Swyer-James-MacLeod syndrome in a surgically treated child: a case report and brief literature review

Swyer-James-Macleod syndrome (SJMS) is a rare, complex disease characterized by unilateral hyperlucent lung or lobe owing to loss of pulmonary vasculature and alveolar hyperdistention. Treatment is generally conservative, and surgical management is rare. In fact, only 4 reports on surgically treated children with SJMS are available in the literature. We describe an 8-year-old patient with a history of recurrent respiratory infections since 2 months old. The patient presented with cough and dyspnea on effort. Chest radiograph showed hyperlucency and reduction of the right lung. Computed tomography revealed reduced volume and vasculature in the right lung, whereas pulmonary scintigraphy showed that 85% of ventilation and perfusion occurred in the left lung. Pulmonary function tests showed a forced expiratory volume in 1 second of 0.85 L (56% of expected value) and a forced vital capacity of 1.20 L (70% of expected value). The child underwent right pneumonectomy because of severe compromise of pulmonary function and recurrent respiratory infections. Histologic examination revealed panacinar emphysema and chronic bronchitis/bronchiolitis. At 6-month follow-up, the child showed improved symptoms and a forced expiratory volume in 1 second of 1.15 L (77% of expected value) and a forced vital capacity of 1.4 L (83% of expected value). This report shows that although rarely indicated, surgical treatment can improve quality of life and pulmonary function in SJMS.