Bilateral Apical Congenital Pulmonary Airway Malformation Presenting With Spontaneous Pneumothorax in an Adolescent:...

Congenital pulmonary airway malformation (CPAM), previously termed congenital cystic adenomatoid malformation (CCAM), is a rare developmental anomaly of the lower respiratory tract that is most commonly diagnosed prenatally or during early infancy. Presentation in adolescence is uncommon, particularly when associated with bilateral lesions and pneumothorax. We report the case of a 14-year-old female with no relevant past medical history who presented with sudden-onset progressive dyspnea and was found to have an approximately 50% right-sided pneumothorax. Contrast-enhanced chest computed tomography demonstrated bilateral apical cystic lesions initially interpreted as pneumatoceles, measuring 25 × 10 mm in the right lung apex and 10 × 18 mm in the left lung apex. After initial pleural drainage, the patient underwent right apical wedge resection, followed one week later by left-sided wedge resection. Histopathological examination of both specimens revealed pulmonary tissue with congested vessels, focal alveolar collapse, and multiple cystic spaces of variable size with thin fibrous walls and focal simple cuboidal epithelial lining. CD31 immunostaining was negative in the cyst lining, and Ziehl-Neelsen staining was negative. The overall findings were consistent with Stocker type 2 CPAM. This case highlights an unusual adolescent presentation of bilateral apical CPAM complicated by pneumothorax and underscores the importance of histopathologic confirmation when imaging findings are atypical or inconclusive.