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Images in clinical medicine. Vanishing lung syndrome.

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A 41-year-old man with a smoking history of 30 pack-years presented with chronic chest pain, dyspnea, and cough. His vital signs were normal, and laboratory studies were unremarkable, including his level of α1-antitrypsin, which was normal. Physical examination revealed cachexia, with decreased apical breath sounds and hyperresonance to percussion in both lungs. Chest radiography showed extensive bullous lung disease in the apex and upper lobes of both lungs, which was suggestive of vanishing lung syndrome; air–liquid levels were seen in the left lung. Computed tomography of the chest confirmed these findings.

Vanishing lung syndrome, otherwise known as idiopathic giant bullous emphysema, typically occurs in young, thin male smokers. The radiographic criteria for vanishing lung syndrome were proposed in 1987, and they include giant bullae in one or both upper lobes occupying at least one third of the hemithorax and compressing surrounding parenchyma. Air–liquid levels within bullae are uncommon and raise the question of bacterial superinfection. Lung-volume–reduction surgery is considered for selected patients with vanishing lung syndrome after assessment of exercise capacity, pulmonary-function testing, and smoking cessation.

This patient underwent successful lung-volume–reduction surgery and is currently without residual symptoms.

N Engl J Med. 2014 Feb 27;370(9):e14
Authors: Ladizinski B, Sankey C
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