Congenital malformations of the lung

Abstract

Congenital malformations of the lung comprise a diverse group of anomalies of the lung and airways. They are distinguished by anatomic and histologic defining characteristics. The etiology and pathogenetic mechanisms of these malformations remain undefined, but unifying theories have been proposed. Congenital pulmonary airway malformations are the most common congenital malformations of the lung. Other entities include bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most congenital malformations of the lung are detected prenatally and are asymptomatic at birth. Some present with variable respiratory distress in the newborn period or recurrent lung infections in childhood. Management is postnatal resection, but for specific high-risk lesions, management entails prenatal therapies. Overall, pulmonary resection in the newborn is very well tolerated and is associated with an excellent long-term functional outcome.