Vascular rings and slings

Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. 

Epidemiology

Vascular rings are rare, occurring in <1% of patients ¹. No gender or ethnic predispositions have been identified ³. 

Clinical presentation

Many vascular rings and slings are asymptomatic although they can present in pediatric patients with wheezing/stridor, recurrent respiratory infections, and/or dysphagia lusoria. 

Pathology

Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic) ⁵. 

Etiology

Causes of vascular rings and slings commonly include ¹:

• double aortic arch (the most common symptomatic vascular ring)

• incomplete double aortic arch

• right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum

• aberrant right subclavian artery (most common)

  • ​not considered a complete vascular ring

  • usually asymptomatic

• pulmonary sling

Associations

• conotruncal cardiac anomalies ²

Radiographic features

Radiographic features will differ depending on the underlying anomaly.

Plain radiograph

Abnormal contour to the trachea may be visible on plain film, as may a right-sided aortic arch.

Fluoroscopy

On contrast esophagogram (and air tracheogram), the pattern of indentations on the frontal and lateral views helps to narrow the differential:

• anterior esophageal indentation, posterior tracheal indentation

  • pulmonary sling (anomalous left pulmonary artery)

• posterior esophageal indentation, tracheal buckling to the right

  • aberrant right subclavian artery

• posterior esophageal indentation, tracheal buckling to the left

  • aberrant left subclavian artery with right aortic arch

• anterior tracheal indentation in infants

  • innominate artery compression syndrome

• posterior and bilateral esophageal indentation, anterior and bilateral tracheal indentation

  • double aortic arch

CT/MRI

• allow precise demonstration of the anomaly

Differential diagnosis

Possible considerations include:

• congenital tracheomalacia

See also 

• other vascular syndromes

• vascular compression disorders