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Congenital pulmonary airway (cystic adenomatoid) malformation

Christopher M Oermann, MD
Section Editors
Joseph A Garcia-Prats, MD
Gregory Redding, MD
Deputy Editor
Alison G Hoppin, MD


Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental anomaly of the lower respiratory tract [1,2]. Affected patients may present with respiratory distress in the newborn period or may remain asymptomatic until later in life. Many cases are now detected by routine prenatal ultrasound examination. Surgical resection is the definitive treatment.

The clinical presentation and postnatal management of CPAM is discussed below. Prenatal diagnosis, course, and management of CPAM are discussed in a separate topic review. (See "Prenatal diagnosis and management of congenital pulmonary airway malformation".)


Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. Although rare, it is the most common congenital lung lesion. The widespread use of antenatal ultrasound examination has resulted in an increase in the prenatal diagnosis of CPAM [3,4]. Data from large population registries suggest an incidence of congenital lung cysts in the range of 1 per 8300 to 35,000 live births [5,6]. Large-cyst subtypes account for about 70 percent of CPAMs, or 2 to 8 per 100,000 live births.

CPAMs occur sporadically. Their formation is not related to maternal factors such as race, age, or exposures. In some series, lesions that present in infancy have a slight male preponderance [7-10], although others found no gender predilection [11,12]. There is no known genetic predisposition, with the exception of type 4 malformations, which have been associated with a familial pleuropulmonary blastoma syndrome. (See 'Pleuropulmonary blastoma' below.)


Congenital pulmonary airway malformations (CPAMs) result from abnormalities of branching morphogenesis of the lung. The different types of CPAMs are thought to originate at different levels of the tracheobronchial tree and at different stages of lung development, possibly influenced by in utero airway obstruction and/or atresia [5,13,14]. (See 'Types' below.)


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Literature review current through: Sep 2016. | This topic last updated: May 9, 2016.
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