Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses |
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Authors: | Kunisaki Shaun M Fauza Dario O Nemes Luanne P Barnewolt Carol E Estroff Judy A Kozakewich Harry P Jennings Russell W |
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Institution: | a Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA b Department of Radiology, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA c Department of Pathology, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA d The Advanced Fetal Care Center, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA |
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Abstract: | PurposeThis study was aimed at determining whether different congenital lung masses represent diverse manifestations of a single developmental abnormality associated with fetal airway obstruction.MethodsWe conducted a 3-year retrospective review of patients who underwent surgical resection of a prenatally diagnosed lung mass. Prenatal imaging was used to define mass position and its effect on adjacent organs. Lung specimens were examined through careful full-specimen microdissections, as well as by plain and contrast roentgenograms.ResultsTwenty-five patients underwent lung resection during this study period. Based on the final pathology reports, 56% were congenital cystic adenomatoid malformations, 12% were congenital lobar emphysemas, 8% were bronchopulmonary sequestrations, and 24% had features of both cystic adenomatoid malformation and bronchopulmonary sequestrations. No bronchogenic cysts were present in this series. Overall, bronchial atresia was identified in 77% of the examined specimens (n = 22) and was associated with all types of lung malformations.ConclusionsBronchial atresia is a common, unrecognized component of prenatally diagnosed congenital cystic adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysemas, and lesions of mixed pathology. Most congenital lung masses may be part of a spectrum of anomalies linked to obstruction of the developing fetal airway as an underlying component in their pathogenesis. |
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Keywords: | Bronchial atresia Congenital lung malformation Congenital lung mass Congenital cystic adenomatoid malformation Bronchopulmonary sequestration Congenital lobar emphysema Prenatal diagnosis Lung development Fetus |
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