title: Anesthetic management of comprehensive dental restoration in a child with glutaric aciduria type 1 using volatile sevoflurane.
abstract: Glutaric aciduria type 1 (GA1) is a rare, inherited mitochondrial disorder that results from deficiency of mitochondrial glutaryl-CoA dehydrogenase. Most patients develop neurological dysfunction early in life, which leads to severe disabilities. We present a 37-month-old girl with GA1 manifested as macrocephaly and hypotonia who received comprehensive dental restoration surgery under general anesthesia with sevoflurane. She was placed on specialized fluid management during a preoperative fasting period and anesthesia was administered without complications. All the physiological parameters, including glucose and lactate blood levels and arterial blood gas were carefully monitored and maintained within normal range perioperatively. Strategies for anesthetic management should include prevention of pulmonary aspiration, dehydration, hyperthermia and catabolic state, adequate analgesia to minimize surgical stress, and avoidance of prolonged neuromuscular blockade. We administered general anesthesia with sevoflurane uneventfully, which was well tolerated by our patient with GA1. Additionally, communication with a pediatric geneticist and surgeons should be undertaken to formulate a comprehensive anesthetic strategy in these patients.
authors.affiliation: Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
authors.forename: Wei-Nung
authors.initials: WN
authors.lastname: Teng
authors.affiliation: Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
authors.forename: Su-Man
authors.initials: SM
authors.lastname: Lin
authors.affiliation: National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC; Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Dau-Ming
authors.initials: DM
authors.lastname: Niu
authors.affiliation: Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
authors.forename: Yi-Min
authors.initials: YM
authors.lastname: Kuo
authors.affiliation: Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
authors.forename: Kwok-Hon
authors.initials: KH
authors.lastname: Chan
authors.affiliation: Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC. Electronic address: cssung@vghtpe.gov.tw.
authors.forename: Chun-Sung
authors.initials: CS
authors.lastname: Sung
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications*; D000758:Anesthesia / Q000379:methods*; D018685:Anesthetics, Inhalation / Q000494:pharmacology*; D001928:Brain Diseases, Metabolic / Q000150:complications*; D002675:Child, Preschool; D058250:Dental Restoration Repair; D005260:Female; D050770:Glutaryl-CoA Dehydrogenase / Q000172:deficiency*; D006801:Humans; D008738:Methyl Ethers / Q000494:pharmacology*; D000077149:Sevoflurane
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane; D050770:Glutaryl-CoA Dehydrogenase
keywords: dental restoration; fluid therapy; general anesthesia; glutaryl-CoA dehydrogenase; inhalation anesthetics