Abstract
BACKGROUND: Succinic semialdehyde dehydrogenase deficiency is a rare autosomal recessive disorder of Gamma-aminobutyric acid metabolism characterized by neurological deficits and an increased risk of seizures. Patients with succinic semialdehyde dehydrogenase deficiency may present unique challenges in anesthesia management due to altered neurotransmitter levels and possible drug sensitivities. The literature is sparse regarding perioperative management of these patients. CASE PRESENTATION: We present the case of a 7-year-old boy, who underwent dental procedures under general anesthesia, with genetically confirmed succinic semialdehyde dehydrogenase deficiency. Anesthesia was initiated by intravenous induction, and sevoflurane was used for maintenance, with careful use of intravenous midazolam and rocuronium. Local anesthetic (lidocaine with epinephrine) was administered. The procedure lasted 120 min and was uneventful. The patient recovered without complications and was discharged the same day. CONCLUSION: This case highlights the feasibility of sevoflurane-based anesthesia in patients with succinic semialdehyde dehydrogenase deficiency. Safe perioperative management can be achieved with individual anesthetic planning, careful drug selection, and appropriate monitoring. Documentation of similar cases is necessary to establish evidence-based anesthetic strategies in this rare patient population.