Background Although fetal ultrasound, fetal MRI and postnatal CT are now widely used in the evaluation of congenital lung malformations (CLM), their diagnostic accuracy remains undefined.
Objective To correlate prenatal and postnatal imaging studies with pathological data after CLM resection.
Design Retrospective, descriptive case series study.
Setting A North American tertiary care centre.
Patients One hundred and three consecutive lung resections for a suspected CLM between 1 January 2005 and 31 December 2015.
Main outcome measures Diagnostic accuracy of imaging diagnosis compared with pathological evaluation.
Results Pathological diagnoses included congenital pulmonary airway malformation ((CPAM) n=45, 44%), bronchopulmonary sequestration (BPS; n=25, 24%), CPAM/BPS hybrid lesions (n=22, 21%) and pleuropulmonary blastoma (n=2, 2%). Fetal ultrasound detected 85 (82.5%) lesions and correctly diagnosed whether or not a lesion was a CPAM in 75% of cases (sensitivity 93%, specificity 32%). Fetal MRI had a similar concordance rate (73%) but was superior in correctly determining whether a systemic feeding vessel was present in 80% of cases (sensitivity 71%, specificity 88%) compared with an ultrasound accuracy rate of 72% (sensitivity 49%, specificity 93%). By comparison, postnatal CT correctly diagnosed whether a CPAM was present in 84% of cases (sensitivity 86%, specificity 77%) and whether a systemic feeding vessel was present in 90% of cases (sensitivity 92%, specificity 88%).
Conclusions Fetal ultrasound remains an important tool in the detection and evaluation of congenital lung malformations. However, it does not correctly predict histology in approximately 25% of prenatally detected CLMs and remains limited by relatively poor sensitivity for systemic feeding vessels pathognomic for a bronchopulmonary sequestration. These data suggest the importance of obtaining additional cross-sectional imaging, preferably a postnatal CT scan, in all patients to help counsel families and to guide in the optimal management of these lesions.
- paediatric surgery
- fetal medicine
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Contributors GBM, MCT and SMK contributed to the study conception and design. RAM, KNJ and AH were responsible for the acquisition of data. RAM, ML-T, MCT and SMK performed the analysis and interpretation of data. RAM and SMK drafted the manuscript. RAM, MCT and SMK contributed to the critical revision of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Not required.
Ethics approval This HIPAA-compliant study was approved by the Michigan Medicine Institutional Review Board (#51132)
Provenance and peer review Not commissioned; externally peer reviewed.
Data sharing statement The University of Michigan is committed to the open and timely dissemination of research outcomes and will adhere fully to the NIH Statement on Sharing Research Data (http://grants.nih.gov/grants/guide/noticefiles/NOT-OD-03-032.html) and the NIH Grants Policy Statement http://grants.nih.gov/grants/policy/nihgps_2003/index.htm). Research Resources generated with funds from this grant will be readily available in a timely fashion for sharing and distribution to any academic investigators for the purposes of non-commercial research.