History and Physical Examination
Symptoms and physical findings produced by vascular rings are primarily those of airway or esophageal compression. Individuals with a narrow or tight ring have a significant degree of constriction of one or both of these structures and present very early in life.
The vast majority of patients with a vascular ring present with symptoms in infancy or very early in childhood. [13] However, a small number of patients do not manifest symptoms until later in life, and others remain entirely asymptomatic. [14] Common symptoms include the following:
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Wheezing
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Respiratory distress
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Apnea
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Characteristic high-pitched, brassy cough
Additional findings include the following:
In some cases, airway symptoms are worsened or aggravated by feedings. Intercostal retractions during respiration are observed in some infants with severe obstruction. Others may try to maintain a position in which the head is hyperextended to improve breathing and minimize the obstruction. Air-trapping and evidence of pulmonary hyperinflation may also be present in one or both lungs in severe cases.
Symptoms of airway obstruction predominate in patients who present in infancy or the first few years of life. Dysphagia and symptoms related to the esophagus are the more likely presenting findings in older children and adults with vascular rings. Esophageal compression is usually posterior. Symptoms that are present soon after birth may include slow breast or bottle feeding, fatigue with feeding, frequent regurgitation, and aspiration pneumonias. In most cases, workup is initiated when solid foods are introduced, which causes more pronounced dysphagia.
The double aortic arch is the anomaly that usually produces the most severe airway compression in the youngest patients. The second most common configuration, right aortic arch and left ligamentum arteriosum with retroesophageal left subclavian artery, displays a spectrum of severity. Some individuals present in infancy or very early in childhood, whereas others do not present until adulthood.
Although patients with associated cardiac lesions may have additional symptoms secondary to that abnormality, symptoms of airway compression related to the constricting vascular ring are usually most prominent.
Finally, it should be kept in mind that some patients with a complete vascular ring have minimal symptoms or remain asymptomatic. In such cases, the abnormal arch anatomy is often discovered incidentally when the patient is undergoing diagnostic studies for another problem.
Conversely, if left aortic arch with associated retroesophageal right subclavian artery is incidentally found during diagnostic studies for symptoms of dysphagia, the clinician should not assume that this vascular abnormality, which is not a true vascular ring, is the source of the symptoms. Additional investigation for the true cause should be pursued. This arch abnormality, previously believed to be responsible for dysphagia symptoms and for which the term dysphagia lusoria was coined long ago, is rarely responsible for esophageal symptoms.
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Slide tracheoplasty and left pulmonary artery sling repair. Procedure performed by Giles Peek MD, FRCS, CTh, FFICM, The Children’s Hospital at Montefiore, Bronx, NY.
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Embryonic aortic arch with dorsal and ventral arches and 6 branchial arches on right and left.
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Double aortic arch.
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Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum.
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Right aortic arch with mirror-image branching and retroesophageal ligamentum arteriosum.
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Left aortic arch with right descending aorta and right ligamentum arteriosum.
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Retroesophageal right subclavian artery with left aortic arch and left ligamentum arteriosum.
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Aberrant left pulmonary artery or pulmonary artery sling.
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Barium esophagogram of 30-year-old woman with vascular ring.
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Single-stage correction of transposition of great arteries with ventricular septal defect, hypoplastic right aortic arch with bilateral ductuses, and aberrant left subclavian artery arising from left duct in newborn. Procedure performed by Giles Peek MD, FRCS, CTh, FFICM, The Children’s Hospital at Montefiore, Bronx, NY.
