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Vascular Rings

  • Benjamin O. BierbachEmail author
  • John Mark Redmond
Reference work entry

Abstract

Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus to form a complete or incomplete compressing ring around them. They make up less than 1% of all congenital cardiac defects. The most common forms are double aortic arch, right aortic arch, pulmonary artery sling, vascular rings associated with left aortic arch, and cervical aorta.

Patients often present with symptoms in the first few months of life and require surgery early in life. The classic sign is the “seal-bark” cough. In addition noisy breathing may be heard both during inspiration and expiration (biphasic stridor). A common finding in all forms of vascular rings is the increased occurrence of respiratory infections.

Diagnostic tests for visualization of the relevant pathology and the exact location of the obstruction include computerized tomography scan, magnetic resonance imaging, and digital subtraction angiography. Echocardiography is useful in the diagnostic workup of associated congenital cardiac defects.

In patients with pulmonary artery sling, repair is undertaken via median sternotomy utilizing cardiopulmonary bypass and reimplanting the left pulmonary artery onto the left side of the main pulmonary artery. If necessary, tracheal repair can be performed concurrently. All other lesions are usually approached via a left-sided thoracotomy. The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. In patients with right-sided aortic arch, the ligamentum arteriosum is divided. Division of the left subclavian artery is not generally necessary for relieving tracheal compression. Patients with an anomalous left subclavian artery and Kommerell’s diverticulum may additionally have resection of the diverticulum and reimplantation of the left subclavian artery to the left carotid artery as a primary operation.

Excellent results have been achieved in recent years without operative mortality in high-volume centers. Most of the patients are essentially free of symptoms in the long term, and only a very small number need further interventions or supportive treatment.

Keywords

Biphasic stridor Cervical aorta Double aortic arch Dysphagia Pulmonary artery sling Kommerell’s diverticulum Right-sided aortic arch Seal-bark cough 

Notes

Acknowledgement

Republished with permission of Taylor and Franciss Group LLC books, from Newborn Surgery, PD. Dr.med. Benjamin Bierbach and Professor Mark Redmond, Vascular rings, chapter 39, fourth edition, 2017; permission conveyed through Copyright Clearance Center, Inc.

References

  1. Anand R, Dooley KJ, Williams WH, Vincent RN. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol. 1994;15:58–61.PubMedGoogle Scholar
  2. Backer CL, Ilbawi MN, Idriss FS. SY DL. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. 1989;97:725–31.PubMedCrossRefGoogle Scholar
  3. Backer CL, Hillman N, Mavroudis C, Holinger LD. Resection of Kommerell’s diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division. Eur J Cardiothorac Surg. 2002;22:64–9.PubMedCrossRefGoogle Scholar
  4. Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. 2005;129:1339–47.PubMedCrossRefGoogle Scholar
  5. Backer CL, Hyde MR, Wurlitzer KC, Rastatter JC, Rigsby CK. Primary resection of Kommerell diverticulum and left subclavian artery transfer. Ann Thorac Surg. 2012;94(5):1612–7.PubMedCrossRefGoogle Scholar
  6. Backer CL, Monge MC, Popescu AR, et al. Vascular rings. Semin Pediatr Surg. 2016;25(3):165–75.PubMedCrossRefGoogle Scholar
  7. Bando K, Turrentine MW, Sun K, Sharp TG, Matt B, Karmazyn B, Heifetz SA, Stevens J, Kesler KA, Brown JW. Anterior pericardial tracheoplasty for congenital tracheal stenosis: intermediate to long-term outcomes. Ann Thorac Surg. 1996;62:981–9.PubMedCrossRefGoogle Scholar
  8. Beierlein W, Elliott MJ. Variations in the technique of slide tracheoplasty to repair complex forms of long-segment congenital tracheal stenoses. Ann Thorac Surg. 2006;82:1540–2.PubMedCrossRefGoogle Scholar
  9. Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA. Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol. 1995a;25:943–7.PubMedCrossRefGoogle Scholar
  10. Burke RP, Wernovsky G, van der Velde M, Hansen D, Castaneda AR. Video-assisted thoracoscopic surgery for congenital heart disease. J Thorac Cardiovasc Surg. 1995b;109:499–507; discussion 508PubMedCrossRefGoogle Scholar
  11. Congdon E. Transformation of the aortic arch system during the development of the human embryo. Contrib Embryol. 1922;14:47.Google Scholar
  12. Cordovilla Zurdo G, Cabo Salvador J, Sanz Galeote E, Moreno Granados F, Alvarez Diaz F. Anillos vasculares de origen aortico: experiencia quirurgica en 43 casos. Rev Esp Cardiol. 1994;47:468–75.PubMedGoogle Scholar
  13. Cotter CS, Jones DT, Nuss RC, Jonas R. Management of distal tracheal stenosis. Arch Otolaryngol Head Neck Surg. 1999;125:325–8.PubMedCrossRefGoogle Scholar
  14. Dayan SH, Dunham ME, Backer CL, Mavroudis C, Holinger LD. Slide tracheoplasty in the management of congenital tracheal stenosis. Ann Otol Rhinol Laryngol. 1997;106:914–9.PubMedCrossRefGoogle Scholar
  15. Edwards J. Anomalies of the derivatives of the aortic arch system. Med Clin N Am. 1948;32:925.PubMedCrossRefGoogle Scholar
  16. Elliott M, Roebuck D, Noctor C, McLaren C, Hartley B, Mok Q, Dunne C, Pigott N, Patel C, Patel A, Wallis C. The management of congenital tracheal stenosis. Int J Pediatr Otorhinolaryngol. 2003;67(Suppl 1):S183–92.PubMedCrossRefPubMedCentralGoogle Scholar
  17. Elliott M, Hartley BE, Wallis C, Roebuck D. Slide tracheoplasty. Curr Opin Otolaryngol Head Neck Surg. 2008;16:75–82.PubMedCrossRefPubMedCentralGoogle Scholar
  18. Glavecke H, Döhle W. Über eine seltene angeborene Anomalie der Pulmonalarterie. Munch Med Wochenschr. 1897;44:950.Google Scholar
  19. Gould SW, Rigsby CK, Donnelly LF, McCulloch M, Pizarro C, Epelman M. Useful signs for the assessment of vascular rings on cross-sectional imaging. Pediatr Radiol. 2015;45(13):2004–16; quiz 2002–3PubMedCrossRefPubMedCentralGoogle Scholar
  20. Grillo HC. Slide tracheoplasty for long-segment congenital tracheal stenosis. Ann Thorac Surg. 1994;58:613–9; discussion 619–21PubMedCrossRefPubMedCentralGoogle Scholar
  21. Grimmer JF, Herway S, Hawkins JA, Park AH, Kouretas PC. Long-term results of innominate artery reimplantation for tracheal compression. Arch Otolaryngol Head Neck Surg. 2009;135:80–4.PubMedCrossRefPubMedCentralGoogle Scholar
  22. Gross R. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med. 1945;233:586–90.PubMedCrossRefPubMedCentralGoogle Scholar
  23. Gross RE, Neuhauser EB. Compression of the trachea by an anomalous innominate artery; an operation for its relief. Am J Dis Child. 1948;75:570–4.PubMedCrossRefPubMedCentralGoogle Scholar
  24. Hawkins JA, Bailey WW, Clark SM. Innominate artery compression of the trachea. Treatment by reimplantation of the innominate artery. J Thorac Cardiovasc Surg. 1992;103:678–82.PubMedCrossRefPubMedCentralGoogle Scholar
  25. Herberhold C, Stein M, von Falkenhausen M. Langzeitresultate von Homograftrekonstruktionen der Trachea im Kindesalter. Laryngorhinootologie. 1999;78:692–6.PubMedCrossRefPubMedCentralGoogle Scholar
  26. Hiller HG, Maclean AD. Pulmonary artery ring. Acta Radiol. 1957;48:434–8.PubMedCrossRefPubMedCentralGoogle Scholar
  27. Hommel TW. On irregularities of the pulmonary artery, arch of the aorta and primary branches of the arch with an attempt to illustrate their mode of origin by a reference to development. Br Foreign Med Chir Rev. 1962;30:173.Google Scholar
  28. Idriss FS, DeLeon SY, Ilbawi MN, Gerson CR, Tucker GF, Holinger L. Tracheoplasty with pericardial patch for extensive tracheal stenosis in infants and children. J Thorac Cardiovasc Surg. 1984;88:527–36.PubMedCrossRefGoogle Scholar
  29. Jonas RA, Spevak PJ, McGill T, Castaneda AR. Pulmonary artery sling: primary repair by tracheal resection in infancy. J Thorac Cardiovasc Surg. 1989;97:548–50.PubMedCrossRefGoogle Scholar
  30. Kirklin J, Barratt-Boyes B. Cardiac surgery. Chichester: Wiley; 1986.Google Scholar
  31. Kogon BE, Forbess JM, Wulkan ML, Kirshbom PM, Kanter KR. Video-assisted thoracoscopic surgery: is it a superior technique for the division of vascular rings in children? Congenit Heart Dis. 2007;2:130–3.PubMedCrossRefGoogle Scholar
  32. Kommerell B. Verlagerung durch eine abnorm verlaufende Arteria subclavia dextra (Arteria lusoria). Fortschr Geb Rontgenstr. 1936;54:590.Google Scholar
  33. Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg. 2005;71:289–91.PubMedPubMedCentralGoogle Scholar
  34. Leonardi B, Secinaro A, Cutrera R, et al. Imaging modalities in children with vascular ring and pulmonary artery sling. Pediatr Pulmonol. 2015;50(8):781–8.PubMedCrossRefPubMedCentralGoogle Scholar
  35. Licari A, Manca E, Rispoli GA, et al. Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonol. 2015;50(5):511–24.PubMedCrossRefPubMedCentralGoogle Scholar
  36. Moes CA, Izukawa T, Trusler GA. Innominate artery compression of the trachea. Arch Otolaryngol. 1975;101:733–8.PubMedCrossRefPubMedCentralGoogle Scholar
  37. Naimo PS, Fricke TA, Donald JS, et al. Long-term outcomes of complete vascular ring division in children: a 36-year experience from a single institution. Interact Cardiovasc Thorac Surg. 2017;24(2):234–9.PubMedPubMedCentralGoogle Scholar
  38. Neuauser E. The roentgen diagnosis of double aortic arch and other anomalies of the great vessels. Am J Roentgenol. 1946;56:1.Google Scholar
  39. Potts WJ, Holinger PH, Rosenblum AH. Anomalous left pulmonary artery causing obstruction to right main bronchus: report of a case. J Am Med Assoc. 1954;155:1409–11.PubMedCrossRefPubMedCentralGoogle Scholar
  40. Riggle KM, Rice-Townsend SE, Waldhausen JHT. Thoracoscopic division of vascular rings. J Pediatr Surg. 2017;52(7):1113–6.PubMedCrossRefPubMedCentralGoogle Scholar
  41. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg. 2009;44:1328–32.PubMedCrossRefPubMedCentralGoogle Scholar
  42. Schlosshauer B. Zur Verhütung und Behandlung von Kehlkopf- und Luftröhrenstenosen im Kindesalter. HNO. 1975;23:342–4.PubMedPubMedCentralGoogle Scholar
  43. Sebening C, Jakob H, Tochtermann U, Lange R, Vahl CF, Bodegom P, Szabo G, Fleischer F, Schmidt K, Zilow E, Springer W, Ulmer HE, Hagl S. Vascular tracheobronchial compression syndromes – experience in surgical treatment and literature review. Thorac Cardiovasc Surg. 2000;48:164–74.PubMedCrossRefPubMedCentralGoogle Scholar
  44. Shinkawa T, Greenberg SB, Jaquiss RDB, Imamura M. Primary translocation of aberrant left subclavian artery for children with symptomatic vascular ring. Ann Thorac Surg. 2012;93:1262–5.PubMedCrossRefPubMedCentralGoogle Scholar
  45. Stewart J, Kincaid O, Edwards J. An atlas of vascular rings and related malformations of the aortic arch system. Springfield: Charles C. Thomas; 1964.Google Scholar
  46. Tola H, Ozturk E, Yildiz O, et al. Assessment of children with vascular ring. Pediatr Int. 2016;59:134.  https://doi.org/10.1111/ped.13101. [Epub ahead of print].CrossRefPubMedPubMedCentralGoogle Scholar
  47. Tsang V, Murday A, Gillbe C, Goldstraw P. Slide tracheoplasty for congenital funnel-shaped tracheal stenosis. Ann Thorac Surg. 1989;48:632–5.PubMedCrossRefPubMedCentralGoogle Scholar
  48. van Son JA, Julsrud PR, Hagler DJ, Sim EK, Pairolero PC, Puga FJ, Schaff HV, Danielson GK. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc. 1993;68:1056–63.PubMedCrossRefPubMedCentralGoogle Scholar
  49. Wolman I. Congenital stenosis of the trachea. Am J Dis Child. 1941;61:1263.Google Scholar
  50. Yilmaz M, Ozkan M, Dogan R, Demircin M, Ersoy U, Boke E, Pasaoglu I. Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management. Heart Surg Forum. 2003;6:149–52.PubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of Paediatric Cardiac SurgeryGerman Paediatric Heart Center Sankt AugustinSankt AugustinGermany
  2. 2.Our Lady’s Children’s’ HospitalDublinIreland
  3. 3.Mater Misericordiae University HospitalDublinIreland

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