Algorithm for management of congenital pulmonary airway malformations (CPAM) in infants and children

CPAM: congenital pulmonary airway malformation; PPB: pleuropulmonary blastoma; CT: computed tomography; MRI: magnetic resonance imaging.

* Symptoms may consist of respiratory distress, tachypnea, and/or failure to thrive.

¶ Either CT or MRI may be used for advanced thoracic imaging.

Δ PPB risk factors include DICER1 mutations; bilateral or multifocal cysts; pneumothorax; or family history of DICER1 syndrome or 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. Experts generally agree that observation entails a measurable risk for complications of CPAM, which occasionally are catastrophic. Moreover, outcomes are worse for patients undergoing surgery after symptoms develop compared with those undergoing elective surgery. Most experts also agree that some patients can be observed if the lesion is small and the patient remains completely asymptomatic.