title: A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses.
abstract: OBJECTIVE
  To document the incidence of difficult airway management and difficult intubation in the era of replacement therapy for Australian children with mucopolysaccharidosis (MPS).
  
  
  BACKGROUND
  Medical treatment for MPS has developed significantly since 1980's with a large number of patients now being offered either bone marrow transplant or enzyme replacement. The impact of these therapies on the incidence of difficult airway management has not been adequately documented. Similarly, anesthesia techniques and airway devices have been developed, which are thought to have greatly increased the safety of managing these patients under anesthesia but their role in children with MPS has not been systematically described.
  
  
  METHODS
  A retrospective chart review of 17 patients with MPS who had received anesthetics at the Royal Children's Hospital during the time frame January 1998-January 2011. The primary outcome was the incidence of difficult or failed intubation. Secondary outcomes were the relationship between the incidence of difficult intubation and treatment with enzyme replacement therapy (ERT) or bone marrow transplantation.
  
  
  RESULTS
  Seventeen patients received 141 anesthetics for 214 procedures. Difficult face mask ventilation occurred in 20 anesthetics (14.2%). Difficult intubation occurred in 40 anesthetics (25%). Failed intubation occurred in two cases (1.6%).The incidence of difficult intubation was 12% in MPS I, 35% MPS II, 86.7% in MPS VI, and 0% in MPS III and IV.
  
  
  CONCLUSIONS
  Hematopoietic stem cell transplantation prior to 2 years of age reduces the incidence of difficult mask ventilation and difficult intubation in children with MPS I. ERT was initiated late in the clinical course of MPS II and VI and induced improvements in upper airway patency but did not reduce the incidence of difficult airway management.
authors.affiliation: Department of Paediatric Anaesthesia and Pain Management, Royal Children's Hospital, Melbourne, Victoria, Australia. geoff.frawley@rch.org.au
authors.forename: Geoff
authors.initials: G
authors.lastname: Frawley
authors.forename: Daniella
authors.initials: D
authors.lastname: Fuenzalida
authors.forename: Susan
authors.initials: S
authors.lastname: Donath
authors.forename: Joy
authors.initials: J
authors.lastname: Yaplito-Lee
authors.forename: Heidi
authors.initials: H
authors.lastname: Peters
mesh_terms: D000293:Adolescent; D058109:Airway Management; D000402:Airway Obstruction / Q000628:therapy; D000758:Anesthesia / Q000379:methods*; D001315:Australia; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D056947:Enzyme Replacement Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000453:epidemiology; D007442:Intubation, Intratracheal; D017214:Laryngeal Masks; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000503:physiopathology; D008059:Mucopolysaccharidosis I / Q000150:complications / Q000628:therapy; D016532:Mucopolysaccharidosis II / Q000150:complications / Q000628:therapy; D009084:Mucopolysaccharidosis III / Q000150:complications / Q000628:therapy; D009085:Mucopolysaccharidosis IV / Q000150:complications / Q000628:therapy; D011183:Postoperative Complications / Q000453:epidemiology; D012121:Respiration, Artificial; D012189:Retrospective Studies; D016896:Treatment Outcome