Type 1 congenital pulmonary airway malformation: diagnostic accuracy of radiological features
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Authors
Cook, James
Muntean, Ancuta
Patel, Shailesh B.
Haque, Saira
Allen, Pamela
Nicolaides, Kypros
Davenport, Mark
Greenough, Anne
Ade‐Ajayi, Niyi
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Issue Date
2026
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Article
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BACKGROUND: There are several different types of congenital lung malformation (CLM). Malignant transformation to adenocarcinoma is almost exclusively associated with type 1 congenital pulmonary airway malformation (CPAM). Histological examination is the only reliable method to define malformation subtype. AIM OF STUDY: To determine if the largest cyst diameter on CT imaging is an accurate diagnostic test for the presence or absence of type 1 CPAM on histological analysis. METHODS: All cases of antenatally identified CLM that underwent surgical resection between 2004 and 2023 were identified. The index test (diameter of largest cyst on CT imaging) was determined for each case. The reference standard test was the diagnosis of type 1 CPAM on the histopathology report. Measures of accuracy of the index test in detecting the presence or absence of type 1 CPAM were calculated. RESULTS: For asymptomatic cases (n = 117) the prevalence of type 1 CPAM was 23.9%. Largest cyst diameter discriminated well between those with and without type 1 CPAM, with an area under the receiver operating characteristic curve of 0.852 (95% CI 0.77-0.91, p < 0.001). At a threshold diameter of ≥ 5 mm the index test performed accurately: Sensitivity 85.7% (95% CI 67.3-96.0), specificity 76.5% (95% CI 65.8-85.2), positive predictive value 55.8% (95% CI 45.3-65.8) and negative predictive value 93.9% (95% CI 86.1-97.5). CONCLUSION: In this cohort type 1 CPAM has a low prevalence and could be accurately distinguished through radiological features.
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Pediatric Pulmonology
Volume
61
Issue
3