Published online by Cambridge University Press: 01 August 2009
Congenital cystic adenomatoid malformations of the lung (CCAMs) were originally described by Ch'In and Tang in 1949 as rare lung lesions occurring in premature or stillborn infants with anasarca. In 1977 Stocker et al divided CCAM into three types based on their clinical and pathological features. The nomenclature has since changed to congenital pulmonary airway malformations (CPAMs) to reflect the possible absence of cystic changes and the probable underlying pathogenesis of different types. CPAMs are relatively rare congenital abnormalities with a reported incidence varying from 1 in 10,000 to 1 in 35,000. There is a slight male predominance, but no racial predilection has been noted. This review will outline the current nomenclature and features of different types of CPAMs, highlight possible mechanisms underlying the development of CPAMs, review current antenatal imaging modalities and interventions, address the debate surrounding the postnatal management of CPAMs, and suggest areas for future research.