Abstract
Congenital lobar emphysema (CLE) is a rare lung congenital malformation. Differential diagnosis of the disease remains challenging in an infant with acute respiratory distress. We report a case of a 3-week-old female infant with a weight of 2.1 kg who presented respiratory distress related to CLE. Left upper lobectomy was performed and she had an uneventful recovery.
Highlights
Congenital lobar emphysema (CLE) is a rare anomaly of lung development and often appears in the neonatal period, with hyperinflation of one or more pulmonary lobes, in the absence of bronchial obstruction [1]
CLE is usually being diagnosed during prenatal evaluation by ultrasonography and may be associated with polyhydramnios and fetal hydrops
Diagnosis of CLE is crucial and in many cases is complicated due to the variety of its clinical presentation that varies from mild tachypnea to severe respiratory distress [4]
Summary
Congenital lobar emphysema (CLE) is a rare anomaly of lung development and often appears in the neonatal period, with hyperinflation of one or more pulmonary lobes, in the absence of bronchial obstruction [1]. The etiology of congenital lobar emphysema is idiopathic in half of cases, whereas the other 50% have several mechanisms proposed to explain the air-trapping, which can be divided into intrinsic and extrinsic subtypes [3]. Diagnosis is crucial and in many cases it is difficult to differentiate between CLE and hyperinflation resulting from extrinsic bronchial obstruction (lymph nodes, vessels, masses, or cysts) that compresses the bronchus and produces valve obstruction. It is stated that even more than half of CLE cases are not related to airway abnormalities. Surgical removal is the most common treatment choice with operative mortality rate about 3 to 7% [2]
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