Congenital pulmonary airway malformation type 2

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Synonyms: CCAM type 2 | CPAM type 2 | Congenital cystic adenomatoid malformation of the lung type 2 | Congenital cystic adenomatous malformation of the lung type 2 | Congenital cystic disease of the lung type 2

A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue consisting of small cysts of less than 2 cm in diameter. The lesions have intracystic communications can be connected to the tracheobronchial tree and are usually unilateral involving a single lobe. The condition often presents with respiratory distress in the neonatal period or in infancy. It is frequently associated with other severe congenital anomalies such as renal agenesis or dysgenesis pulmonary sequestration or cardiac abnormalities.

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 June 2024

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