CTICasesDiffuse Pulmonary Lymphangiomatosis
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Imaging findings
Early lymphangiogram and CT demonstrate contrast in the abdominal lymphatics and supraclavicular nodes, accompanied by large bilateral pleural effusions. Follow-up CT obtained four years later shows severe low-attenuation thickening of the pleura, chest wall, and mediastinal tissues, a pericardial effusion, and diffuse pulmonary lymphatic infiltration characterized by thickened interlobular septa, peribronchovascular bundle thickening, and subpleural lines.
Key takeaways
Diffuse pulmonary lymphangiomatosis is a rare, progressive disease characterized by the abnormal proliferation of lymphatic vessels infiltrating the lungs, mediastinum, pleura, and chest wall. The imaging findings mimic other diffuse lymphatic patterns such as sarcoidosis, Kaposi sarcoma, and lymphangitic carcinomatosis. The clinical course is progressive and can be exacerbated by physiological stressors such as pregnancy.
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