Abstract
BACKGROUND: Non-ketotic hyperglycinemia (NKH) is a rare autosomal recessive disorder caused by defects in the glycine cleavage system, leading to elevated glycine levels in the central nervous system. NKH manifests in various forms, with the neonatal type being the most severe and often associated with high mortality and significant neurological impairment. This case report highlights the successful uses of desflurane and nitrous oxide for anesthetic management in a patient with NKH. CASE PRESENTATION: A 6-year-old girl with severe NKH, who had a history of delayed emergence from sevoflurane anesthesia, underwent tracheostomy for recurrent upper airway obstruction and severe obstructive sleep apnea. To address the previous issues with sevoflurane, general anesthesia was induced with propofol and fentanyl and maintained with 4% desflurane and 60% nitrous oxide. The electroencephalogram (EEG) showed near-complete suppression upon induction, which gradually resolved. Following cessation of desflurane and nitrous oxide, the patient exhibited early recovery, with eyes opening 3 min later and spontaneous breathing restored 19 min later. The patient experienced no postoperative complications and was discharged on the 14th postoperative day. CONCLUSION: This case suggests that desflurane, with its favorable pharmacological profile, may offer a superior alternative to sevoflurane for anesthetic management in NKH patients, particularly those with a history of delayed emergence. The observed EEG suppression may indicate heightened sensitivity to anesthetics in NKH, highlighting the need for tailored anesthetic strategies in this population.