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      Double aortic arch: a rare cause of stridor in infants

      case-report
      , , ,
      Journal of Surgical Case Reports
      Oxford University Press

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          Abstract

          Double aortic arch is the most common vascular ring anomaly. It usually presents with symptoms related to tracheal and oesophageal compression. The constricting vascular ring may lead to stridor in infants and young children, which could be mistaken for upper respiratory tract infections or foreign body aspiration. It is therefore prudent to have a high index of suspicion when evaluating cases of paediatric stridor. Contrast-enhanced computed tomography and cardiac magnetic resonance imaging are the diagnostic modalities of choice to investigate vascular rings. We report a case of a stridulous infant with a double aortic arch.

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          Vascular rings.

          The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time.
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            Prenatal Sonographic Features of a Double Aortic Arch: Literature Review and Perinatal Management.

            A double aortic arch is a relatively uncommon anomaly occasionally associated with congenital heart disease or the chromosome 22q11 deletion. We report a case of prenatal diagnosis of a double aortic arch in which the sonographic features in the 3-vessel and trachea view are highlighted. A PubMed-based search was made to retrieve all cases of prenatal diagnosis of double aortic arch. A total of 13 articles and 35 cases were found. The average gestational age at diagnosis was 29 weeks. Six cases had associated cardiac anomalies. Only 1 case had the 22q11 deletion, showing extracardiac anomalies without cardiac defect. The postnatal evolution was characterized by symptoms of tracheoesophageal compression in 72.4% of the cases. Detection of a double aortic arch should be followed by a thorough fetal scan and echocardiography, and a chromosomal study should be considered when the sonographic findings are consistent with the 22q11 deletion.
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              Double aortic arch as a source of airway obstruction in a child

              Double aortic arch (DAA) is a congenital vascular anomaly. The diagnosis was difficult till the child was symptomatic, and other causes were ruled out. We present the interesting images of a child of respiratory distress because of tracheal compression from DAA.
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                Author and article information

                Contributors
                Journal
                J Surg Case Rep
                J Surg Case Rep
                jscr
                Journal of Surgical Case Reports
                Oxford University Press
                2042-8812
                December 2021
                24 December 2021
                24 December 2021
                : 2021
                : 12
                : rjab567
                Affiliations
                Department of Otolaryngology , University Hospitals of Derby and Burton, Derby, UK
                Department of Otolaryngology , East Kent Hospitals University NHS Foundation Trust, Ashford, UK
                Department of Otolaryngology , University Hospitals of Derby and Burton, Derby, UK
                Department of Otolaryngology , East Kent Hospitals University NHS Foundation Trust, Ashford, UK
                Department of Otolaryngology , University Hospitals of Derby and Burton, Derby, UK
                Department of Otolaryngology , East Kent Hospitals University NHS Foundation Trust, Ashford, UK
                Department of Otolaryngology , University Hospitals of Derby and Burton, Derby, UK
                Department of Otolaryngology , East Kent Hospitals University NHS Foundation Trust, Ashford, UK
                Author notes
                Correspondence address. Department of Otolaryngology, East Kent Hospitals University NHS Foundation Trust, 32 Nuthatch Drive, Finberry, Ashford TN25 7AA, UK. Tel: +44-7438827559; E-mail: youssef.aladham@ 123456alexmed.edu.eg
                Author information
                https://orcid.org/0000-0002-2843-4412
                Article
                rjab567
                10.1093/jscr/rjab567
                8711860
                0a76b7f5-e4b6-43f7-a048-56acd1280436
                Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2021.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 31 October 2021
                : 22 November 2021
                Page count
                Pages: 2
                Categories
                Case Report
                AcademicSubjects/MED00910
                jscrep/070

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