TIVA AND SUGAMMADEX IN A PATIENT WITH MELAS SYNDROME: A CASE REPORT

Aim. MELAS (mitochondrial myopathy, encephalopathy, lac- tic acidosis, and stroke-like episodes) syndrome is a maternal- ly inherited multisystem disease caused by mutations of the mitochondrial DNA. Clinical features are: encephalopathy manifesting as dementia and seizures, stroke-like episodes at young age (usually <40), lactic acidosis, myopathy with rag- ged-red fibres, migraines, depression, cardiomyopathy, and diabetes mellitus.1 General anaesthesia has several potential hazards to patients with MELAS syndrome, such as mali- gnant hyperthermia, hypothermia, and resistance to muscle relaxant.2,3 This case report describes the anaesthesiological management of a thyroidectomy in a MELAS syndrome patient. Case report. A 34-year-old woman with MELAS underwent thyroidectomy for adenocarcinoma. She suffered paralysis of the left vocal cord (in paramedian position), and some cha- racteristic clinical symptoms including: muscle weakness, psy- chomotor retardation, lactic acidosis (4.5 mM/L), diabetes, mellitus and renal failure in chronic dialysis treatment. Gene- ral anaesthesia was induced with Propofol, Remifentanil and Rocuronium, and maintained with continuous infusion of propofol and remifentanil. Lidocaine 2% spray in the hypo- pharynx was administered before the intubation. Monitoring in the operating room consisted in ECG, SpO2, minimally invasive hemodynamic monitoring from VigileoTM monitor/FloTracTM sensor (Edwards Lifesciences LLC, Irvi- ne, CA), temperature, capnogram. Neuromuscular function was assessed by Train-of-Four quantitative monitoring (acce- leromyography). Glycemia and arterial blood gases (ABG) tension were measured before, during and after the surgery. During the operation, the patient’s vitals were kept stable with blood pressure at 128 ± 8/75 ± 6 mmHg, heart rate 86 ± 8 beats/min, oxygen saturation 100%, body temperature 36.7 ± 0.2° C, CO 5.32 ± 0.31 l/m. The ABG showed metabolic acidosis (pH 7.31 ± 0.02; PaCO2 36.6 ± 1.32 mmHg; HCO3 19,3 ± 0,3 mM/L) and lactic acid values 4.93 ± 0.32 mM/l. The operation lasted around 30 minutes. Sugammadex 120 mg (2 mg/kg) was administered to reverse the neuromuscular block at TOF-Count = 2. Patient was extubated and moved to the recovery room with stabilized spontaneous breathing, and kept under observation for about 2 hours. Discussion. General anaesthesia for MELAS patients requi- res attention to the choices of the anaesthetic protocol, and careful monitoring because this syndrome affects many organs. We avoided volatile anesthetic and succinylcholine to prevent malignant hyperthermia. TIVA with propofol infused under one hour prevented propofol infusion syndrome4 and ensured hemodynamic stability. We chose sugammadex for the reversal of neuromuscular block because its safe, comple-te and rapid action (the time from administration to TOF- Ratio >0.90 was 280 sec.) allowed to reduce the risk of resi- dual muscle weakness and adverse respiratory events in postoperative period5. References 1. Sproule DM, Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes. basic concepts, clinical phenotype, and therapeutic manage- ment of MELAS syndrome. Ann NY Acad Sci 2008;1142:133-58. 2. Fricker RM, et al. Positive malignant hyperthermia susceptibility in vitro test in a patient with mitochondrial myopathy and myoadenylate deaminase deficiency. Anesthesiology 2002;97:1635-7. 3. Aouad MT, et al. Resistance to cisatracurium in a patient with MELAS syndro- me. Paediatr Anaesth 2005;15:1124-7. 4. Vasile B, et al. “e pathophysiology of propofol infusion syndrome: a simple name for a complex syndrome. Intensive Care Med 2003; 29: 1417-25. 5. Grattan-Smith PJ, et al. Acute respiratory failure precipitated by general ane- sthesia in Leigh’s syndrome. J Child Neurol 1990; 5:137-41.