Congenital lung and airway malformations — clinical case

Case summary

This child presents the classic postnatal face of a congenital lung malformation in a setting without antenatal detection: pneumonia that keeps returning to the same lobe with a wet cough that never fully clears. Same-site recurrence is the signal that a fixed structural lesion is providing a reservoir for infection, and in a child born without antenatal imaging a previously undetected congenital malformation such as a CPAM or an intralobar sequestration is high on the list rather than simple bad luck with viruses. [1] [2]

Initial assessment and investigations

The assessment confirms true recurrence and defines the pattern by reviewing every previous film, which shows that each pneumonia struck the same left lower zone and that the current opacity has not fully resolved. Because the disease is fixed in one site, the priority is structural imaging rather than a broad systemic screen, and a normal-looking area on plain film cannot be trusted to exclude a malformation. A contrast-enhanced chest CT is obtained and is the pivotal test: it characterises a left lower lobe lesion and, importantly, looks for a systemic feeding artery from the aorta that would define a sequestration. [1] [2]

The CT demonstrates a partly cystic left lower lobe lesion supplied by an anomalous systemic artery, confirming an intralobar sequestration that has been recurrently infected. Identifying the feeding vessel is essential because it both secures the diagnosis and warns the surgical and interventional teams what to expect at operation, since an unrecognised systemic artery is a source of serious intraoperative haemorrhage. [2] [1]

Management

Because the lesion is symptomatic with recurrent infection, the definitive management is resection, and the current infection is first treated to community-acquired pneumonia guidelines with airway clearance. Surgery removes the reservoir for further infection and the small long-term risk of malignant change, and the standard operation is lobectomy, with lung-sparing anatomical segmentectomy considered where the anatomy allows and expertise exists. The anomalous feeding artery is controlled deliberately at operation. [3] [2]

Disposition and counselling

After resection the child is followed to confirm that the recurrent pneumonia stops and that the remaining lung compensates, with the expectation of preserved long-term respiratory function. The family is counselled that a previously undetected congenital lesion, not a weak immune system, caused the repeated infections, and that same-site recurrence in any child should prompt imaging and a surgical opinion rather than repeated antibiotic courses. Structured respiratory follow-up is arranged, with attention to equitable access given the family arrived without antenatal or newborn imaging. [3] [1]