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KMID : 1144020220180020069
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Journal of Korean Association for Disability and Oral Health
2022 Volume.18 No. 2 p.69 ~ p.75
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Dental treatment of a patient with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome under general anesthesia: a case report
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Kim Han-Byeol
Kim Hyun-Tae
Song Ji-Soo
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
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Abstract
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Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare disease caused by a mitochondrial DNA mutation. Patients with MELAS syndrome have physical restrictions that increase their risk of developing dental caries, and it can be challenging for them to receive dental care due to the possibility of seizures, vomiting, and mouthopening limitation. This report details the dental procedure performed on a 13-year-old girl with MELAS syndrome who had generalized mild hypomineralization of her permanent teeth. Dental caries on the occlusal surfaces of the mandibular second molars and maxillary first molars were restored with composite resin under general anesthesia. Patients with MELAS syndrome may benefit from dental treatment under careful general anesthesia. It is recommended to perform the procedure early in the morning and use the fluid containing 5% dextrose in place of lactate to reduce metabolic stress and prevent the progression of lactic acidosis. Some patients with MELAS syndrome may exhibit increased sensitivity to neuromuscular blockade, and sugammadex may be useful to counteract rocuronium’s effects. Additional flumazenil may help patients who regularly take benzodiazepines as antiepileptic medications recover from general anesthesia.
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KEYWORD
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Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome, Dental treatment, General anesthesia
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