Right Aortic Arch, Kommerell Diverticulum, and Symptomatic Retro-Esophageal Vascular Ring in an Adult (Case Presentation and Review)

Abstract

A 24 year-old Hispanic man presented with progressive retrosternal chest pain, shortness of breath, and dysphagia. Chest X-ray, computed tomography angiography, and magnetic resonance (MRA) studies were compatible with a right aortic arch and a 3.5-cm descending right aortic Kommerell diverticulum. Barium esophagogram and esophagoscopy demonstrated a constricting band crossing the esophagus at the level of the diverticulum. After discussion with other cardiothoracic surgeons, with a combined 300 years of experience, they reported only 1 other possible such case. Kommerell's diverticula are usually repaired in children and rarely are seen in the adult. Kommerell diverticular treatment options include endovascular stenting, cardiopulmonary bypass with resection and grafting, right thoracotomy, and a cervical approach. Because of the patient's findings of a right arch, a Kommerell diverticulum, and a constricting band causing esophageal compression and respiratory distress, it was felt stenting alone would not relieve his symptoms. Therefore, we used a left third posterior intercostal thoracotomy approach to resect the constricting esophageal band connecting the Kommerell diverticulum to the left subclavian artery and to perform the Kommerell diverticulectomy. Releasing the esophageal and tracheal compressing band and resection of the diverticulum provided complete symptom relief with elimination of the dysphagia, dyspnea, wheezing, and chest pain, using a single surgical procedure.