Congenital lung and airway malformations — short-answer question

Part A — Classification and investigation (10 marks)

a) How do you classify congenital lung malformations, and where does this lesion sit (4 marks)

I sort these lesions first by blood supply. A systemic feeding artery arising from the aorta defines a bronchopulmonary sequestration, while all the other lesions keep a normal pulmonary supply. Among the pulmonary-supplied lesions are CPAM, congenital lobar emphysema, bronchogenic cyst and bronchial atresia, and hybrid lesions combining features are common. A multicystic lower-lobe lesion with a pulmonary supply is most consistent with a congenital pulmonary airway malformation. ^[1]

b) Outline your postnatal investigation of this well baby (6 marks)

I would first confirm the baby is genuinely asymptomatic with a comfortable respiratory pattern, normal saturations and symmetrical air entry, and take a history of the antenatal scans and any family history of DICER1-associated tumours. A chest radiograph is only a screen and must never be taken to exclude the lesion, because many malformations become inconspicuous once the neonatal lung fills with air. The definitive investigation is a contrast-enhanced chest CT, usually obtained in the first few months of life, which characterises the lesion and maps any systemic feeding vessel. I would also actively exclude a congenital diaphragmatic hernia by confirming the diaphragm and stomach position. ^{[2] [1]}