Imaging Chylo- and Lympho-thorax in Children with Congenital Heart Defects

先天性心脏缺陷儿童的乳糜胸和淋巴胸成像

• 批准号: 9186459
• 负责人: Eva M. Sevick-Muraca
• 金额: $ 19.25万
• 依托单位: UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
• 依托单位国家: 美国
• 财政年份: 2015
• 资助国家: 美国
• 起止时间: 2015-12-01 至 2018-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9186459
• 关键词: Adult; Age-Months; Bilateral; Cardiac Surgery procedures; Chest; Chest Tubes; Child; Childhood; Chyle; Chylothorax; Clinical Research; Clinical Trials Data Monitoring Committees; Complication; Congenital Heart Defects; Conscious; Critical Illness; Critically ill children; Defect; Devices; Diagnostic; Diagnostic Imaging; Diagnostic Procedure; Diet Modification; Disease Management; Dose; Drainage procedure; Extravasation; Fluorescence; Hand; Hypoplastic Left Heart Syndrome; Image; Imagery; Imaging Device; Immunologics; Incidence; Individual; Indocyanine Green; Infant; Institutional Review Boards; Intensive Care; Investigation; Laceration; Lead; Length of Stay; Ligation; Light; Liquid substance; Lymph; Lymphatic; Metabolic; Monitor; Morbidity - disease rate; Near-infrared optical imaging; Noise; Nutritional; Obstruction; Operative Surgical Procedures; Pathogenesis; Pathway interactions; Patients; Pattern; Pediatric Intensive Care Units; Pediatric cardiology; Pilot Projects; Pleural cavity; Pleural effusion disorder; Pleurodesis; Population; Procedures; Resort; Rest; Rupture; Signal Transduction; Source; Surgical Management; System; Techniques; Thoracic Duct; Time; Time Management; Tissues; Translational Research; Venous; Venous system; base; contrast imaging; detector; foot; improved; inter-institutional; lymphatic drainage; male; minimally invasive; mortality; operation; point of care; pressure; public health relevance; response; success

 DESCRIPTION (provided by applicant): Chylothorax is a rare, but serious complication in children who undergo heart surgery, and is an important factor for the length of stay in pediatric intensive care units (PICU). Incidence of these pleural effusions following cardiac surgery in children is estimated to be 1.3 - 9.2% and has increased significantly in the last two decade. Chylo- and lympho-thorax can lead to serious metabolic, immunologic, and nutritional complications, and is associated with high morbidity and mortality rate. The pathogenesis is poorly understood but is thought to arise from (i) direct damage to the thoracic duct by rupture, laceration, tear, or compression, (ii) central venous (CV) obstruction or high pressure that impedes thoracic duct drainage into the venous system, or (iii) smaller lymphatic defects that may accompany congenital heart defects. Treatments for effective management need to be based upon known mechanism, but there is no diagnostic method to interrogate the causes of pleural effusion in any individual case. Currently, management for pleural effusions is initially conservative, consisting of gut rest as well as dietary modification over a period of 3-4 weeks. Although 75 to 85% of patients respond to conservative treatment, last resort surgical treatments such as pleurodesis and thoracic duct ligation have only limited success in treating pleural effusions, probably due to their lateness of treatment. Improving the poor response to these therapies demands a better understanding of the specific lymphatic drainage mechanisms responsible. Yet the inability to dynamically image lymphatic drainage in infants within the PICU has limited the understanding of pathogenesis of pleural effusions, which in turn has impeded the application of appropriate and timely management for these critically ill children. Near-infrared fluorescence (NIRF) imaging has been successfully used in investigational studies to assess the lymphatic vasculature and its function in adults and more recently in an infant with chylothorax. In this application, we propose to conduct a pilot study of dynamic lymphatic imaging of young children and infants who present with pleural effusion. Our specific aims of the study will be (i) to adapt our current investigational NIRF imaging device to enable ambient light rejection for improved bedside application in the PICU; (ii) to use the modified device to characterize dynamic lymphatic drainage in children with pleural effusion to assess drainage pathways; and (iii) to monitor response to therapy, whether conservative treatment, pleurodesis, or thoracic duct ligation in order to gain a better understanding of the management of chylothorax.

 描述（由申请人提供）：乳糜胸是接受心脏手术的儿童的一种罕见但严重的并发症，是儿科重症监护室（PICU）住院时间的重要因素。儿童心脏手术后胸腔积液的发生率估计为1.3 - 9.2%，并且在过去二十年中显著增加。乳糜胸和淋巴胸可导致严重的代谢、免疫和营养并发症，并与高发病率和死亡率相关。发病机制尚不清楚，但认为是由以下原因引起的：（i）胸导管破裂、撕裂、撕裂或压迫造成的直接损伤，（ii）中心静脉（CV）阻塞或高压阻碍胸导管引流至静脉系统，或（iii）可能伴随先天性心脏病的较小淋巴缺陷。有效的治疗需要基于已知的机制，但没有诊断方法来询问任何个别情况下胸腔积液的原因。目前，胸腔积液的管理最初是保守的，包括肠道休息以及3-4周的饮食调整。虽然75 - 85%的患者对保守治疗有反应，但最后的手术治疗（如胸膜固定术和胸导管结扎术）在治疗胸腔积液方面的成功率有限，这可能是由于其治疗时间较晚。改善对这些疗法的不良反应需要更好地了解负责的特定淋巴引流机制。然而，无法动态图像的淋巴引流在PICU内的婴儿限制了胸腔积液的发病机制的理解，这反过来又阻碍了适当和及时的管理，这些危重患儿的应用。近红外荧光（NIRF）成像已成功地用于调查研究，以评估淋巴管及其功能的成人和最近在婴儿乳糜胸。在本申请中，我们建议进行一项试点研究的动态淋巴显像的幼儿和婴儿谁目前与胸腔积液。本研究的具体目的是（i）调整我们目前的研究性NIRF成像设备，以实现环境光排斥，从而改善PICU的床边应用;（ii）使用改良设备表征胸腔积液儿童的动态淋巴引流，以评估引流途径;以及（iii）监测对治疗的反应，无论是保守治疗、胸膜固定术还是胸导管结扎术，以便更好地理解乳糜胸的处理。

项目成果

Multimodality lymphatic imaging of postoperative chylothorax in an infant with Noonan syndrome: a case report.

• DOI: 10.1186/s40001-020-00455-w
• 发表时间: 2020-11-04
• 期刊: European journal of medical research
• 影响因子: 4.2
• 作者: Pham KT;Balaguru D;Tammisetti VS;Guevara CJ;Rasmussen JC;Zvavanjanja RC;Hanfland R;Sevick-Muraca EM;Aldrich MB
• 通讯作者: Aldrich MB