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Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment
Maxim Itkin1, Aaron Chidekel2, Kelly A Ryan3
1Center for Lymphatic Imaging, Penn Medicine, United States; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States.
Insights
Pulmonary lymphatic disorders involve abnormal thoracic lymphatic tissues, leading to conditions like chylothorax. Advanced imaging and minimally invasive interventions are improving diagnosis and treatment for these complex lymphatic diseases.
Area of Science:
- Pulmonology
- Vascular Medicine
- Radiology
Background:
- Pulmonary lymphatic disorders manifest as abnormal lymphatic tissue in the thorax.
- Clinical presentations include chylothorax, chylopericardium, chyloptysis, interstitial lung disease, and plastic bronchitis.
- Specific conditions encompass neonatal chylothorax, plastic bronchitis, and complex lymphatic malformations.
Purpose of the Study:
- To elucidate the pathophysiological mechanisms of pulmonary lymphatic disorders.
- To highlight the role of advanced lymphatic imaging techniques.
- To present novel minimally invasive treatment options.
Main Methods:
- Utilized intranodal lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography.
- Investigated abnormal pulmonary lymphatic flow from the thoracic duct into the pulmonary parenchyma.
- Reviewed minimally invasive lymphatic interventions.
Main Results:
- Demonstrated abnormal pulmonary lymphatic flow as a key pathophysiological mechanism.
- Identified specific lymphatic imaging techniques for diagnosis.
- Confirmed the efficacy of novel interventions.
Conclusions:
- Pulmonary lymphatic disorders are linked to abnormal lymphatic flow into the lungs.
- Advanced imaging techniques are crucial for understanding disease mechanisms.
- Minimally invasive lymphatic interventions offer effective treatment strategies.
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.

