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Congenital Pulmonary Airway Malformation

Imaging findings

Chest CT in an 18-year-old male with a non-resolving spontaneous pneumothorax demonstrates a large multi-cystic mass (CPAM) involving the posterior segment of the right upper lobe and extending into the superior segment of the right lower lobe, associated with bronchial atresia and an aberrant subclavian artery.

Key takeaways

Congenital pulmonary airway malformation (CPAM) is a congenital cystic lung lesion that can occasionally present in adolescents or young adults with a spontaneous pneumothorax or recurrent infection. It is frequently associated with other congenital anomalies, such as bronchial atresia. Surgical management (segmentectomy or lobectomy) is required to resolve the pneumothorax and prevent infection.

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