Complex Thoracic Lymphatic Disorders of Adults

The pulmonary lymphatics play a key role in maintenance of a dry alveolar and pleural space, and in pulmonary immunity. They are also involved in the pathogenesis of a large number of lung disorders, including those associated with inflammation, infection, fibrosis, and edema. The thoracic lymphatic diseases are a heterogeneous group of uncommon congenital and acquired conditions that are associated with thoracic masses, interstitial pulmonary infiltrates, and chylous complications, including pleural and pericardial effusions and chyloptysis. New imaging techniques to characterize lymphatic flow, such as Dynamic Contrast-Enhanced MR Lymphangiography (DCRML), are refining our approaches to diagnosis and management, including mapping of the extent of disease, chylous leaks, and planning of embolization. Characterization of the genetic basis of several of these disorders has led to an improved understanding of their molecular pathogenesis, and in some cases, to the discovery of effective targeted molecular treatments. It is therefore increasingly important that pulmonary physicians who care for adults become familiar with the disease presentations and therapeutic options for lymphatic pulmonary disorders.