CTICasesDiffuse Pulmonary Lymphangiomatosis
Imaging findings
High-resolution chest CT shows diffuse, bilateral interlobular septal thickening, peribronchovascular cuffing, and pleural effusions. Lymphangiography demonstrates dilated, tortuous retroperitoneal and mediastinal lymphatic channels with retrograde chylous reflux from the thoracic duct into the lung parenchymal lymphatics, which is treated with coils and glue embolization.
Key takeaways
Diffuse pulmonary lymphangiomatosis (DPL) is a rare, congenital lymphatic disorder characterized by the proliferation of abnormal lymphatic channels. It presents with chylothorax, chyloptysis, and interstitial disease. Contrast lymphangiography is diagnostic in demonstrating retrograde reflux of chyle from the thoracic duct into the lung parenchyma. Embolization or ligation of the thoracic duct is a therapeutic option to control symptoms.
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