Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment. (November 2020)

• Record Type: Journal Article
• Authors: Itkin, Maxim; Chidekel, Aaron; Ryan, Kelly A.; Rabinowitz, Deborah
• Is Part Of: Paediatric respiratory reviews. Volume 36(2020)
• Page Start: 15
• Page End: 24
• Publication Date: 2020-11
• Subjects: PLD pulmonary lymphatic disorders -- PLF pulmonary lymphatic flow -- PB plastic bronchitis -- CLFD Central Lymphatic Flow Disorder -- LM lymphatic malformations -- TD thoracic duct -- MR magnetic resonance -- US ultrasound -- DCMRL dynamic contrast enhanced magnetic resonance lymphangiograms -- IL intranodal lymphangiography -- N-BCA N-butyl cyanoacrylate -- GSD Gorham Stout Disease -- KLA Kaposiform Lymphangiomatosis -- GLA Generalized Lymphatic Anomaly -- DIC disseminated intravascular coagulopathy -- NPO nil per os [mouth] -- MTOR mammalian target of rapamycin -- VEGF vascular endothelial growth factor; Lymphatic malformations -- Plastic bronchitis -- Embolization -- Lymphangiography; Pediatric respiratory diseases -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory Therapy -- Periodicals; Child -- Periodicals; Appareil respiratoire -- Infections, chez l'enfant -- Périodiques; Appareil respiratoire -- Maladies, chez l'enfant -- Périodiques; 618.922
• DOI: 10.1016/j.prrv.2020.07.001
• Languages: English
• ISSNs: 1526-0542

Abstract

Abstract: 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.