Pneumonectomy in a Patient with Swyer-James-MacLeod Syndrome

Swyer-James-MacLeod syndrome is a rare disease that results in parenchymal damage believed to be caused by recurrent lung infections in childhood. We report a case of a previously healthy young woman that presented with insidious progression of exertional dyspnea with restrictive lung physiology. Axial imaging demonstrated a hyperinflated emphysematous right lung with lower lobe fibrosis, nodules and air-fluid filled cysts, with a small right pulmonary artery and contralateral shifting of the mediastinum. She underwent right pneumonectomy ultimately resulting in improvement of her symptoms, with surgical pathology indicating extensive emphysema, bronchiectasis, fibrosis with osseous metaplasia, and placental transmogrification of alveolar septa, being consistent with a diagnosis of SJMS.