Congenital Pulmonary Airway Malformations

Abstract

Congenital Pulmonary Airway Malformation (CPAM) is a rare congenital malformation affecting the lungs which is characterised by a growth of a mass that originates from airway or lung tissue. These lesions are usually diagnosed in utero during maternal ultrasound screening. Most children are born asymptomatic, but some experience respiratory distress at birth, especially those with large lesions. If the masses grow very large in utero, it can lead to lung hypoplasia and mediastinal shift, resulting in hydrops fetalis, indicating an intrauterine intervention. Treatment of choice is lobectomy in the first year of life, either open or thoracoscopic. However, some surgeons prefer conservative treatment even though this is controversial.