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Congenital lobar emphysema is a rare developmental lung disorder characterized by overdistention of one or more pulmonary lobes due to air trapping during expiration. The condition typically presents in newborns and infants with respiratory distress, and most commonly affects the left upper lobe or right middle lobe.
The primary mechanism involves defective bronchial cartilage development or intrinsic airway obstruction that creates a ball-valve effect, allowing air entry during inspiration but preventing air exit during expiration. This leads to progressive hyperinflation of the affected lobe, which can compress adjacent lung tissue and shift mediastinal structures, potentially causing cardiovascular compromise.
Diagnosis should be suspected in infants presenting with progressive respiratory distress, asymmetric chest expansion, and decreased breath sounds over the affected area. Chest imaging typically shows hyperinflated lobes with compressed adjacent lung tissue, and treatment often requires surgical lobectomy for symptomatic cases to prevent respiratory failure and allow normal development of remaining lung tissue.