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      Structural and perfusion magnetic resonance imaging of congenital lung malformations

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          Abstract

          Background

          A radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children.

          Objective

          To describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification.

          Materials and methods

          This 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.

          Results

          Main 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.

          Conclusion

          A 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.

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          Most cited references27

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          Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation.

          Congenital lung abnormalities are being detected more frequently at routine high-resolution prenatal ultrasonography. The most commonly encountered anomalies include lung agenesis-hypoplasia complex (pulmonary underdevelopment), congenital pulmonary airway malformations, congenital lobar overinflation, bronchial atresia, bronchogenic cysts, congenital high airway obstruction syndrome, scimitar syndrome, and bronchopulmonary sequestration. Recognizing the antenatal and postnatal imaging features of these abnormalities is necessary for optimal prenatal counseling and appropriate peri- and postnatal management. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.306105508/-/DC1. © RSNA, 2010.
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            Congenital cystic lung disease: contemporary antenatal and postnatal management.

            Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.
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              New concepts in the pathology of congenital lung malformations

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                Author and article information

                Contributors
                christian.kellenberger@kispi.uzh.ch
                Journal
                Pediatr Radiol
                Pediatr Radiol
                Pediatric Radiology
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                0301-0449
                1432-1998
                17 April 2020
                17 April 2020
                2020
                : 50
                : 8
                : 1083-1094
                Affiliations
                [1 ]GRID grid.412341.1, ISNI 0000 0001 0726 4330, Department of Diagnostic Imaging, , University Children’s Hospital Zürich, ; Steinwiesstr. 75, CH 8032 Zürich, Switzerland
                [2 ]GRID grid.412341.1, ISNI 0000 0001 0726 4330, Children’s Research Centre, , University Children’s Hospital Zürich, ; Zürich, Switzerland
                [3 ]GRID grid.412341.1, ISNI 0000 0001 0726 4330, Department of Pediatric Surgery, , University Children’s Hospital Zürich, ; Zürich, Switzerland
                [4 ]GRID grid.7400.3, ISNI 0000 0004 1937 0650, Institute of Pathology, University Hospital Zürich, , University of Zürich, ; Zürich, Switzerland
                [5 ]GRID grid.412341.1, ISNI 0000 0001 0726 4330, Division of Pulmonology, , University Children’s Hospital Zürich, ; Zürich, Switzerland
                Article
                4658
                10.1007/s00247-020-04658-5
                7329781
                32303778
                d76d12bd-cc1a-4e57-90b0-5afacabe7e99
                © The Author(s) 2020

                Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 2 September 2019
                : 30 December 2019
                : 10 March 2020
                Categories
                Original Article
                Custom metadata
                © Springer-Verlag GmbH Germany, part of Springer Nature 2020

                Pediatrics
                bronchopulmonary malformation,children,lung,magnetic resonance imaging,perfusion imaging

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