Case Presentation: A 28-year-old male with a history of hiatal hernia presented with progressive dysphagia to solids and liquids over several weeks. His symptoms worsened to the point where he was reliant on a blended foods and liquid diet. Initial evaluation with a barium swallow demonstrated extrinsic compression of the upper thoracic esophagus. Subsequent CT angiography revealed a complete double aortic arch forming a vascular ring compressing the esophagus. Endoscopy confirmed extrinsic pulsatile compression approximately 25 cm from the incisors. Balloon dilation provided minimal symptomatic relief. The patient underwent a left posterolateral thoracotomy via the fourth intercostal space, with successful ligation and division of the non-dominant left aortic arch. Intraoperative intercostal cryoablation (T3–T7) was performed. His postoperative course was uncomplicated. He was discharged home on postoperative day 3. On follow-up, the patient has had total resolution of his symptoms.
Discussion: Double aortic arch (DAA) is a congenital anomaly where the right and left aortic arches persist, forming a complete vascular ring that can compress both structures and cause symptoms of airway or esophageal obstruction (1).In this case, the patient presented with progressive dysphagia to solids and liquids without respiratory compromise. The differential diagnosis for dysphagia includes intrinsic esophageal causes such as strictures, achalasia, diverticula, webs, and malignancy, as well as extrinsic compressive lesions such as mediastinal masses (5). These possibilities were excluded through imaging and endoscopic evaluation. Endoscopy revealed pulsatile external compression without an intraluminal lesion, ruling out intrinsic disease. The barium swallow was concerning external compression, while CT angiography confirmed a complete double aortic arch forming a vascular ring around the esophagus. Supportive management remains an integral part of management prior to surgical correction. Management includes monitoring for airway compromise, modifying the diet, and treating secondary complications such as aspiration (1, 6). The definitive treatment for DAA is surgical division of the non-dominant arch to relieve esophageal or tracheal compression (1, 3, 7). Intercostal cryoablation is done to aid in postoperative pain control. The patient had an uncomplicated recovery and complete resolution of symptoms. In a retrospective review, surgical correction of vascular rings was associated with 97 % symptom resolution and minimal recurrence (4). Prognosis after surgical repair is excellent (2, 4). Most patients experience improvement in swallowing and respiratory function (8).
Conclusions: Overall, our case emphasizes that vascular anomalies such as DAA, though rare in adults, should remain a key consideration when evaluating unexplained dysphagia. Early diagnosis and prompt surgical intervention result in an excellent long-term outcome.