Question

A newborn presents with stridor and respiratory distress and dysphagia. Imaging shows compression of the trachea, esophagus and right main bronchus. Which vascular anomaly is most likely responsible?

• A. Right-sided aortic arch
• B. Double aortic arch
• C. Pulmonary sling (anomalous left pulmonary artery)
• D. Patent ductus arteriosus

Hint:

Vascular Ring Key Points:
- Double Aortic Arch: Most common complete ring; compresses both anterior trachea and posterior esophagus (stridor + dysphagia).
- Pulmonary Sling: Only vascular anomaly that courses BETWEEN trachea and esophagus (compresses posterior trachea and anterior esophagus).

Answer 1

The Correct Option is B

Concept:
Vascular rings are congenital malformations of the aortic arch complex that completely or incompletely encircle and compress the trachea and esophagus, causing variable degrees of upper airway obstruction (stridor, wheezing) and gastrointestinal obstruction (dysphagia, feeding difficulties).
Explanation:
• Double aortic arch is the most common complete vascular ring and the most frequent cause of symptomatic vascular airway and esophageal compression in early infancy.

• It results from the failure of regression of the right fourth pharyngeal arch artery, resulting in two separate functioning aortic arches (a larger right-sided posterior arch and a smaller left-sided anterior arch) forming a tight vascular ring around the trachea and esophagus.

• Because the ring completely encircles both structures, infants classically present very early in life with both respiratory symptoms (inspiratory/expiratory stridor that worsens during feeding or in supine position) and digestive symptoms (dysphagia, choking, regurgitation during swallowing, termed dysphagia lusoria).

• Imaging in double aortic arch typically demonstrates bilateral and circumferential compression of the trachea and bilateral indentation of the esophagus (posterior compression on barium swallow) along with right main bronchus compression.

• A pulmonary sling (anomalous left pulmonary artery arising from the right pulmonary artery) courses between the trachea and esophagus, compressing the anterior esophagus and posterior trachea (and right main bronchus), but does not form a complete bilateral circumferential aortic ring.
Final Answer:
A double aortic arch forms a complete vascular ring around the mediastinal structures, causing simultaneous compression of the trachea, esophagus, and right main bronchus with early stridor and dysphagia.