title: Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS).
abstract: BACKGROUND
  Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders characterized by tissue deposition of glycosaminoglycans (GAG). Their musculoskeletal abnormalities and the GAG storage in the airway result in increased risk for patients undergoing anesthesia. This study evaluates a multi-disciplinary airway management approach and reports upper and lower airway findings of flexible bronchoscopy performed during these procedures.
  
  
  METHODS
  This is a retrospective study over 10 years evaluating approaches to and outcomes of airway management and bronchoscopic findings in this patient group.
  
  
  RESULTS
  Thirty-one patients underwent a total of 105 anesthetic events of which 74 involved multiple surgical services. The majority of patients were either MPS I (n = 9) or MPS II (n = 19). The median age was 8.6 years (range 1.1-24 years). Airway management by anesthesiologists alone occurred in 31 cases including natural airway (n = 7), perilaryngeal airway (n = 7), oral or nasal intubation (n = 7) or tracheostomy (n = 6) and emergent fiberoptic intubation in four cases. In 74 of the procedures, flexible bronchoscopy was performed which included fiberoptic intubation in 22 cases. Post-operative complications occurred in eight cases mostly when prolonged airway instrumentation had occurred. The most frequent findings on bronchoscopy were GAG deposits/adenoid hypertrophy in 72%, laryngomalacia in 31% and lower airway deposits and/or tracheobronchomalacia in 46% of procedures. Deposits of GAG were seen in patients as young as 4 years of age.
  
  
  CONCLUSIONS
  Our experience demonstrates that a multidisciplinary approach and combined surgeries in MPS provides for safe airway management and allows diagnostic assessments for further patient care without added risks. Significant, multi-factorial airway compromise may occur already in early childhood including upper and lower airway GAG deposits.
authors.affiliation: Division of Pulmonology, Department of Pediatrics, University of North Carolina, Chapel Hill, North Carolina 27599-7217, USA. marianne_muhlebach@med.unc.edu
authors.forename: Marianne S
authors.initials: MS
authors.lastname: Muhlebach
authors.forename: Christopher B
authors.initials: CB
authors.lastname: Shaffer
authors.forename: Linda
authors.initials: L
authors.lastname: Georges
authors.forename: Kathy
authors.initials: K
authors.lastname: Abode
authors.forename: Joseph
authors.initials: J
authors.lastname: Muenzer
mesh_terms: D000293:Adolescent; D058109:Airway Management / Q000379:methods*; D000465:Algorithms; D000758:Anesthesia / Q000379:methods*; D001999:Bronchoscopy / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003661:Decision Support Techniques; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009083:Mucopolysaccharidoses / Q000175:diagnosis / Q000628:therapy; D012189:Retrospective Studies; D055815:Young Adult