Anesthetic Management of a Child With Unspecified Mitochondrial Disease in an Outpatient Dental Setting

Abstract

Mitochondrial disease (MD) represents a category of metabolic disorders with a wide range of symptoms across a variety of organ systems. It occurs with an incidence of greater than 1:5000 and can be difficult to specifically diagnose because of the variety of clinical presentations and multiple genomic origins. Although phenotypically variable, MD symptoms often include hypotonia, cardiac defects, dysautonomia, and metabolic dysfunction. Mitochondrial disease presents a unique challenge in terms of anesthetic management, as many anesthetic drugs suppress mitochondrial function. Additional considerations may need to be made in order to evaluate the patient's metabolic compensation prior to surgery. This article presents an in-depth discussion of a case involving a nearly 10-year-old boy with a history of an unspecified form of MD, who presented for endodontic treatment of tooth No. 30 under deep sedation. The article also provides a thorough review of the current literature surrounding the anesthetic management of patients with MD.

Dental Treatment of a Child with Mitochondrial Myopathy Under General Anesthesia

“…There are several reports in the literature of this type of disease tolerant to a wide range of anesthetics including volatile anesthetics, propofol and local anesthetics. 6,7 In 2013, Gentili et al published a letter to the editor in which they described a severe case of mitochondrial myoptahy (MELAS syndrome: mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) that was operated for combined colectomy and ovariectomy under laparotomy with an intra-operative time of 5 hours. 7 The operation was performed under general anesthesia in combination with postoperative epidural anesthesia.…”