Congenital pulmonary airway malformation type 3

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Synonyms: CCAM type 3 | CPAM type 3 | Congenital cystic adenomatoid malformation of the lung type 3 | Congenital cystic adenomatous malformation of the lung type 3 | Congenital cystic disease of the lung type 3

A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue consisting of numerous microcysts of less than 0.5 cm in diameter. The lesions have intracystic communications can be connected to the tracheobronchial tree and are usually unilateral involving an entire lobe. The condition may be associated with polyhydramnios fetal hydrops and stillbirth or present with severe respiratory distress in the neonatal period.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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