Congenital Lobar Emphysema in a Mother and Daughter

Abstract

Congenital lobar emphysema (CLE) is a well-described cause of respiratory distress in infancy and is characterized by hyperinflation of the affected lobe with compression ofsurrounding normal lung tissue. On occasion a specific cause for the lobar hyperinflation, such as torsion of the bronchus, extrabronchial vascular compression, or bronchial atresia, may be demonstrated.1 More common, however, is the association of CLE with presumed hypoplastic bronchial cartilage1-3 and dynamic bronchial collapse. CLE is not usually described as an inherited disease. To our knowledge, there have been no previously reported cases of a parent and child with CLE. We report here a mother and daughter, both of whom had congenital right middle lobar emphysema.