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Paediatr Respir Rev. 2013 Sep;14(3):169-70. doi: 10.1016/j.prrv.2013.06.003. Epub 2013 Jul 12.

Shall all congenital cystic lung malformations be removed? the case in favour.

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AP-HP, Hôpital Necker-Enfants Malades, Service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France.


The need to systematically remove congenital cystic lung lesions is based on three main arguments. First, cystic malformations are often considered as congenital cystic adenomatoid malformations (CCAM), while other less favorable diagnoses are possible, such as pleuropulmonary blastoma. Only postsurgical pathological analysis allows diagnosis. Second, there are clinical and biological arguments for considering macrocystic lesions as likely to degenerate. The only prevention is surgical removal. Finally, there is no recommendation on how to follow these children, in the absence of removal, causing unnecessary family stress. This seems unjustified, compared to a feasibility of thoracoscopic removal in most cases.

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