• Geoff Frawley, Daniella Fuenzalida, Susan Donath, Joy Yaplito-Lee, and Heidi Peters.
• Department of Paediatric Anaesthesia and Pain Management, Royal Children's Hospital, Melbourne, Victoria, Australia. geoff.frawley@rch.org.au
• Paediatr Anaesth. 2012 Aug 1; 22 (8): 737-44.

Objectives And AimsTo document the incidence of difficult airway management and difficult intubation in the era of replacement therapy for Australian children with mucopolysaccharidosis (MPS).BackgroundMedical 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.MethodsA 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.ResultsSeventeen 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.ConclusionsHematopoietic 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.© 2012 Blackwell Publishing Ltd.

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