Congenital Lung Malformations

Congenital lung malformations (CLM) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The prenatal growth pattern of CLMs is unpredictable with larger lesions causing life-threatening complications, such as hydrops fetalis, and smaller lesions remaining asymptomatic and potentially regressing. The most common CLMs are congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. All these lesions have clinically similar presentations when symptomatic, but pathophysiologic differences that must be considered when evaluating and treating. This article reviews the most common CLMs, their pathophysiology, clinical presentation, diagnostic considerations, and current literature on the controversies surrounding CLM management. [Pediatr Ann. 2019;48(4):e169-e174.].