{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T09:52:01Z","timestamp":1790070721118,"version":"4.0.1"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2012,9,4]],"date-time":"2012-09-04T00:00:00Z","timestamp":1346716800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Elaprase","award":["GHT-HIT 050"],"award-info":[{"award-number":["GHT-HIT 050"]}]},{"name":"Elaprase","award":["HGT-HIT-045"],"award-info":[{"award-number":["HGT-HIT-045"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>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\u2010disciplinary airway management approach and reports upper and lower airway findings of flexible bronchoscopy performed during these procedures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a retrospective study over 10 years evaluating approaches to and outcomes of airway management and bronchoscopic findings in this patient group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010one patients underwent a total of 105 anesthetic events of which 74 involved multiple surgical services. The majority of patients were either MPS I (n\u2009=\u20099) or MPS II (n\u2009=\u200919). The median age was 8.6 years (range 1.1\u201324 years). Airway management by anesthesiologists alone occurred in 31 cases including natural airway (n\u2009=\u20097), perilaryngeal airway (n\u2009=\u20097), oral or nasal intubation (n\u2009=\u20097) or tracheostomy (n\u2009=\u20096) and emergent fiberoptic intubation in four cases. In 74 of the procedures, flexible bronchoscopy was performed which included fiberoptic intubation in 22 cases. Post\u2010operative 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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\u2010factorial airway compromise may occur already in early childhood including upper and lower airway GAG deposits. Pediatr Pulmonol. 2013; 48:601\u2013607. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22629","type":"journal-article","created":{"date-parts":[[2012,9,4]],"date-time":"2012-09-04T18:47:21Z","timestamp":1346784441000},"page":"601-607","source":"Crossref","is-referenced-by-count":31,"title":["Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS)"],"prefix":"10.1002","volume":"48","author":[{"given":"Marianne S.","family":"Muhlebach","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher 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