Dental treatment of a patient with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome under general anesthesia: a case report
김한별, 김현태, 송지수, 신터전, 현홍근, 김영재, 김정욱, 장기택,
소속 상세정보
김한별 ( Kim Han-Byeol ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김현태 ( Kim Hyun-Tae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
송지수 ( Song Ji-Soo ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
신터전 ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
현홍근 ( Hyun Hong-Keun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김영재 ( Kim Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김정욱 ( Kim Jung-Wook ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
장기택 ( Jang Ki-Taeg ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
Abstract
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.
키워드
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome; Dental treatment; General anesthesia
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