Diffuse Pulmonary Lymphangiomatosis
Melissa L. Rosado-de-Christenson, MD, FACR
Key Facts
Terminology
Congenital disorder characterized by proliferation and dilatation of lymphatic channels
Imaging Findings
Radiography: Reticular opacities and pleural effusions
CT and HRCT
Smooth interlobular septal thickening
Thick peribronchovascular interstitium
Soft tissue infiltration of mediastinal fat
Top Differential Diagnoses
Pulmonary Lymphangiectasis
Pulmonary Edema
Lymphangitic Carcinomatosis
Erdheim-Chester Disease
Clinical Issues
Presentation: Infants, children, young adults
Dyspnea, wheezing, hemoptysis, chyloptysis
Progressive disease, often fatal
 Axial NECT of a patient with diffuse pulmonary lymphangiomatosis and chronic dyspnea shows smooth  interlobular septal thickening and thickening of the peribronchovascular interstitium  . |
 Axial NECT of a young patient with diffuse pulmonary lymphangiomatosis shows mediastinal lymphadenopathy  and mild induration and soft tissue infiltration of the anterior mediastinal fat  . |
TERMINOLOGY
Abbreviations and Synonyms
Diffuse pulmonary lymphangiomatosis (DPL)
Synonym: Diffuse pulmonary angiomatosis; uncertain nature of vascular abnormalities
Definitions
Congenital disorder characterized by dilatation and proliferation of pulmonary lymphaticsRelated posts:
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