Congenital lung disease: a plea for clear thinking and clear nomenclature

Abstract

Antenatal ultrasound has allowed the discovery of abnormalities which in the past often escaped detection. It may be unclear what advice to offer. Many large lung malformations seen at the routine 20-weeks gestation scan have largely disappeared at term. There is now even more confusion in terms of how these malformations should be described. The nomenclature of congenital lung disease was never very clear, with terms such as sequestrated segment, cystic adenomatoid malformation, hypoplastic lung, and malinosculation being used to describe often overlapping abnormalities. However, today these terms are used inconsistently in the ante- and postnatal periods. For example, congenital cystic adenomatoid malformation (CCAM) is used prenatally to describe a lesion which may well disappear before birth, but is used postnatally to describe an abnormality which may require lobectomy. CCAM may have a pulmonary arterial supply, or be supplied like a sequestration from the aorta, and histological features of both lesions may coexist. Thus, a complete reappraisal of the nomenclature of congenital lung disease is timely; this review does not discuss treatment options.