Congenital Lobar Emphysema

Excerpt

Congenital lung diseases are rare but exceptionally distinct in their presentation, ranging from large masses requiring immediate surgical intervention to small and asymptomatic lesions. The effects of congenital lung malformations can be critical; thus, knowledge of the diagnosis and treatment of these abnormalities is essential. Lung development and anatomy are critical fundamentals in considering these malformations and help in comprehension of the pathophysiology of each disease. Congenital lobar emphysema (CLE), or congenital lobar overinflation, is the excessive inflation of the lung lobes without destroying the alveoli. This condition arises from a "ball valve" effect, causing bronchial blockage and inadequate bronchial cartilage development.

CLE is a rare developmental malformation of the lung with a wide range of presentations that pose a diagnostic and therapeutic dilemma and are associated with high morbidity and mortality. CLE is characterized by respiratory distress due to overexpansion of 1 or more pulmonary lobes of the histologically normal lung without destroying alveolar walls with compression of surrounding lung parenchyma. Air trapping in the lung during the expiratory phase of respiration due to deficient bronchial cartilage causes repeated episodes of respiratory distress. The affected lobe is essentially nonfunctional because of overdistention and air trapping. CLE is frequently recognized in newborns; however, a few cases do not appear until adulthood. This disease is potentially reversible if diagnosed and treated on time.