Extremely preterm infant with double aortic arch presenting with severe tracheal obstruction and devastating esophageal hemorrhage

Abstract

Background: Double aortic arch is the most constrictive type of vascular ring, completely encircling the trachea and esophagus, and can cause severe respiratory failure, dysphagia, or devastating esophageal hemorrhage. Although surgical repair is usually recommended shortly after diagnosis, surgery in extremely preterm infants presents significant challenges. The optimal timing of surgery and the management of complications that arise while awaiting surgery are not well understood.

Case presentation: We report the case of an Asian female infant weighing 509 g at 24 + 5 weeks of gestation with a double aortic arch. Initially managed conservatively to allow for growth, the patient developed progressive tracheal granulation and recurrent, life-threatening esophageal hemorrhage. Gastrostomy and division of the vascular ring were successfully performed on days 65 and 66 (postmenstrual age 33 weeks; weight 1126 g), and tracheal granulation was managed conservatively. Despite mild respiratory symptoms persisting, the patient survived until discharge without any recurrence of esophageal bleeding.

Conclusion: The management of double aortic arch in extremely preterm infants is particularly challenging. In this case, surgical repair was successfully performed at a weight of 1126 g without lethal postoperative complications. Early gastrostomy before the surgical repair of the double aortic arch may be advantageous in preventing devastating esophageal hemorrhage, especially in patients with severe tracheal narrowing indicative of a tight vascular ring and significant esophageal compression.