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From Childhood to Adulthood: A Case of Congenital Tracheal Stenosis
Inês Duarte1, Filipa Canedo1, Mario Pinto1
1Pulmonology, Hospital Santa Marta, Unidade Local de Saúde São José, Lisbon, PRT.
Abstract:
Congenital tracheal stenosis (CTS) is a rare and potentially life-threatening malformation, often associated with cardiovascular anomalies, such as pulmonary artery sling. We report a 20-year-old woman with a history of pulmonary artery sling surgically corrected in infancy, who presented with diffuse CTS. Early symptoms of dyspnea, wheezing, and episodes of airway obstruction prompted evaluation, and imaging with computerized tomography and flexible bronchoscopy confirmed complete tracheal rings with a lumen of 6-8 mm. Pulmonary function tests showed fixed airway obstruction and flattening of inspiratory and expiratory flow-volume curves. At 19 years, she remained symptomatic with exertional stridor but preserved exercise tolerance, and follow-up imaging demonstrated stable diffuse stenosis. Given relative clinical stability and diffuse involvement unsuitable for endoscopic intervention, conservative management with regular multidisciplinary follow-up and symptomatic therapy was pursued. This case highlights the diagnostic and therapeutic challenges of CTS in adulthood and emphasizes the importance of early recognition, functional assessment, and individualized multidisciplinary management. Further studies are needed to determine long-term outcomes and optimal surveillance strategies in adults with persistent diffuse tracheal stenosis.
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