Abstract
Introduction: congenital bronchopulmonary malformations are uncommon but potentially life-threatening. Methods: 73 patients were evaluated for primary or secondary bronchopulmonary abnormalities, in 2018. Results: 10 had static airway anomalies: congenital subglottic stenosis (n=2), left bronchial stenosis (n=4), congenital tracheal stenosis (n=4). 23 had dynamic airway abnormalities: tracheomalacia (n=21), laryngomalacia (n=2), bronchomalacia (n=3). 27 had bronchial tree and lung development anomalies: left bronchial atresia (n=2), right bronchial atresia (n=2), supernumerary bronchus (n=2), “tracheal” bronchus (n=4), bronchus rotation (n=1), cystic adenomatoid malformation (n=6), bronchogenic cyst (n=1), congenital lobar emphysema (n=2), lung hypoplasia (4), lung hypo-dysplasia (n=2). 8 patients had diaphragmatic anomalies: congenital diaphragmatic hernias (n=6) and relaxation (n=2). 35 had vascular anomalies determining trachea-bronchial tree involvement: double aortic arch (n=7), right aortic arch and arteria lusoria with or without Kommerell diverticulum (n=respectively 9 and 6) were the most frequent. 56 children had respiratory symptoms. All the woman performed a prenatal US: 18 detected a malformation, 1 was false negative. 6/7 fetal cardiac US were positive. 69 patients underwent chest CT scan, all were positive. 38 patients underwent flexible laryngo-tracheo-bronchoscopy (LTBS), 35 were positive. Surgery was performed in 31 children. Conclusions: we propose the description of large sample of pediatric patients with congenital bronchopulmonary malformations, followed from one Center. Chest CT and LTBS are important investigation in congenital airway anomalies.
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