Imaging description

Diffuse pulmonary lymphangiomatosis (DPL) is a disease of uncertain etiology that results in an increase in number and dilatation of the lymphatic channels. CT findings consist of smooth thickening of the interlobular septa and bronchovascular interstitium bilaterally (Figure 73.1A), [1]. Chylous pleural effusions and pleural thickening are common (Figure 73.1A) [1]. Mediastinal adenopathy and increased attenuation of mediastinal fat may also be present (Figure 73.1B), [1].

Importance

DPL accounts for approximately 5% of all chronic interstitial lung disease, presents in the pediatric to young adult population, and causes a restrictive and/or obstructive lung disease [2, 3].