title: Are children with Angelman syndrome at high risk for anesthetic complications?
abstract: OBJECTIVE
  To review seven children with Angelman syndrome (AS) undergoing 16 general anesthetics for both invasive and noninvasive procedures to determine if these children are at greater risk for anesthetic-related complications than the general population.
  
  
  BACKGROUND
  Children with AS may exhibit unpredictable responses to GABA agonists because of abnormal GABA receptors. These abnormal receptors may affect AS patients' responses to sedation and general anesthesia.
  
  
  METHODS
  The study design was a retrospective chart review of seven patients with AS who underwent a total of 16 general anesthetics for a variety of invasive and noninvasive procedures between the years 4/25/2005 and 12/31/2010. We reviewed the preoperative orders, anesthesia record and PACU records for preoperative medication orders and intraoperative and PACU adverse events.
  
  
  RESULTS
  We could not find documentation of complications attributed to the delivery of general anesthesia regardless of chromosomal defect, the use of GABA activating drugs, or a history of seizures. Six patients received a propofol-based anesthetic while 10 patients received potent inhalation agent for anesthetic maintenance. There were no statistical differences between the PACU lengths of stay (LOS) for AS patients as compared to the average PACU LOS for the 60 969 postprocedure patients cared for between 1/1/06 through 12/31/10.
  
  
  CONCLUSIONS
  We found no data to suggest that these patients demonstrate exaggerated responses to GABA stimulating drugs. In fact, it appears that regardless of the anesthetic agent, the perioperative course was unremarkable.
authors.affiliation: Anesthesiology and Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA. ira.landsman@vanderbilt.edu
authors.forename: Ira S
authors.initials: IS
authors.lastname: Landsman
authors.forename: Heather M
authors.initials: HM
authors.lastname: Mitzel
authors.forename: Sarika U
authors.initials: SU
authors.lastname: Peters
authors.forename: Terry J
authors.initials: TJ
authors.lastname: Bichell
mesh_terms: D000759:Adjuvants, Anesthesia / Q000009:adverse effects; D000758:Anesthesia / Q000009:adverse effects*; D000777:Anesthetics / Q000009:adverse effects*; D018686:Anesthetics, Intravenous; D017204:Angelman Syndrome / Q000150:complications* / Q000453:epidemiology* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003422:Critical Care; D005260:Female; D018755:GABA Agonists / Q000009:adverse effects; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000139:chemically induced / Q000453:epidemiology; D007902:Length of Stay; D008297:Male; D008874:Midazolam / Q000009:adverse effects; D010641:Phenotype; D015742:Propofol / Q000009:adverse effects; D011963:Receptors, GABA-A / Q000235:genetics; D012189:Retrospective Studies; D012306:Risk
chemical_list: D000759:Adjuvants, Anesthesia; D000777:Anesthetics; D018686:Anesthetics, Intravenous; D018755:GABA Agonists; C546273:GABRB3 protein, human; D011963:Receptors, GABA-A; D008874:Midazolam; D015742:Propofol