Perioperative considerations in the patient with Angelman syndrome.
Angelman syndrome arises by one of 4 genetic mechanisms. Patients often have craniofacial abnormalities, vagal hypertonia, skeletal muscle atrophy or underdevelopment, a history of seizure disorders, and pharmacodynamic unpredictability. Its pathogenesis, clinical manifestations, diagnosis and treatment options, and perioperative anesthetic considerations are presented.
Department of Anesthesiology, University of Alabama School of Medicine, Birmingham, AL 35294, USA.
Cara M
CM
Bevinetto
Department of Anesthesiology, Louisiana State University School of Medicine, New Orleans, LA 70112, USA. Electronic address: akaye@lsuhsc.edu.
Alan D
AD
Kaye
D000328:Adult; D000402:Airway Obstruction / Q000150:complications / Q000503:physiopathology; D000758:Anesthesia / Q000379:methods*; D017204:Angelman Syndrome / Q000150:complications / Q000503:physiopathology* / Q000601:surgery*; D000818:Animals; D002648:Child; D004827:Epilepsy / Q000150:complications / Q000503:physiopathology; D006331:Heart Diseases / Q000150:complications / Q000503:physiopathology; D006801:Humans; D051379:Mice; D009133:Muscular Atrophy / Q000150:complications / Q000503:physiopathology; D019990:Perioperative Care / Q000379:methods*
Anesthesia; Angelman syndrome; Craniofacial abnormalities; Vagal hypertonia