rocuronium and Angelman-Syndrome

Topics: Adrenergic Agonists; Anaphylaxis; Androstanols; Anesthesia; Anesthetics, Inhalation; Anesthetics, Intravenous; Angelman Syndrome; Anti-Allergic Agents; Anti-Inflammatory Agents; Blood Pressure; Cardiopulmonary Resuscitation; Child; Diphenhydramine; Epinephrine; Female; Heart Arrest; Humans; Male; Methyl Ethers; Methylprednisolone; Midazolam; Monitoring, Physiologic; Neuromuscular Nondepolarizing Agents; Nitrous Oxide; Rocuronium; Sevoflurane; Tachycardia, Sinus; Vagus Nerve

We describe the perioperative treatment of a patient diagnosed with Angelman syndrome, which is usually caused by a deletion in chromosome 15. The patient showed the characteristic signs of psychomotor retardation, epilepsy, lack of speech, frequent laughter and happy demeanor, light skin, blue eyes, and blond hair, hyperactivity, and sleep disturbance. He was scheduled for multiple tooth extractions under general anesthesia. Intravenous anesthesia was induced using ketamine, propofol, and rocuronium, and was maintained with low concentrations of sevoflurane. There were no incidents during or after surgery. The chromosomal abnormality that causes Angelman syndrome is located on the same genes that control the production of gamma-aminobutyric acid-A receptors, which are activated by most intravenous and inhaled anesthetic agents. The effect of the condition on the response of these agents is unknown. The combined use of propofol, ketamine, and sevoflurane at low doses provided adequate anesthesia for this patient. Other characteristics of the syndrome that may affect the use of anesthesia in these patients are discussed.

Topics: Adolescent; Androstanols; Anesthesia, Inhalation; Anesthesia, Intravenous; Angelman Syndrome; Bradycardia; Bundle-Branch Block; Bupivacaine; Contraindications; GABA Agonists; GABA-A Receptor Agonists; Humans; Ketamine; Male; Methyl Ethers; Neuromuscular Nondepolarizing Agents; Propofol; Receptors, GABA-A; Rocuronium; Sevoflurane; Tooth Extraction