Congenital lobar emphysema

A 22-year-old man was referred due to symptoms of dyspnea and chest pain. Chest radiography revealed large lucency in the right hemithorax with contralateral mediastinal shift (Picture 1). Chest CT showed a hyper-lucent, hyperextended right lower lung (Picture 2). Spirometry showed forced expiratory volume (FEV)1/forced vital capacity (FVC) 80.4%, FVC (Pred) 1.99 L (40%), FEV1 1.6 L. Lung perfusion/ventilation scan showed a large non-segmental V/ Q matched defect. He received volume reduction therapy. Microscopic sections showed irregular large cystic spaces lined with bronchiolar epithelium, and bronchiole-like structures with a fibromuscular core (Picture 3). The patient was diagnosed with congenital lobar emphysema. Dyspnea improved after surgery and follow-up spirometry showed FEV 1/FVC 83.3%, FVC (Pred) 2.63 L (52%), FEV1 2.19 L.Congenital lobar emphysema is uncommon and characterized by an over-distension of a histologically normal lung. It is caused by obstruction of the developing airway, thus leading to air trapping through a "ball-valve" mechanism. This condition usually presents within the first month of life, but is rare after 6 months of age (1). The left upper lobe is the most frequently affected lobe. Surgical resection of the affected lobes is appropriate for symptomatic patients (2).

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Congenital lobar emphysema

Cited by 3 publications

References 2 publications

Medical and Surgical Interventions for Respiratory Distress and Airway Management

Medical and Surgical Interventions for Respiratory Distress and Airway Management

Surgical Interventions for Respiratory Distress and Airway Management

Adult Unilateral Pulmonary Lucency

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