Abstract
This report describes a case of a 15-year-old pediatric patient with mitochondrial depletion syndrome, associated with hypertrophic cardiomyopathy, who underwent scoliosis correction surgery under neurophysiological monitoring. As propofol is mitochondrion-toxic, the anesthetic approach was modified to involve dexmedetomidine infusion combined with boluses of midazolam and ketamine. The patient was hemodynamically stable during surgery with effective neuromonitoring readings, and the surgery was concluded uneventfully. This case highlights the safety and efficacy of this anesthetic regimen in managing patients with complex metabolic and cardiac conditions.