Anaesthetic Management in a Patient with Mitochondrial Encephalo Cardiomyopathy Undergoing a Dental Procedure: A Case Report
Denada Haka, Deniz Sivrioğlu, Nedim Çekmen
Başkent Universitesi Hastanesi
Giriş: MELAS is a mitochondrial syndrome characterized by myopathy, encephalopathy, lactic acidosis, stroke-like episodes, diabetes and sensorineural hearing loss. From the anaesthesiologist's point of view, muscle fatigue and weakness, prolonged muscle blockage, hypothermia, tachycardia, acidosis and difficulty in breathing are some of the challenges to be faced during general anesthesia.
Olgu: This is the case of a 10-year-old boy with MELAS undergoing a dental procedure. Standard monitoring, including a pulse oximeter, electrocardiogram, capnography, noninvasive blood pressure, Bispectral Index and Train of Four were placed. After preoxygenation by 80% O2 for 3 min, general anesthesia was induced with propofol 2 mg/kg, fentanyl 1 μg/kg, and rocuronium 0.6 mg/kg. His mask ventilation was difficult because of his triangular face, so we used an oral airway to provide adequate ventilation. An arterial blood sample obtained from the patient at the beginning of the operation showed lactic acidosis. He was given 500 mL of lactate free containing fluid intravenously to provide hydration. A circulating-water mattress and a forced-air warming device were used to prevent hypothermia. A glucometer measured blood glucose once in 30 minutes to avoid hypoglycemia.
Tartışma ve Sonuç: We emphasize the importance of decreasing prolonged fasting ad hypoglycemia, postoperative nausea and vomiting, hypothermia, acidosis, and multidisciplinary assessment. All of the anesthetic agents potentially affect the mitochondria functions at different rates, and there is yet not a particular combination of drugs especially recommended for this group of patients. We used sevoflurane in our patient, which may cause more natural muscle relaxation than desflurane and isoflurane.MELAS patients have a more definitive risk of rhabdomyolysis and malignant hyperthermia than regular patients. The use of succinylcholine with muscle wasting can still predispose patients to hyperkalemia; that is why we used rocuronium to provide neuromuscular relaxation and obtained arterial blood gases to detect the level of K+, which remained within average values
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