• A Hadchouel-Duvergé, G Lezmi, J de Blic, and C Delacourt.
• Service de pneumologie et d'allergologie pédiatriques, hôpital Necker-Enfants-Malades, 149 rue de Sévres, Paris cedex 15, France.
• Rev Mal Respir. 2012 Apr 1; 29 (4): 601-11.

IntroductionCongenital lung lesions comprise a broad spectrum of various malformations including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema, bronchial atresia and bronchogenic cyst. This review aims at the description of their natural history, and of the underlying pathophysiological mechanisms.State Of The ArtCongenital lung lesions are frequently diagnosed antenatally and many remain asymptomatic after birth. In the absence of antenatal identification, they are usually revealed by the occurrence of infection. In some cases, spontaneous resolution of the malformation can occur. Different pathogenic hypotheses are discussed for the origin of these abnormalities, and common processes appear likely to all of these malformations. Factors involved in the process of branching seem to play a particularly important role.PerspectivesProspective follow-up of operated and unoperated children would complete our knowledge about the natural history of these lesions. The contribution of experimental models has led to advances in the understanding of pathogenic mechanisms. Further studies are needed to identify the factors initiating the malformative process.Copyright © 2012 SPLF. Published by Elsevier Masson SAS. All rights reserved.

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