UC Irvine

Anesthetic management of patients with mitochondrial disorders is challenging due to their rare nature, and difficulties to develop evidence-based protocols for general anesthesia. We report a case of a 52-year-old male with hypertension, congenital deafness, optic neuritis, hypogonadism and myotonia, who developed severe refractory hypotension and bradycardia, and intractable severe lactic acidosis during induction of general anesthesia with fentanyl, propofol, and succinylcholine. The surgery had to be postponed and supportive management started. After extensive work-up, the patient was diagnosed with probable mitochondrial dysfunction. Patient returned for surgery under a different anesthetic regimen consisting of ketamine, fentanyl, ephedrine and dexmedetomidine; yielding a successful perioperative course and highlighting potential alternative strategies to manage anesthesia in patients with such disorders.