperatively, and 2/10 (20.0%) animals were euthanized early due to post-operative complications. However, using an optimized surgical technique, the success rate for creating a thoracic duct-to-pulmonary vein LVA in six animals was 100%, all of which survived to their intended study endpoint without any complications (median 6 [IQR 4–7] days). LVAs remained patent for up to seven days.</p></div><div><h3>Conclusion</h3><p>A thoracic duct-to-pulmonary vein LVA can be completed safely and remain patent for at least one week with systemic anticoagulation, which provides an important proof-of-concept that this novel intervention could effectively offload the lymphatic circulation in patients with lymphatic failure and elevated central venous pressures.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151427"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141137244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"fm i -- Contents","authors":"","doi":"10.1053/S1055-8586(24)00052-0","DOIUrl":"https://doi.org/10.1053/S1055-8586(24)00052-0","url":null,"abstract":"","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151431"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141308365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neonatal perspective on central lymphatic disorders","authors":"Brian M. Dulmovits,&nbsp;Dalal K. Taha","doi":"10.1016/j.sempedsurg.2024.151424","DOIUrl":"10.1016/j.sempedsurg.2024.151424","url":null,"abstract":"<div><p>Lymphatic disorders presenting in the first year of life are difficult to identify and manage given the broad range of underlying etiologies. Neonatal lymphatic disease arising from congenital or acquired conditions results in the abnormal accumulation of lymph fluid in the pleura (chylothorax), peritoneum (chylous ascites) and skin (edema/anasarca). There is also increasing recognition of lymphatic losses through the intestine resulting in protein-losing enteropathy (PLE). While the incidence of lymphatic disorders in neonates is unclear, advances in genetic testing and lymphatic imaging are improving our understanding of the underlying pathophysiology. Despite these advancements, medical management of neonatal lymphatic disorders remains challenging and variable among clinicians.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151424"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141143968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lymphatic failure and lymphatic interventions: Knowledge gaps and future directions for a new frontier in congenital heart disease","authors":"Benjamin Smood ,&nbsp;Christopher Smith ,&nbsp;Yoav Dori ,&nbsp;Constantine D. Mavroudis ,&nbsp;Stephanie Fuller ,&nbsp;J. William Gaynor ,&nbsp;Katsuhide Maeda","doi":"10.1016/j.sempedsurg.2024.151426","DOIUrl":"10.1016/j.sempedsurg.2024.151426","url":null,"abstract":"<div><p>Lymphatic failure is a broad term that describes the lymphatic circulation's inability to adequately transport fluid and solutes out of the interstitium and into the systemic venous circulation, which can result in dysfunction and dysregulation of immune responses, dietary fat absorption, and fluid balance maintenance. Several investigations have recently elucidated the nexus between lymphatic failure and congenital heart disease, and the associated morbidity and mortality is now well-recognized. However, the precise pathophysiology and pathogenesis of lymphatic failure remains poorly understood and relatively understudied, and there are no targeted therapeutics or interventions to reliably prevent its development and progression. Thus, there is growing enthusiasm towards the development and application of novel percutaneous and surgical lymphatic interventions. Moreover, there is consensus that further investigations are needed to delineate the underlying mechanisms of lymphatic failure, which could help identify novel therapeutic targets and develop innovative procedures to improve the overall quality of life and survival of these patients. With these considerations, this review aims to provide an overview of the lymphatic circulation and its vasculature as it relates to current understandings into the pathophysiology and pathogenesis of lymphatic failure in patients with congenital heart disease, while also summarizing strategies for evaluating and managing lymphatic complications, as well as specific areas of interest for future translational and clinical research efforts.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151426"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141143153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"State-of-the-art imaging for children with central lymphatic disorders","authors":"Karen I. Ramirez-Suarez ,&nbsp;Sean Schoeman ,&nbsp;Hansel J. Otero ,&nbsp;Christopher L. Smith ,&nbsp;David M. Biko","doi":"10.1016/j.sempedsurg.2024.151417","DOIUrl":"10.1016/j.sempedsurg.2024.151417","url":null,"abstract":"<div><p>Lymphatic imaging plays a crucial role in novel lymphatic interventions, offering valuable insights into central lymphatic drainage. Lymphatic system abnormalities may appear in various pediatric disorders, and accurate imaging is crucial for effective diagnosis and tailored therapeutic interventions. Traditional imaging modalities have offered valuable insights, but the demand for non-invasive, high-resolution techniques has fueled the development of innovative lymphatic imaging methods. In this review, we explore the state of the art in lymphatic imaging specifically within the context of pediatric surgery.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151417"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141143416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complex lymphatic anomalies: Molecular landscape and medical management","authors":"Alexandra J. Borst ,&nbsp;Allison Britt ,&nbsp;Denise M. Adams","doi":"10.1016/j.sempedsurg.2024.151422","DOIUrl":"10.1016/j.sempedsurg.2024.151422","url":null,"abstract":"<div><p>The lymphatic system is one of the most essential and complex systems in the human body. Disorders that affect the development or function of the lymphatic system can lead to multi-system complications and life-long morbidity. The past two decades have seen remarkable progress in our knowledge of the basic biology and function of the lymphatic system, the molecular regulators of lymphatic development, and description of disorders associated with disrupted lymphangiogensis. In this chapter we will touch on the clinical features of complex lymphatic anomalies, new molecular knowledge of the drivers of these disorders, and novel developmental therapeutics for lymphatic disease.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151422"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141132520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lymphatic interventions in congenital heart disease","authors":"Christopher L. Smith ,&nbsp;Ganesh Krishnamurthy ,&nbsp;Abhay Srinivasan ,&nbsp;Yoav Dori","doi":"10.1016/j.sempedsurg.2024.151419","DOIUrl":"10.1016/j.sempedsurg.2024.151419","url":null,"abstract":"<div><p>Congenital heart disease affects 1/100 live births and is one of the most common congenital abnormalities. The relationship between congenital heart disease and lymphatic abnormalities and/or dysfunction is well documented and can be grossly divided into syndromic and non-syndromic etiologies. In patients with genetic syndromes (as examples listed above), there are known primary abnormal lymphatic development leading to a large pleiotropic manifestation of lymphatic dysfunction. Non-syndromic patients, or those without clear genetic etiologies for their lymphatic dysfunction, are often thought to be secondary to physiologic abnormalities as sequelae of congenital heart disease and palliative surgeries. Patients with congenital heart disease and lymphatic dysfunction have a wide variety of clinical manifestations for which there were not many therapeutic interventions available. The development of new imaging techniques allows us to understand better the pathophysiology of these problems and to develop different percutaneous interventions aiming to restore normal lymphatic function.</p></div>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 3","pages":"Article 151419"},"PeriodicalIF":1.7,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141142469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}