Congenital pulmonary airway malformation type 1
Synonyms: CCAM type 1 | CPAM type 1 | Congenital cystic adenomatoid malformation of the lung type 1 | Congenital cystic adenomatous malformation of the lung type 1 | Congenital cystic disease of the lung type 1
A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue with one or more dominant cysts of 2 to 10 cm in diameter which may be surrounded by smaller cysts. The lesions have intracystic communications can be connected to the tracheobronchial tree and are usually unilateral involving a single lobe. Small lesions may remain asymptomatic while most cases present with respiratory distress in the neonatal period or in infancy or with recurrent respiratory infections later in life. Pulmonary hypoplasia and severe fetal hydrops are rare complications. The condition is associated with an increased risk of pulmonary malignancy such as bronchoalveolar carcinoma.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version September 2023.
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