1 - Unidade de Pediatria Médica 5.1, Área da Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central
2 - Unidade de Gastroenterologia Pediátrica, Área da Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central
3 - Unidade de Cirurgia Cardíaca, Área do Coração, Vasos e Tórax, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central
- 11th Excellence in Pediatrics. 6 de dezembro de 2019, Copenhaga, Dinamarca. Poster
Introduction: Stridor is a frequent physical sign in the pediatric population, present in a great number of both acute and chronic conditions. Differential diagnosis is based on the age of presentation and speed of onset. In infants, persistent biphasic stridor is more often due to a congenital abnormality, resulting in fixed airway obstruction, either by extrinsic compression, intraluminal mass or mural change of the airway.
Case Description: Infant boy, 5 months old, admitted since birth in a level III pediatric hospital, with a history of prematurity (32 weeks and 5 days), low weight at birth and corrected esophageal atresia with tracheoesophageal fistula, with associated severe gastroesophageal reflux disease and feeding difficulties. Over the course of several weeks, he presented with persistent biphasic stridor with multiple episodes of severe dyspnea, requiring supplementary oxygen. These episodes were not related to feeding and were not always attributable to body position changes. A bronchoscopy was performed, revealing tracheomalacia of the middle third with a reduction of 40-50% of the lumen, as well as a pulsatile impression on the anterior aspect of the trachea consistent with brachiocephalic artery. CT angiography confirmed the diagnosis of innominate artery compression syndrome, with the aberrant vessel molding the trachea and left main bronchus. The patient was referred for surgery and underwent aortopexy with excellent outcome.
Conclusions: Innominate artery compression syndrome is a rare congenital anomaly, included in the group of the vascular rings and slings. These abnormalities of the aortic arch are frequently associated with other congenital malformations, including tracheoesophageal fistulae. Surgical treatment is indicated only in symptomatic cases, which represent a minority of patients. This case illustrates the importance of investigating uncommon causes of stridor in infants presenting with persistent and not fully explained stridor, especially in those with known congenital malformations.
Keywords: infant, innominate artery compression syndrome, stridor, vascular ring