Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment

Maxim Itkin; Aaron Chidekel; Kelly A Ryan; Deborah Rabinowitz

doi:10.1016/j.prrv.2020.07.001

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment

Paediatr Respir Rev. 2020 Nov:36:15-24. doi: 10.1016/j.prrv.2020.07.001. Epub 2020 Jul 10.

Authors

Maxim Itkin¹, Aaron Chidekel², Kelly A Ryan³, Deborah Rabinowitz⁴

Affiliations

¹ Center for Lymphatic Imaging, Penn Medicine, United States; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States. Electronic address: itkinmax@gmail.com.
² 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.
³ Cardiac Center, Nemours/duPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, United States.
⁴ Dept of Medical Imaging, Division of Interventional Radiology, Nemours/duPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, United States.

PMID: 32792289
DOI: 10.1016/j.prrv.2020.07.001

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.

Keywords: Embolization; Lymphangiography; Lymphatic malformations; Plastic bronchitis.

Publication types

Review

MeSH terms

Bronchitis / diagnosis
Bronchitis / therapy
Chylothorax / diagnosis
Chylothorax / therapy
Disease Management
Heart Defects, Congenital / surgery
Humans
Lung Diseases / congenital
Lung Diseases / diagnosis*
Lung Diseases / therapy*
Lung Neoplasms / diagnosis
Lung Neoplasms / therapy
Lymphangiectasis / congenital
Lymphangiectasis / diagnosis
Lymphangiectasis / therapy
Lymphangioma / diagnosis
Lymphangioma / therapy
Lymphatic Abnormalities / diagnosis
Lymphatic Abnormalities / therapy
Lymphatic Diseases / diagnosis*
Lymphatic Diseases / therapy*
Lymphography
Magnetic Resonance Imaging
Mediastinal Neoplasms / diagnosis
Mediastinal Neoplasms / therapy
Noonan Syndrome / diagnosis
Noonan Syndrome / therapy
Osteolysis, Essential / diagnosis
Osteolysis, Essential / therapy
Pericardial Effusion / diagnosis
Pericardial Effusion / therapy
Postoperative Complications / diagnosis
Postoperative Complications / therapy

Supplementary concepts

Lymphangiectasia, pulmonary, congenital