Aortopexy for Tracheobronchomalacia in a Patient With Severe Motor and Intellectual Disabilities

Abstract

Aortopexy is a widely used technique for tracheomalacia associated with congenital esophageal atresia and tracheoesophageal fistula. We report the case of tracheobronchomalacia in a patient with severe motor and intellectual disabilities (SMIDs), who was successfully treated by aortopexy. The patient was a 20-year-old man with SMIDs due to hypoxic encephalopathy caused by status asthmaticus at the age of eight months. After undergoing gastrostomy and laryngotracheal separation, he had been on home ventilator support. Dying spells due to tracheobronchomalacia occurred at the age of 18. Contrast-enhanced computed tomography showed that the most stenotic part caused by tracheobronchomalacia coincided with the site of compression by the aortic arch and the vertebral body. Tube stenting was performed using a tracheal cannula, but the right main bronchus remained stenotic owing to aortic compression. He continued to have frequent dying spells and underwent aortopexy at the age of 20. Two years postoperatively, at the time of writing, his respiratory status is stable, and the postoperative course has been uneventful with no recurrence of dying spells. Aortopexy can be an effective treatment option for tracheomalacia caused by aortic compression in patients with SMIDs.