An Unusual Presentation of Congenital Lobar Emphysema

Abstract

Congenital lobar emphysema is an uncommon bronchopulmonary malformation characterized by lobar overinflation and accompanying alveolar septum damage that leads to compression atelectasis of the lung parenchyma and displacement of mediastinal structures, with the resultant ventilation-perfusion mismatch. We present a case of a 33-year-old lady with progressive exertional dyspnea. Chest radiograph findings lead to the suspicion of congenital lobar emphysema, which was then confirmed by a computed tomography (CT) scan. This condition is most commonly identified in newborns, with very few cases being reported in adults. Lobectomy remains the treatment of choice and in general has good outcome.

Persistent respiratory distress in a neonate: a diagnostic dilemma

“…There are published reports of CLE being diagnosed beyond the neonatal period also 5. Similar to our index case, there is a published report of CLE presenting as recurrent pneumothorax in an adolescent 6.…”

Management of Congenital Lobar Emphysema: The Current Challenges

“…Rusakow and Khare [17] described radio graphically occult CLE presented as unexplained neonatal tachypnea. Arnaud et al [18] presented a case of a 33-year-old lady with progressive exertional dyspnea.…”

A rare case of congenital lobar emphysema diagnosed in a 13-year-old boy

“…Rare abnormalities of the vessels, e.g. abnormal pulmonary venous return, may also be the cause of CLE [ 2 , 3 ]. According to some authors CLE can be hereditary [ 4 ].…”

“…Most cases are diagnosed in neonates, with male preponderance (boy to girl ratio 3 : 1) [ 1 – 4 ]. Typically the left upper lobe is involved (43%), then the right middle lobe (32%) and the right upper lobe (21%) [ 3 , 4 ]. CLE localized in the lower lobes is rather unique (2%) [ 3 , 4 ].…”

“…Typically the left upper lobe is involved (43%), then the right middle lobe (32%) and the right upper lobe (21%) [ 3 , 4 ]. CLE localized in the lower lobes is rather unique (2%) [ 3 , 4 ]. Bilateral localization was also observed [ 1 , 2 ].…”