Congenital > Diffuse Pulmonary Lymphangiomatosis

Diffuse Pulmonary Lymphangiomatosis

- Clinical:

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disorder characterized by the proliferation of lymphatic vessels [1]. It differs from pulmonary lymphangiectasis in that the lymphatics are not only dilated, but also increased in number and extensively interconnected [2]. The disease usually occurs in children and young adults with an equal sex prevalence [1]. The onset of the disease is usually insidious and the symptoms are nonspecific including dyspnea, cough, chest pain, milky sputum, shortness of breath, and wheezing [1]. It tends to be more aggressive in patients who present at a younger age, usually with progressive dyspnea leading to death from respiratory failure [2].

- X-ray:

Radiographic findings include diffuse mediastinal soft-tissue thickening without vessel malformation, diffuse bilateral thickening of the interlobular septa particularly in the upper lobes due to proliferation and dilatation of lymphatic vessels, and pleural thickening [1]. Patchy areas of ground-glass attenuation can reflect edema, hemorrhage, or the accumulation of hemosiderin-laden macrophages [2]. Chylous pleural and pericardial effusions are common [1].


(1) AJR 2017; Sun X, et al. Diffuse pulmonary lymphangiomatosis: MDCT findings after direct lymphangiography. 208: 300-305

(2) AJR 2009; Raman SP, et al. Imaging of thoracic lymphatic diseases. 193: 1504-1513

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