Anesthetic management of patient with myoclonic epilepsy with ragged red fibers, a mitochondrial disease - A case report.

Abstract

Background: Myoclonic epilepsy with ragged red fibers (MERRF) is a rare mitochondrial disorder caused by pathogenic mutations in mitochondrial DNA. Anesthetic management in these patients is challenging because, commonly used anesthetic agents may exacerbate mitochondrial dysfunction and increase the risk of perioperative respiratory, cardiac, and metabolic complications.

Case: A 44-year-old woman with MERRF underwent a unilateral salpingo-oophorectomy. General anesthesia was induced applying low-dose midazolam, remifentanil and rocuronium, and maintained with desflurane and remifentanil. Perioperative management focused on minimizing metabolic stress through the maintenance of normothermia, avoidance of lactate-containing solutions, and close hemodynamic and respiratory monitoring. The intraoperative and immediate postoperative courses were uneventful.

Conclusions: This case highlights that safe anesthetic care in patients with MERRF can be achieved through comprehensive preoperative evaluation, cautious selection and dosing of anesthetic agents, strict metabolic control, and perioperative monitoring. Hence, individualized anesthetic strategies are essential to optimize perioperative outcomes in patients with mitochondrial disorders.