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Congenital lung malformations are a spectrum of disorders arising from errors in formation of the airways or vascular supply of the lung. Although major congenital lung malformations are often presented as separate entities, there is great overlap between these abnormalities, and a single congenital abnormality may not fit neatly into one category.

The wheel theory of airway and vessel malinosculations (Figure 14-1) helps to understand the malformations.1  In this approach, the developmental interactions of bronchial, acinar, and vascular components are interpreted along a continuum. A congenital abnormality may be isolated to large airways (eg, tracheomalacia), involve abnormalities...

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