Tracheal compression by aberrant innominate artery: clinical presentations in infants and children, indications for surgical correction by aortopexy, and short- and long-term outcome

BackgroundAberrant innominate artery (AIA) may cause various degrees of tracheal compression (TC).PurposeThe aim of this study is to define the clinical manifestations of AIA-induced TC and outcome after aortopexy in infants and older children.MethodsChildren with significant AIA-induced TC were evaluated, and information after surgery or conservative management was obtained by telephonic interview after 1 to 4 years since discharge.ResultsOverall, 15 infants (mean age, 8 months; group A) and 13 older children (mean age, 56 months; group B) were evaluated. Although median age at onset of symptoms was comparable in the 2 groups, mean delay to diagnosis was higher in group B (P < .0001). Analysis of the most prevalent symptoms showed that reflex apneas were more frequent in group A (P = .02), whereas chronic “intractable” cough was more frequent in group B (P < .001). Because of the type and severity of symptoms and the degree of TC, 16 patients underwent aortopexy. Follow-up evaluation showed, in all but 1 patient, a significant improvement in symptoms and quality of life, measured by a modified Visick score.ConclusionsAberrant innominate artery-TC leads to a variety of respiratory disorders, with a difference in prevalence between infants and older children. When choice of treatment is based on clinical presentation and degree of TC, a good clinical outcome may be obtained also in children in whom aortopexy is indicated, that is, those presenting initially with more severe symptoms.