Congenital Pulmonary Airway Malformation Presenting as Pneumothorax: Two Case Reports

Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lower respiratory tract, with an estimated incidence ranging from 1 in 10,000 to 1 in 35,000 live births. CPAM may present during infancy with severe respiratory distress and complications such as pneumothorax or recurrent pulmonary infection.

We report two female infants aged 7 months and 2 months who presented with severe respiratory distress and radiological findings initially suggestive of pneumothorax. In Case 1, chest computed tomography (CT) demonstrated a large solitary cyst consistent with Type I CPAM. The patient required prolonged pediatric intensive care admission complicated by ventilator-associated infection with Pseudomonas aeruginosa and Acinetobacter baumannii before successful right lower lobectomy and recovery. In Case 2, chest CT demonstrated multiple cystic hyperinflated lesions suggested Type III CPAM; however, alternative differentials of bronchial atresia and other congenital cystic lung lesions could not be excluded. Despite aggressive ventilatory and antimicrobial management, the infant deteriorated and died from progressive respiratory failure prior to surgical intervention.

These cases highlight the diagnostic complexity of congenital cystic lung lesions presenting with respiratory distress in resource-limited settings. Early advanced imaging, multidisciplinary evaluation, and timely surgical management remain critical for improving outcomes.