A 4-month-old male with a medical history of late prematurity and two hospitalizations for viral bronchiolitis presented to the clinic for a new-patient well-child check. His previous hospitalizations for bronchiolitis did not present with hypoxia or respiratory distress, and he did not require any respiratory support. During further discussion, the parents reported that the patient had noisy breathing and congestion since birth. He often sounded “like he has something in his nose”. He had no history of fatigue, spit-up, or choking with feeds. He continued to formula-feed without difficulty and had appropriate weight gain. He was hospitalized for respiratory syncytial virus (RSV) bronchiolitis and rhinovirus bronchiolitis within a 2 week period at 3 months of age. During his first hospital admission, he had a chest X-ray (CXR) that was read as normal. Upon examination, the baby had audible congestion, noisy breathing, and coarse breath sounds that did not improve with albuterol in the clinic. No respiratory distress or stridor was appreciated. Given the history of persistent noisy breathing that parents reported for several weeks, he was referred to otolaryngology for further evaluation. He was noted to have stridor during his ear, nose, and throat (ENT) evaluation, and a computed tomography angiography (CTA) was ordered. The CTA demonstrated a double aortic arch with a dominant right aortic arch and narrowing of the trachea (Figure 1) and proximal mainstem bronchi bilaterally at the level of the vascular ring (Figure 2). The patient was diagnosed with a double aortic arch with tracheal narrowing and tracheobronchomalacia.

A 3D CTA demonstrates a double aortic arch with a dominant right aortic arch. There is a focal short segment of atresia involving the mid-portion of the left aortic arch and narrowing of the trachea. (A) Trachea, (B) atresia of the left aortic arch, and (C) dominant right aortic arch.

Computed tomography angiography (CTA) demonstrates narrowing of the proximal mainstem bronchi bilaterally at level of the vascular ring. (A) Distal trachea compression, (B) esophagus, (C) aortic arch.
The diagnosis of vascular rings can be challenging for pediatricians because symptoms are nonspecific and often overlap with common pediatric presentations [1, 2]. Pediatricians should have a high index of suspicion for a vascular ring in a child with noisy breathing, chronic cough or stridor, or dysphagia [3, 4]. Initial evaluation should include a two-view chest radiograph, followed by a bronchoscopy, echocardiogram, and computed tomography (CT) scan or magnetic resonance imaging (MRI) [5]. A comprehensive preoperative evaluation is essential to assess for concurrent cardiac and airway anomalies. Delays in diagnosis are common and can lead to further airway damage or death, thus prompt evaluation is important.
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Research funding: None reported.
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Author contributions: Both authors provided substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; both authors drafted the article or revised it critically for important intellectual content; both authors gave final approval of the version of the article to be published; and both authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
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Competing interests: None reported.
References
1. Licari, A, Manca, E, Rispoli, GA, Mannarino, S, Pelizzo, G, Marseglia, GL. Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonol 2015;50:511–24. https://doi.org/10.1002/ppul.23152.Search in Google Scholar PubMed
2. Backer, CL, Mongé, MC, Popescu, AR, Eltayeb, OM, Rastatter, JC, Rigsby, CK. Vascular rings. Semin Pediatr Surg 2016;25:165–75. https://doi.org/10.1053/j.sempedsurg.2016.02.009.Search in Google Scholar PubMed
3. Humphrey, C, Duncan, K, Fletcher, S. Decade of experience with vascular rings at a single institution. Pediatrics 2006;117:e903–8. https://doi.org/10.1542/peds.2005-1674.Search in Google Scholar PubMed
4. Schmidt, AMS, Larsen, SH, Hjortdal, VE. Vascular ring: early and long-term mortality and morbidity after surgical repair. J Pediatr Surg 2018;53:1976–9. https://doi.org/10.1016/j.jpedsurg.2017.12.022.Search in Google Scholar PubMed
5. Leonardi, B, Secinaro, A, Cutrera, R, Albanese, S, Trozzi, M, Franceschini, A, et al.. Imaging modalities in children with vascular ring and pulmonary artery sling. Pediatr Pulmonol 2015;50:781–8. https://doi.org/10.1002/pp.Search in Google Scholar
© 2022 Alexandra Byrne and Puneet Tung, published by De Gruyter, Berlin/Boston
This work is licensed under the Creative Commons Attribution 4.0 International License.
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Articles in the same Issue
- Frontmatter
- Cardiopulmonary Medicine
- Case Report
- Propionibacterium acnes: an uncommon cause of lung abscess in chronic obstructive pulmonary disease complicated with bullous emphysema
- General
- Case Report
- Reactivation of minimal change disease after Pfizer vaccine against COVID-19
- Innovations
- Original Article
- At-home ECG monitoring with a real-time outpatient cardiac telemetry system during the COVID-19 pandemic
- Medical Education
- Original Article
- Multisite assessment of emergency medicine resident knowledge of evidence-based medicine as measured by the Fresno Test of Evidence-Based Medicine
- Neuromusculoskeletal Medicine (OMT)
- Brief Report
- Osteopathic manipulative treatment use among family medicine residents in a teaching clinic
- Public Health and Primary Care
- Original Article
- Impact of an osteopathic peer recovery coaching model on treatment outcomes in high-risk men entering residential treatment for substance use disorders
- Clinical Images
- Recurrent bronchiolitis and stridor in an infant
- Bilateral idiopathic superior ophthalmic vein dilation
- Letters to the Editor
- Comments on “Reported completion of the USMLE Step 1 and match outcomes among senior osteopathic students in 2020”
- Response to “Comments on ‘Reported completion of the USMLE Step 1 and match outcomes among senior osteopathic students in 2020’”