title: Perioperative complications in children with Apert syndrome: a review of 509 anesthetics.
abstract: OBJECTIVE
  To perform a retrospective, anesthesia case note review in children with Apert Syndrome.
  
  
  OBJECTIVE
  To identify perioperative complications in this group of patients.
  
  
  BACKGROUND
  Apert syndrome is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly (cutaneous and bony fusion) of the hands and feet. Children with this syndrome require general anesthetics for a number of different operations and procedures. Our institution has records of 71 children with Apert syndrome. Analysis of their general anesthetic records was undertaken, and the incidence of perioperative complications was investigated.
  
  
  METHODS
  A retrospective case note review was performed on 61 children with Apert syndrome over a 14-year period. There were a total of 509 general anesthetics administered to these children during this period of time.
  
  
  RESULTS
  There were a total of 31 perioperative respiratory complications occurring in 21 patients (6.1% of the total cases). Twenty-three of these complications were supraglottic airway obstruction (4.5% of total cases).
  
  
  CONCLUSIONS
  We found there to be a low incidence of major perioperative major complications in this group of patients. Nevertheless, a significant proportion of these children have obstructive sleep apnoea and may develop supraglottic airway obstruction on induction and emergence from anesthesia due to the associated mid-face anatomical abnormalities.
authors.affiliation: Great Ormond Street Hospital, London, UK. sarahfbarnett@googlemail.com
authors.forename: Sarah
authors.initials: S
authors.lastname: Barnett
authors.forename: Claire
authors.initials: C
authors.lastname: Moloney
authors.forename: Robert
authors.initials: R
authors.lastname: Bingham
mesh_terms: D000168:Acrocephalosyndactylia / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D000759:Adjuvants, Anesthesia; D000293:Adolescent; D000758:Anesthesia; D000768:Anesthesia, General; D000769:Anesthesia, Inhalation; D001285:Atropine; D002648:Child; D002675:Child, Preschool; D045422:Continuous Positive Airway Pressure; D019465:Craniofacial Abnormalities / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019637:Orthopedic Procedures; D019990:Perioperative Care; D059035:Perioperative Period; D019651:Plastic Surgery Procedures; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D020181:Sleep Apnea, Obstructive / Q000150:complications
chemical_list: D000759:Adjuvants, Anesthesia; D001285:Atropine