==== Front J Anaesthesiol Clin PharmacolJ Anaesthesiol Clin PharmacolJOACPJournal of Anaesthesiology, Clinical Pharmacology0970-91852231-2730Medknow Publications & Media Pvt Ltd India JOACP-34-26910.4103/0970-9185.173379Letters to EditorAnesthetic management of a patient with MELAS Thampi Suma Mary Srinivasan Chitra George Gladdy Davis Kirubakaran Department of Anaesthesia, Christian Medical College and Hospital, Vellore, Tamil Nadu, IndiaAddress for correspondence: Dr. Suma Mary Thampi, Department of Anaesthesia, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu, India. E-mail: sumadavid@gmail.comApr-Jun 2018 34 2 269 271 Copyright: © 2018 Journal of Anaesthesiology Clinical Pharmacology2018This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. ==== Body Madam, Mitochondrial diseases (MDs) have an incidence of 1:4000 live births.[1] With advancing diagnostic and treatment facilities, increasing number of patients present for anesthesia for diagnostic procedures or palliative surgeries. MELAS is a subgroup of MDs characterized by mitochondrial encephalomyopathy, lactic acidosis, and stroke. These diseases have variable clinical presentation with multisystem involvement [Table 1]. Although clues to the disease may manifest in early years, most cases become clinically symptomatic after late childhood. Pediatric onset disease is more progressive with neurological, cardiac and liver dysfunction. These patients have increased sensitivity to most drugs used in anesthesia [Table 2]. However, to the best of our knowledge, there are no reports of any adverse events with ketamine and fentanyl in MD patients. Table 1 Typical features of MELAS Table 2 Effects of anesthetics in MD We report a 9-year-old girl, weighing 19 kg, with MELAS, and recurrent aspiration pneumonia due to bulbar involvement. She was on nasogastric tube (NGT) feeds and was planned for laparoscopic gastrostomy under general anesthesia. On examination, she was alert, conscious and dysarthric. Her respiratory rate was 30/min, oxygen saturation 93% on room air and 96% with oxygen supplementation. Bilateral coarse crepitations were present. Chest X-ray showed features suggestive of aspiration pneumonia. In view of her pulmonary condition and increased vulnerability to anesthetic agents, an open procedure was settled on, after discussion with the surgeons, under a combination of sedation with fentanyl-ketamine and local field block. She was adequately fasted, while maintained on dextrose containing intravenous fluid to avoid increasing metabolic burden. The risk of potential aspiration was considered minimal as she was kept NPO adequately, in addition to the presence of NGT allowing for suctioning of gastric contents (if any). After establishing intravenous access and routine monitoring, ketamine 10 mg bolus (0.5 mg/kg) was given with which the child fell asleep. Subsequently, a field block was given with a mixture of 0.2% ropivacaine and 1% lignocaine. Fentanyl 5 mcg (0.25 mcg/kg) was given just before skin incision. With these minimal doses, the respiratory rate fell to 3/min. A pediatric open circuit was used to assess respiration and assist if necessary. The child did not have any response to surgical incision, neither did she require any further doses. The procedure was completed successfully at the end of which she was shifted to a high dependency unit for monitoring. Though Markham et al. state that ketamine has been shown to inhibit oxidation in mitochondria in animal models,[2] there is paucity in literature on its untoward effects in patients with MD. In our patient, we chose ketamine for its analgesic effects, bronchodilatory properties and ability to maintain airway reflexes as well as spontaneous respiration. There are no adverse reports with the use of fentanyl either, except at higher doses.[3] However, we found an increased sensitivity to even a very minimal dose of both drugs, as manifested by a significant drop in respiratory rate. Close monitoring of the patient helped avert an untoward event. However, we wish to highlight that even these seemingly safe agents should be titrated to patient needs very cautiously, rather than a standard weight-based dosing. Two large cases series reported suggest the possibility of safe anesthesia with appropriate preoperative assessment and monitoring.[45] Nonetheless, it is also important to be aware of reports of delayed worsening of respiratory function with or without neurologic degeneration in mildly affected patients whose anesthetic course had been notably uneventful.[678] Choosing between local, regional, or general anesthesia, depends on the patient (tolerability of an awake procedure, degree of neuropathy/myopathy, spinal cord involvement) as well as nature of surgery (degree of muscle relaxation required, postoperative analgesia). Even in the absence of negative literature, it should be considered that the requirement of any agent may be lower than in normal individuals, and titration of drugs to effect is more appropriate than a weight-based nomogram. It will be prudent to remember that a successful use of one agent in a patient does not mean that the agent is safe to use in all, but may simply be due to a biased reporting. ==== Refs 1 Driessen JJ Neuromuscular and mitochondrial disorders: What is relevant to the anaesthesiologist? Curr Opin Anaesthesiol 2008 21 350 5 18458553 2 Markham A Cameron I White SJ The effect of ketamine hydrochloride, a non-barbiturate parenteral anaesthetic on oxidative phosphorylation in rat liver mitochondria Biochem Pharmacol 1981 30 2165 8 6457604 3 Shipton EA Prosser DO Mitochondrial myopathies and anaesthesia Eur J Anaesthesiol 2004 21 173 8 15055888 4 Driessen J Willems S Dercksen S Giele J van der Staak F Smeitink J Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects Paediatr Anaesth 2007 17 16 21 17184426 5 Footitt EJ Sinha MD Raiman JA Dhawan A Moganasundram S Champion MP Mitochondrial disorders and general anaesthesia: A case series and review Br J Anaesth 2008 100 436 41 18285393 6 Casta A Quackenbush EJ Houck CS Korson MS Perioperative white matter degeneration and death in a patient with a defect in mitochondrial oxidative phosphorylation Anesthesiology 1997 87 420 5 9286907 7 Cooper MA Fox R Anesthesia for corrective spinal surgery in a patient with Leigh's disease Anesth Analg 2003 97 1539 41 14570684 8 Grattan-Smith PJ Shield LK Hopkins IJ Collins KJ Acute respiratory failure precipitated by general anesthesia in Leigh's syndrome J Child Neurol 1990 5 137 41 2345280