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Left aortic arch and aberrant right subclavian artery in children: spectrum of symptoms and response to surgical intervention

Published online by Cambridge University Press:  18 October 2024

Lianne Cole Open the ORCID record for Lianne Cole [Opens in a new window] ,
Jennifer Brown ,
Jessica Yasuda ,
Peter Ngo ,
Steven J. Staffa ,
Naomi Crilley ,
Peter Chiu Open the ORCID record for Peter Chiu [Opens in a new window] ,
Benjamin Zendejas  and
Christopher Baird
Lianne Cole*
Affiliation:
Department of Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
Jennifer Brown
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
Jessica Yasuda
Affiliation:
Department of Gastroenterology, Boston Children’s Hospital, Boston, Massachusetts, USA
Peter Ngo
Affiliation:
Department of Gastroenterology, Boston Children’s Hospital, Boston, Massachusetts, USA
Steven J. Staffa
Affiliation:
Department of Anesthesiology, Boston Children’s Hospital, Boston, USA
Naomi Crilley
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
Peter Chiu
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
Benjamin Zendejas
Affiliation:
Department of Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
Christopher Baird
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA
*
Corresponding author: L. Cole; Email: [email protected]
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Abstract

Background:

Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery.

Materials and methods:

This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022. Primary outcome was symptom improvement. Pre- and post-operative questionnaires were used to assess dysphagia (PEDI-EAT-10) and respiratory symptoms (PEDI-TBM-7). Paired t-test and Fisher’s exact test were used to analyse symptom resolution. Secondary outcomes included perioperative outcomes, complications, and length of stay.

Results:

Twenty children, median age 2 years (IQR 1–11), were included. All presented with swallowing symptoms, and 14 (70%) also experienced respiratory symptoms. Statistically significant improvements in symptoms were reported for both respiratory and swallowing symptoms. Paired (pre- and post-op) PEDI-EAT-10 and PEDI-TBM-7 scores were obtained for nine patients, resulting in mean (± SD) scores decreasing (improvement in symptoms) from 19.9 (± 9.3) to 2.4 (± 2.5) p = 0.001, and 8.7 (± 4.7) to 2.8 (± 4.0) p = 0.006, respectively. Reoperation was required in one patient due to persistent dysphagia from an oesophageal stricture. Other complications included lymphatic drainage (n = 4) and transient left vocal cord hypomobility (n = 1).

Conclusion:

Children with a left aortic arch with aberrant right subclavian artery can present with oesophageal and respiratory symptoms beyond solid food dysphagia. A thorough multidisciplinary evaluation is imperative to identify patients who can benefit from surgical repair, which appears to be safe and effective.

Keywords

Vascular ringdysphagiatracheobronchomalaciaaberrant right subclavian artery
Type
Original Article
Information
Cardiology in the Young , Volume 34 , Issue 12 , December 2024 , pp. 2663 - 2669
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Copyright
© The Author(s), 2024. Published by Cambridge University Press

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References

Nelson, JS, Hurtado, CG, Wearden, PD. Surgery for dysphagia lusoria in children. Ann Thorac Surg 2020; 109: e131–e133.CrossRefGoogle ScholarPubMed
Epperson, MV, Howell, R. Dysphagia lusoria: problem or incidentaloma? Curr Opin Otolaryngol Head Neck Surg 2019; 27: 448–452.CrossRefGoogle ScholarPubMed
Polguj, M, Chrzanowski, Ł., Kasprzak, JD et al.The aberrant right subclavian artery (arteria lusoria): the morphological and clinical aspects of one of the most important variations—a systematic study of 141 reports. TheScientificWorld 2014; 2014: 292734–292736.Google ScholarPubMed
Coles, M, Sharma, A. Dysphagia lusoria: Is the dysmotility connection illusory or real? Dig Dis Sci 2020; 65: 942–945.CrossRefGoogle ScholarPubMed
Dranseika, V, Erdil, T, Schweiger, M, Balmer, C, Pretre, R, Dave, H. Dysphagia and an aberrant subclavian artery: more than just a coincidence. Interact Cardiovasc Thorac Surg, 2020; 31: 228–231.CrossRefGoogle Scholar
Chiu, P, Zendejas, B, Baird, C. Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review. Transl Pediatr 2023; 12: 1258–1277.CrossRefGoogle ScholarPubMed
Serel Arslan, S, Demir, N, Karaduman, AAşe, Belafsky, PC. The pediatric version of the eating assessment tool: a caregiver administered dyphagia-specific outcome instrument for children. Disabil Rehabil 2018; 40: 2088–2092.CrossRefGoogle Scholar
Kamran, A, Zendejas, B, Jennings, RW. Current concepts in tracheobronchomalacia: diagnosis and treatment. Semin Pediat Surg 2021; 30: 151062.CrossRefGoogle ScholarPubMed
Sripriya, R, Prabavathy, G. Horner’s syndrome following thoracic paravertebral block-radiological evidence of spread to stellate ganglion and fascial anatomy facilitating the spread. Asian J Anesthesiol 2022; 60: 87–88.Google ScholarPubMed
Choi, S, Lawlor, C, Rahbar, R, Jennings, R. Diagnosis, classification, and management of pediatric tracheobronchomalacia: a review. JAMA Otolaryngol Head Neck Surg, 2019; 145: 265–275.CrossRefGoogle ScholarPubMed
Labuz, DF, Kamran, A, Jennings, RW, Baird, CW. Reoperation to correct unsuccessful vascular ring and vascular decompression surgery. J Thorac Cardiovasc Surg 2022; 164: 199–207. DOI: 10.1016/j.jtcvs.2021.08.089.CrossRefGoogle ScholarPubMed
Tallarita, T, Rogers, RT, Bower, TC et al. Characterization and surgical management of aberrant subclavian arteries. J Vasc Surg 2023; 77: 1006–1015.CrossRefGoogle ScholarPubMed
Nguyen, D, Leon, LR, Berman, SS. Hybrid repair of aberrant right subclavian artery using open and endovascular techniques. J Vasc Surg Cases Innov Tech 2023; 9: 101307.CrossRefGoogle ScholarPubMed
Shieh, HF, Smithers, CJ, Hamilton, TE et al. Posterior tracheopexy for severe tracheomalacia. J Pediatr Surg 2017; 52: 951–955.CrossRefGoogle ScholarPubMed
Torre, M, Reali, S, Rizzo, F et al. Posterior tracheopexy for tracheomalacia: study of clinical and radiological consequences on esophagus. Eur J Pediatr Surg 2023; 34: 306–313.Google ScholarPubMed
Griffeth, EM, Stephens, EH, Dearani, JA et al. Outcomes of surgical repair of aberrant subclavian arteries in adults. Ann Thorac Surg 2024; 117: 396–402.CrossRefGoogle ScholarPubMed
Svetanoff, WJ, Zendejas, B, Frain, L et al. When to consider a posterolateral descending aortopexy in addition to a posterior tracheopexy for the surgical treatment of symptomatic tracheobronchomalacia. J Pediatr Surg 2020; 55: 2682–2689. DOI: 10.1016/j.jpedsurg.2020.04.018.CrossRefGoogle ScholarPubMed
Bath, J, D’Oria, M, Rogers, RT et al. Contemporary outcomes after treatment of aberrant subclavian artery and kommerell’s diverticulum. J Vasc Surg 2023; 77: 1339–1348.e6.CrossRefGoogle ScholarPubMed