Abstract

BackgroundA radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children.ObjectiveTo describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification.Materials and methodsThis is a retrospective analysis of consecutive MRI examinations performed for suspected congenital lung anomalies in 39 children (median age: 3.8 months, range: 2 days–15 years). Morphological and perfusion findings were characterised on respiratory-gated fast spin echo and dynamic contrast-enhanced sequences obtained at 1.5 tesla. Abnormalities were classified independently by two readers and compared to an expert diagnosis based on pathology, surgery and/or other imaging.ResultsMain diagnoses included bronchopulmonary lesions in 33 patients, scimitar syndrome in 4 patients, pulmonary arteriovenous malformation and oesophageal duplication cyst in one patient each. Of 46 observed abnormalities, 44 (96%) were classified correctly with very good interobserver agreement (96% concordance rate). The 39 detected lung lesions included isolated overinflation (17/39, 44%), cystic pulmonary airway malformation (8/39, 21%), bronchopulmonary sequestration (7/39, 18%), bronchogenic cyst (4/39, 10%) and hybrid lesion (3/39, 8%). All lung lesions presented as perfusion defect at peak pulmonary enhancement. Non-cystic lesions showed a delayed peak (median delay: 2.8 s, interquartile range: 0.5 to 4.0 s) in relation to normal lung parenchyma.ConclusionA dedicated lung MRI protocol including respiratory compensated sequences, dynamic angiography and perfusion is able to reliably delineate parenchymal and vascular components of congenital bronchopulmonary foregut malformations. Therefore, MRI may be considered for comprehensive postnatal evaluation of congenital thoracic malformations.

Highlights

  • Congenital lung disease includes a broad variety of rare developmental anomalies that may be clinically relevant [1, 2], including congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration, bronchogenic cyst, congenital segmental or lobar overinflation, venolobar syndrome and other vascular malformations

  • Lung magnetic resonance imaging (MRI) studies performed to assess congenital lung lesions from July 2013 to July 2019 were searched on the picture archiving and communication system (PACS) of our tertiary university paediatric hospital

  • The study population consisted of 39 children, 18 girls and 21 boys, with a median age of 3.8 months, interquartile range of 3.1 to 12.1 months and age range of 2 days to 14.7 years at MRI examination

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Summary

Introduction

Congenital lung disease includes a broad variety of rare developmental anomalies that may be clinically relevant [1, 2], including congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration, bronchogenic cyst, congenital segmental or lobar overinflation, venolobar syndrome and other vascular malformations. With the advent of prenatal ultrasound (US), congenital lung lesions have been estimated at about 1 in 3,000 live births [3]. Imaging plays an eminent role in lesion detection and characterisation, which are the basis for antenatal and postnatal management of affected foetuses and infants. Many congenital thoracic malformations are considered a spectrum of an in utero airway obstruction–lung malformation sequence with accompanying. A radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children

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