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.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA1054.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019