Andy Wellsover 1 year ago

Lymphatic Plastic Bronchitis

A 36-year-old man with obesity presented to the pulmonary clinic with a 3-year history of shortness of breath, wheezing, and “coughing up lung.” He reported having daily expectoration of large, branching bronchial casts and brought a photograph of such a cast to the appointment. His oxygen saturation was 85% while he was breathing ambient air, and his respiratory rate was 16 breaths per minute. On auscultation, scattered wheezes were heard throughout all lung fields. He was admitted to the hospital to receive supplemental oxygen and undergo further evaluation. A computed tomographic scan of the chest showed diffuse ground-glass opacities. Histopathological analysis of a bronchoalveolar-lavage sample revealed neutrophil-predominant mucus plugs and lipid-laden macrophages without evidence of bacterial, fungal, or mycobacterial infection. Results of a diagnostic workup for allergic bronchopulmonary aspergillosis, cystic fibrosis, sickle cell disease, and asthma were unremarkable. The patient subsequently underwent dynamic contrast-enhanced magnetic resonance lymphangiography, which revealed an occlusion of the thoracic duct, a finding that suggested a diagnosis of lymphatic plastic bronchitis. Although plastic bronchitis typically manifests during childhood, the disorder can occur in adults as a result of aberrant pulmonary lymphatic flow and may lead to fatal asphyxiation if left untreated. The patient underwent percutaneous lymphatic embolization and had complete resolution of symptoms. Ishan Mehta, M.D. Emory University, Atlanta, GA source: nejm.org