Physical Address
304 North Cardinal St.
Dorchester Center, MA 02124
You’re surrounded: Vascular ring/sling
Case presentation
A 2-year-old female presents to the emergency department with wheezing and cough. The family states that she has had mild fever to 100.1 degrees Fahrenheit for the past 2 days. There has been no vomiting or diarrhea. She has been taking oral fluids well and is otherwise behaving at her baseline. Her physical examination reveals a well-appearing child in no respiratory distress. She has nasal congestion and some inspiratory wheezing at the upper lung fields.
The parents state they are frustrated and “want something for the cough and wheezing,” which has been intermittently present “as long as they can remember” and seems to get worse when the child has upper respiratory infections. They have seen their primary care provider multiple times for these symptoms and have been told the child has reactive airway disease. Albuterol was prescribed, but the family states that this medication is not helping the child’s wheezing. Multiple courses of oral steroids have been prescribed and the child has been given a fluticasone inhaler but this too has had no impact on her symptoms.
Imaging considerations
Plain radiography
Two-view chest radiography (anteroposterior [AP] and lateral views) is the first-line imaging modality in patients with respiratory symptoms and serves as a helpful screening examination for vascular rings. On AP views, one should evaluate the relationship of the aorta relative to the trachea, determining the side of the aortic arch, and one should evaluate for tracheal narrowing on the lateral view, which may suggest compression from a vascular ring or sling. A right-sided aortic arch may be associated with a variety of cardiac lesions (up to 50% of patients), including persistent truncus arteriosus, pulmonary atresia with ventricular septal defect, and tetralogy of Fallot. Anomalies of the aortic arch and the resulting aortic arch branching pattern and position of the ductus arteriosus will determine whether the vasculature forms a complete vascular ring, producing clinical symptoms. Two branching patterns of a right-sided aortic arch, a retroesophageal aberrant left subclavian artery (65%) and mirror-image branching (35%), can be seen, with the mirror-image branching type more commonly associated with congenital heart disease. A left-sided aortic arch with a an aberrant right subclavian artery may also be associated with congenital heart disease, but this configuration will form an incomplete vascular ring, thus producing fewer clinical symptoms compared to a right-sided arch configuration.
Findings suspicious for a vascular ring on AP images include a right-sided aortic arch, abnormal tracheal indentation, and a right descending aorta, whereas lateral radiographic findings include increased retrotracheal opacity, tracheal narrowing, and anterior tracheal bowing. , If a double aortic arch is present, the aorta may not be easily visualized. , Unilateral hyperinflation of the right lung, tracheal narrowing, and an atypical orientation of main bronchi suggest a pulmonary artery sling. Chest radiography has the advantage of availability, rapidity, and minimal ionizing radiation exposure. However, more advanced imaging techniques become necessary if an abnormality is suspected.
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