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]

Part B — The management decision (10 marks)

a) How would you approach the decision between surgery and surveillance (6 marks)

Because the baby is asymptomatic, this is the central and unsettled controversy of the field, and I would present both sides honestly to the family. The case for elective resection is that it removes a lifelong reservoir for recurrent same-site infection and a small but real risk of malignancy, and that operating on a well infant is technically favourable. The case for structured surveillance is that many lesions never cause trouble and that surgery carries its own anaesthetic and operative risk. Recent systematic review evidence supports the safety of conservative management of a truly asymptomatic CPAM when follow-up is reliable, while matched cohorts continue to weigh long-term operative against conservative outcomes. [3] [4]

b) If surveillance is chosen, what does safe follow-up require (4 marks)

Safe surveillance must be genuinely structured rather than a vague promise to keep an eye on things. It requires a definitive CT diagnosis, clear imaging intervals, explicit triggers to proceed to surgery such as infection or growth, and a family who understand the plan and can access specialist review. I would document the plan, arrange coordinated local and tertiary follow-up, and counsel the family to seek review for recurrent same-site pneumonia, which would prompt imaging and a surgical opinion rather than repeated antibiotics. [4] [3]