Algorithm for management of congenital abnormalities of the lower airway

CPAM: congenital pulmonary airway malformation (also known as congenital cystic adenomatoid malformation [CCAM]); BPS: bronchopulmonary sequestration; PPB: pleuropulmonary blastoma; CT: computed tomography; MRI: magnetic resonance imaging.
* This pathway also applies to asymptomatic patients in whom a congenital abnormality of the lower airway is identified as an incidental finding on chest radiograph.
¶ Either CT or MRI may be used for advanced thoracic imaging. Doppler ultrasound can be considered if contrast-enhanced imaging is not used to identify the vascular supply of the lesion.
Δ PPB risk factors include bilateral or multifocal cysts, pneumothorax, or a family history of PPB-associated conditions (including lung cysts, spontaneous pneumothorax, and several specific childhood cancers or dysplasias).
◊ There is no consensus among experts in this field regarding observation versus elective surgical resection for asymptomatic infants with low-risk characteristics. Refer to UpToDate topic text for discussion.