Congenital lobar emphysema: A rare cause of respiratory failure in neonates.

Abstract

Congenital lobar overinflation, also known as congenital lobar emphysema (CLE), is an uncommon (1/20,000-30,000 live births) abnormality characterized by hyperinflation of one or more pulmonary lobes, usually with contralateral displacement of the mediastinum. While the etiology of most cases of CLE is poorly understood and labeled idiopathic, some cases are thought to be due to an intrinsic or extrinsic bronchial wall abnormality causing a ball valve mechanism with resultant hyperinflation of the affected lobe. CLE tends to have a predilection for males presenting insidiously in the first 6 months of life and have respiratory distress and progressive failure, with 50% of cases being asymptomatic at birth. Acquired forms of lobar emphysema are similar but are secondary to prolonged exposure to oxygen and positive pressure ventilation in premature infants. Clinical presentation is variable, ranging from wheezing, increased respiratory effort, cyanosis, feeding difficulties, and reflux or respiratory failure. Chest radiography (CXR) is the initial imaging obtained for any neonate with respiratory distress, which can aid in diagnosis showing overinflation, while computerized tomography (CT) remains the gold standard for confirmatory diagnosis. Treatment is variable and based on clinical severity. Some cases can be managed conservatively, while more severe cases require surgical intervention with lobectomy. Here, we present a case of a neonate in respiratory distress soon after birth, had initial improvement with supportive care, and was found to be secondary to CLE.