Congenital pulmonary airway malformation type 4
Synonyms: CPAM type 4 | Congenital cystic adenomatoid malformation of the lung type 4 | Congenital cystic adenomatous malformation of the lung type 4
A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue with peripheral large thin-walled often multiloculated cysts which may be 8 cm in diameter. The lesions have intracystic communications can be connected to the tracheobronchial tree and are usually unilateral involving a single lobe. Patients present with respiratory distress or respiratory infections in the neonatal period or in infancy. The condition is often associated with tension pneumothorax signs of mediastinal shift and malignant transformation to pleuropulmonary blastoma type 1.
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 http://www.orphadata.org. Data version April 2024
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