Congenital lung malformations – Primer (2023) Federica Pederiva

Description

This Primer summarizes current progress in the diagnosis, management, and molecular understanding of Congenital Lung Malformations (CLMs), including congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration. It addresses the range of postnatal presentations, the role of antenatal ultrasonography and chest CT angiography in diagnosis, the ongoing debate regarding surgical versus expectant management for asymptomatic cases, and the associated risks of malignancy. The review also explores the biological mechanisms, such as KRAS mutations, linked to malignant transformation in CLMs.