2018 Volume 46 Issue 2 Pages 80-82
Leigh encephalopathy (LE) is a clinical subtype of mitochondrial encephalomyopathy characterized by delayed development of psychomotor functions and regression from infancy. Problems associated with the anesthetic management of patients with mitochondrial disease include the possibility of malignant hyperthermia, the prolongation of muscle relaxation, and lactic acidosis.
A 7-year-old boy with LE had dental caries in his right mandibular first molar. He was uncooperative with dental treatment, and a dental procedure under intravenous sedation (IVS) was planned. Laboratory data including blood tests, an electrocardiogram, a chest X-ray, and chest auscultation did not show any abnormalities. We planned an anesthetic management without the use of propofol in consideration of the possibility of propofol infusion syndrome. Therefore, IVS with dexmedetomidine hydrochloride (DEX) in combination with midazolam (MDZ) was selected.
We initially administered 1 mg of MDZ and loaded DEX at a dose of 3 μg/kg/h over 10 min, subsequently reducing to 0.5 μg/kg/h for maintenance. Additional doses of MDZ were administered so that the OAA/S score was maintained between 2 and 4. During the procedure, the patient’s vital signs were stable, and no signs of respiratory depression were observed.
In conclusion, IVS with DEX in combination with MDZ was useful for the dental treatment of a patient with LE. Further studies are needed to determine the optimal doses of DEX and MDZ.
