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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).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.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 (EC50) 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 EC50 and 95% effective end-tidal concentrations (EC95) of sevoflurane without movement were 1.77% (95% CI 1.59-2.35) and 2.09% (95% CI 1.89-5.80), respectively.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.

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.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.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 protein-rich diet before surgery.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.She recovered consciousness with slightly increased ammonia level immediately after anesthesia.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 protein-rich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemia-related neurological dysfunction.

Rationale Lesch-Nyhan syndrome (LNS) is an X-linked recessive disorder presenting with uric acid overproduction, neurocognitive disability, and behavioral disturbances. Inhalational anesthesia has been frequently used in LNS patients undergoing surgery. Characteristic compulsive self-injurious behavior and high risk of emesis may hinder inhalational induction. Propofol may be beneficial for these patients because of its easy and rapid titration for anesthetic depth during induction, early recovery from anesthesia, and antiemetic effect as well as uricosuric effect. Patient concerns A 16-year-old male adolescent was scheduled for percutaneous nephrolithotomy. He exhibited poorly controlled muscle, self-injurious behaviors and intellectual disability. Diagnosis The patient presented with neurodevelopmental delay in the first year of life, and was diagnosed with LNS, with a substitution of phenylalanine to leucine in hypoxanthine-guanine phosphoribosyltransferase (HPRT) 1 gene on the X-chromosome at 3 years of age. Interventions Total intravenous anesthesia was used for induction and maintenance of anesthesia with propofol and remifentanil using target-controlled infusion. Outcomes Time to recovery of consciousness was prolonged after uneventful surgery. Serum uric acid levels gradually increased during postoperative period. Lessons Propofol anesthesia using target-controlled infusion does not provide significant clinical advantages in rapid emergence from anesthesia and management of hyperuricemia in LNS patients undergoing urological surgery.

Rationale Perioperative management of patients with bronchobiliary fistula (BBF) is an anesthetic challenge because they typically exhibit poor lung function preoperatively, require meticulous lung isolation intraoperatively and need postoperative respiratory support. Patient concerns A 44-year-old man with a past surgical history of pancreaticoduodenectomy presented fluctuating fever, jaundice, dyspnea and yellowish sputum. Despite intravenous antibiotic treatment and repeated percutaneous drainage, patient showed gradual deterioration with hypoxemia, and uncontrolled pneumonia. Diagnoses The patient was diagnosed with BBF based on the clinical manifestation such as biloptysis with pneumonia, and imaging studies. Interventions Resection of the fistula and bilobectomy was performed under general anesthesia. Avoidance of positive pressure ventilation before lung isolation and precise lung isolation are essential for patients with BBF to protect the unaffected lung. Therefore, rapid sequence induction was performed. Left-sided double-lumen tube was inserted for lung isolation and position of the tube was confirmed by visualization with fiberoptic bronchoscopy. Bile-stained secretion was repeatedly suctioned in trachea and both bronchi during surgery. Outcomes In spite of decrease in SpO2 with institution of one-lung ventilation, the patient's oxygenation was gradually improved as surgery progressed without hemodynamic instability. At the end of surgery, the double-lumen tube was replaced with a single-lumen endotracheal tube for postoperative mechanical ventilation. Lessons Absolute lung isolation using double-lumen tube for one-lung ventilation and bronchial toilet during surgery and replacement of single-lumen tube for postoperative respiratory support at the end of surgery are effective to improve oxygenation in patients with BBF.