Congenital lobar emphysema – rare cause of acute respiratory distress

Abstract

Congenital lobar emphysema is defined as the hyper­in­fla­tion of one or more pulmonary lobes in the absence of an extrinsic bronchial obstruction. Clinical presentation ranges from asymptomatic (incidental discovery) to pro­gres­sive respiratory failure installed in the first months of life or acute respiratory failure installed immediately after birth. The sooner the symptom appears, the more the disease causes severe respiratory distress syndrome and the need for surgery is more stringent. The diagnosis will be radiologically established, confirmed by CT scan or nuclear magnetic resonance, and completed by echocardiography for possible associated cardiac abnormalities. The treatment is surgical, namely lobectomy, classically, or video assisted thoracoscopy, and is also indicated for cases discovered by accident, due to the risk of infection or malignancy. The prog­nosis is generally favorable, including in cases where re­section involves several lobes, probably due to the com­pen­sa­tory alveolar growth in the child, although in the case of multiple lobes affected, mortality may exceed 10%.