congenital lobar emphysema
Developmental abnormality causing progressive overdistention of one or more pulmonary lobes in infants.
Full Definition
Congenital lobar emphysema is a rare developmental abnormality characterized by progressive overdistention of one or more pulmonary lobes due to air trapping during expiration. The condition typically results from bronchial obstruction caused by deficient bronchial cartilage, external compression, or inflammatory stenosis. The left upper lobe is most commonly affected, followed by the right middle and right upper lobes. Infants may present with progressive respiratory distress, particularly during feeding or crying, as the affected lobe continues to expand and compress adjacent normal lung tissue. Severe cases can cause mediastinal shift and cardiovascular compromise.
Usage
Usage note: Commonly abbreviated as CLE; often requires surgical lobectomy in symptomatic cases.
In Context
- "The three-month-old infant presented with progressive dyspnea due to congenital lobar emphysema of the left upper lobe." — Pediatric emergency department evaluation
- "Chest CT demonstrated characteristic findings of congenital lobar emphysema with marked hyperinflation and mediastinal shift." — Pre-operative imaging assessment