Background: This prospective study aimed to determine the optimum end-tidal sevoflurane concentration required for immobility during botulinum toxin injection in spontaneously breathing children with cerebral palsy (CP). Methods: Twenty-three children with spastic CP, aged 3 to 12 years, with American Society of Anesthesiologists (ASA) physical status I and II, scheduled to receive botulinum toxin type A injection were enrolled in the study. After induction of deep sedation using pre-filled 8% sevoflurane in oxygen and maintenance of the predetermined end-tidal sevoflurane concentration, the botulinum toxin was injected in spontaneously breathing children. The response to the botulinum toxin injection was classified as “movement” or “no movement” by an independent investigator who was blinded to the predetermined end-tidal sevoflurane concentration and bispectral index (BIS) value. The end-tidal sevoflurane concentration was predetermined, initiating at 2.0% with 0.2% as a step size in the next patient depending on the previous patient’s response using the modified Dixon’s up-and-down method. Results: Of 21 children, 12 (57.1%) showed “no movement” in response to the botulinum toxin injection. By Dixon’s up-and-down method, the 50% effective end-tidal concentration (EC 50 ) of sevoflurane for successful botulinum toxin injection was 1.76 ± 0.15% (95% CI 1.62–1.90). Based on the dose‐response curve using probit analysis, the predicted EC 50 and 95% effective end-tidal concentrations (EC 95 ) of sevoflurane without movement were 1.77% (95% CI 1.59–2.35) and 2.09% (95% CI 1.89–5.80), respectively. Conclusion: Botulinum toxin injection can be successfully accomplished at an end-tidal sevoflurane concentration of 1.76 ± 0.15% in 50% of spontaneously breathing children with CP aged 3–12 years.

Cardiovascular autonomic neuropathy (CAN) is characterized by dysregulation of sympathetic and parasympathetic nervous systems that causes cardiovascular and respiratory disorders. The number of diabetic patients undergoing surgery is increasing in line with the prevalence of DM. Anesthesiologists should pay attention to diabetic patients with CAN because it is related to serious cardiovascular morbidity and mortality. We report an 80-year-old male who underwent cervical laminoplasty. He had severe bradycardia and hypotension from induction to the suspension of surgery. His blood pressure dropped to 70/40 mmHg and his heart rate to 20 bpm. Ephedrine, phenylephedrine, and atropine administration had minimal effect, but after epinephrine administrations, his heart rate and blood pressure increased to 70 bpm and 170/90 mmHg. The operation was discontinued because of the patient's unstable, fluctuating vital signs. The results of autonomic nervous system function examination indicated postganglionic cholinergic sympathetic dysfunction, sympathetic adrenergic dysfunction, and parasympathetic cholinergic dysfunction.

Rationale: Hyperammonemia, metabolic derangement, and/or the prolonged effects of anesthetics may lead to delayed emergence from general anesthesia as well as the onset of type 2 citrullinemia, even in compensated patients with citrin deficiency.Patient concern: A 5-year-old girl with citrin deficiency was scheduled for blepharoplasty under general anesthesia. She developed hyperammonemia with temporary interruption of medication for a few days before surgery. Diagnosis:The patient was genetically diagnosed as citrin deficiency with a mutation in the SLC25A13 gene via newborn screening for metabolic disorders. Her citrulline and ammonia levels were well-controlled with arginine medication and protein-rich diet. Her elevated ammonia level by temporary interruption of medication was corrected with resumption of arginine medication and proteinrich diet before surgery.Interventions: We used desflurane and remifentanil for general anesthesia to avoid hyperammonemia and delayed emergence. End-tidal desflurane concentration and anesthetic depth were carefully monitored to avoid excessive anesthesia.Outcomes: She recovered consciousness with slightly increased ammonia level immediately after anesthesia.Lessions: General anesthesia of the shortest duration with the least metabolized drugs using desflurane and remifentanil, would be beneficial for rapid emergence in surgical patients with citrin deficiency. Maintenance of nitrogen scavenging medication, a proteinrich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemiarelated neurological dysfunction.Abbreviations: BIS = bispectral index, CTLN2 = type 2 citrullinemia, NICCD = neonatal intrahepatic cholestasis caused by citrin deficiency.