No abstract

Congenital pulmonary airway malformations (CPAM) are rare anomalies of the lung development, arising during intra-uterine life. Currently, CPAM are diagnosed prenatally, due to improved imaging techniques. They may be asymptomatic or cause a broad range of symptoms. Symptomatic lesions require treatment, which might be performed during fetal period or after birth. Surgical options vary from minimally invasive to open surgery. Concerning the long-term assessment of respiratory function after lung resection, long-term outcome studies are still lacking. A multi-disciplinary team play an essential role in ensuring that the patients affected by CPAM receive the most appropriate treatment and follow-up to preserve the lung function.IMPACT STATEMENT: The purpose of this revision is to clarify the still controversial management of congenital pulmonary airways malformation in pediatric age.