Background
Leigh syndrome is a mitochondrial disorder in which impaired oxidative phosphorylation causes progressive neurologic damage. Brainstem lesions may cause central hypoventilation, apnea, and recurrent pulmonary infection, increasing perioperative risk.
Case
We describe a patient with Leigh syndrome who had chronic central respiratory dysfunction, recurrent pneumonia, and a recent intensive care admission for sepsis and who subsequently underwent tracheostomy under general anesthesia. Anesthesia was induced and maintained with remimazolam and remifentanil. Hemodynamic parameters remained acceptable neuromuscular recovery, spontaneous ventilation remained inadequate, and the patient was returned to the intensive care unit with ventilatory support.
Conclusion
In mitochondrial disease, propofol warrants careful consideration because of concerns regarding mitochondrial effects and propofol infusion syndrome. Remimazolam was selected based on severe respiratory vulnerability, recent multiorgan illness, and the anticipated need for postoperative ventilatory support, rather than as evidence of superiority over other agents.