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. 2023 Jul 21:11:1227819.
doi: 10.3389/fped.2023.1227819. eCollection 2023.

Pediatric airway compression in aortic arch malformations: a multidisciplinary approach

Affiliations

Pediatric airway compression in aortic arch malformations: a multidisciplinary approach

Francesca Petreschi et al. Front Pediatr. .

Abstract

Background: Aortic arch malformations (AAMs) should be suspected in the presence of persistent respiratory symptoms despite medical treatment or feeding problems at the pediatric age.

Aim: We report a descriptive cohort of patients with AAMs and the local management protocol applied.

Methods: A total of 59 patients with AAM were retrospectively reviewed. Three groups were identified: double aortic arch (DAA), group 1; complete vascular ring (non-DAA), group 2; and anomalous origin of the innominate artery (IA), group 3.

Results: Prenatal diagnosis was available for 62.7% of the patients. In all, 49.2% of children were symptomatic. There was a significantly different prevalence of respiratory symptoms within the three groups: 73.7% in group 1, 24.2% in group 2, and 100% in group 3 (p-value: <0.001). Surgery was considered in the presence of symptoms in patients with DAA and in those with reduction of the tracheal section area greater than 50%. A total of 52.5% of the patients underwent surgical repair (median age 6 months). The median follow-up interval was 21.9 months. Respiratory symptoms improved in most symptomatic patients.

Conclusions: No specific protocols are available for the management of patients with AAMs. Conservative treatment seems to be reasonable for asymptomatic patients or those with airway stenosis less than 50%. A close follow-up is necessary to identify early patients who become symptomatic.

Keywords: airway compression; aortic arch malformations; stridor; surgical treatment; tracheomalacia.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
CT axial view showing double aortic arch with dominant right aortic arch (RAA) and hypoplastic left aortic arch (LLA) compressing the trachea.
Figure 2
Figure 2
CT axial view showing right aortic arch (RAA) with aberrant left subclavian artery and Kommerell's diverticulum compressing the trachea with estimated lumen reduction of 55%; left coronary artery (LCA).
Figure 3
Figure 3
CT axial (A) and coronal (B) views showing left aortic arch with normal branching pattern and innominate artery (IA) anteriorly and laterally compressing the trachea; LCA, left coronary artery; LSA, left subclavian artery.
Figure 4
Figure 4
Timing and need of surgery (expressed in months) in 31 children: Kaplan–Meier analysis.
Figure 5
Figure 5
Diagnostic and therapeutic approach to pediatric airway compression in aortic arch malformations adopted at Bambino Gesù Children's Hospital.

References

    1. Kocis KC, Midgley FM, Ruckman RN. Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair. Pediatr Cardiol. (1997) 18(2):127–32. 10.1007/s002469900130 - DOI - PubMed
    1. Woods RK, Sharp RJ, Holcomb GW, 3rd, Snyder CL, Lofland GK, Ashcraft KW, et al. Vascular anomalies and tracheoesophageal compression: a single institution’s 25-year experience. Ann Thorac Surg. (2001) 72(2):434–8; discussion 438–439. 10.1016/s0003-4975(01)02806-5 - DOI - PubMed
    1. Porcaro F, Ciliberti P, Petreschi F, Secinaro A, Allegorico A, Coretti A, Cutrera R. Long term respiratory morbidity in patients with vascular rings: a review. Ital J Pediatr. (2023) 49(1):24. 10.1186/s13052-023-01430-x - DOI - PMC - PubMed
    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(5):511–24. 10.1002/ppul.23152 - DOI - PubMed
    1. Tola H, Ozturk E, Yildiz O, Erek E, Haydin S, Turkvatan A, et al. Assessment of children with vascular ring. Pediatr Int. (2017) 59(2):134–40. 10.1111/ped.13101 - DOI - PubMed

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