Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

Vascular rings—presentation, investigation and outcome

  • 638 Accesses

  • 63 Citations


Our aim was to determine the presentation of patients with vascular rings and evaluate the effectiveness of investigations. Surgical outcomes and respiratory sequelae were also examined. The design was a retrospective case note study over a 13-year period set in a tertiary children’s hospital. Children below the age of 16 years presenting with a vascular ring to the Royal Hospital for Sick Children, Glasgow were studied. Demographic data at presentation, including symptoms, were recorded. The ability of diagnostic investigations to identify the presence of a vascular ring was evaluated. Surgical outcomes were determined by measuring surgical complications and mortality. Respiratory sequelae were recorded by the presence of persistent symptoms or the need for tracheostomy or long-term ventilation following surgery. A total of 24 patients were identified with a median age at presentation of 4.5 months. Stridor was the commonest presenting symptom (14/24). Angiography, chest CT scanning and MRI were the most accurate imaging modalities (accurate in 100% of cases used). Chest X-ray films and echocardiography had the lowest detection rates. Surgical complications (4/24) and mortality (1/24) were low. A substantial number of patients available to follow-up (7/20) were still experiencing stridor 3 months post-operatively. Conclusion:Vascular rings are rare, however, often present with common symptoms. Most children present in early infancy, but a minority presents much later. The investigation of choice is a barium swallow followed by high-resolution computed tomography. Surgery is safe although a number of patients will have persisting symptoms.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5


  1. 1.

    Bakker DAH, Berger RMF, Witsenburg M, Bogers AJJC. (1999) Vascular rings: a rare cause of common respiratory symptoms. Acta Paediatr 88: 947–952

  2. 2.

    Chun K, Colombani PM, Dudgeon DL, Haller JA Jr (1992) Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thoraci Surg 53: 597–602

  3. 3.

    Klinkhamer AC (1969) Esophagography in anomalies of the aortic arch system. Williams and Wilkins, Baltimore

  4. 4.

    Lillehei CW, Colan S (1992) Echocardiography in the preoperative evaluation of vascular rings. J Pediatr Surg 27: 1118–1121

  5. 5.

    Lowe GM, Donaldson JS, Backer CL (1991) Vascular rings: 10-year review of imaging. Radiographics 11: 637–646

  6. 6.

    Stewart JR, Kincaid OW, Edwards JE (1964) An atlas of vascular rings and related malformations of the aortic system. Charles C Thomas, Springfield, p 111

  7. 7.

    Valleta EA, Pregarz M, Bergamo-Andreis IA, Boner AL (1997) Tracheoesophageal compression due to congenital vascular anomalies (vascular rings). Pediatr Pulmonol 24: 93–105

  8. 8.

    Van Son JA, Julsrud PR, Hagler DJ (1993) Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clinic Proc 68: 1131–1133

Download references

Author information

Correspondence to Alastair Turner.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Turner, A., Gavel, G. & Coutts, J. Vascular rings—presentation, investigation and outcome. Eur J Pediatr 164, 266–270 (2005).

Download citation


  • Congenital heart disease
  • Double aortic arch
  • Echocardiography
  • Neonatal respiratory distress
  • Vascular rings