Congenital lobar emphysema

Congenital lobar emphysema (CLE), also known as congenital lobar overinflation or infantile lobar emphysema, is a rare developmental anomaly of the lung characterized by hyperinflation of one or more pulmonary lobes. The condition results from overdistension of the alveoli within the affected lobe, leading to air trapping and progressive expansion of lung tissue. This hyperinflated lobe can compress adjacent normal lung tissue and may cause mediastinal shift, compromising respiratory and cardiovascular function. The left upper lobe is most commonly affected, followed by the right middle lobe and right upper lobe. Lower lobes are rarely involved. CLE primarily affects the respiratory system and typically presents in the neonatal period or early infancy with progressive respiratory distress, tachypnea, wheezing, cyanosis, and difficulty feeding. In some cases, the condition may be detected prenatally on ultrasound or may remain asymptomatic and be discovered incidentally on chest imaging. The underlying cause is often idiopathic, though in some cases it may be associated with deficiency or dysplasia of bronchial cartilage, external bronchial compression by aberrant vessels, or endobronchial obstruction such as mucus plugging or mucosal folds. CLE can occasionally occur in association with congenital heart defects or other congenital anomalies. Management depends on the severity of symptoms. Severely symptomatic neonates and infants typically require surgical intervention, with lobectomy (surgical removal of the affected lobe) being the definitive treatment and generally yielding excellent outcomes. Mildly symptomatic or asymptomatic patients may be managed conservatively with close monitoring, as some cases may stabilize or improve over time. Prognosis after surgical treatment is generally very good, with most children achieving normal lung function and growth. Early diagnosis and appropriate management are essential to prevent life-threatening respiratory compromise.

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