Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use

Abstract

Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this “state-of-the-art” review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma. Conclusion: Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown.What is known:• Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed.• While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies.What is new:• Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery.• New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.