Center for Lymphatic Imaging, Penn Medicine, United States; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States.
Department of Pediatrics, Division of Pulmonology and Sleep Medicine, Nemours/duPont Hospital for Children, Wilmington, DE, United States; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States.
Paediatr Respir Rev. 2020 Nov;36:15-24. doi: 10.1016/j.prrv.2020.07.001. Epub 2020 Jul 10.
Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.
肺淋巴管疾病的特征是胸腔内存在异常的淋巴组织，临床上表现为乳糜胸、乳糜心包、乳糜咯痰、间质性肺病和塑型性支气管炎。这些疾病包括：新生儿乳糜胸、心内型和心外型塑型性支气管炎、非创伤性乳糜胸、先天性心脏病手术后乳糜胸和复杂性淋巴管畸形。最近发展的淋巴成像技术，如淋巴结内淋巴管造影和动态对比增强磁共振淋巴造影，显示了胸导管内的异常肺淋巴流进入肺实质，这是这些疾病的病理生理机制。新型微创淋巴管介入治疗，如胸导管栓塞、间质淋巴管栓塞和手术淋巴静脉吻合术，为这些疾病提供了有效的治疗方法。
