-
Pediatric pulmonology · Jun 2013
Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS).
- Marianne S Muhlebach, Christopher B Shaffer, Linda Georges, Kathy Abode, and Joseph Muenzer.
- Division of Pulmonology, Department of Pediatrics, University of North Carolina, Chapel Hill, North Carolina 27599-7217, USA. marianne_muhlebach@med.unc.edu
- Pediatr. Pulmonol. 2013 Jun 1; 48 (6): 601-7.
IntroductionMucopolysaccharidoses (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.MethodsThis is a retrospective study over 10 years evaluating approaches to and outcomes of airway management and bronchoscopic findings in this patient group.ResultsThirty-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.ConclusionOur 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.Copyright © 2012 Wiley Periodicals, Inc.
Notes
Knowledge, pearl, summary or comment to share?You can also include formatting, links, images and footnotes in your notes
- Simple formatting can be added to notes, such as
*italics*
,_underline_
or**bold**
. - Superscript can be denoted by
<sup>text</sup>
and subscript<sub>text</sub>
. - Numbered or bulleted lists can be created using either numbered lines
1. 2. 3.
, hyphens-
or asterisks*
. - Links can be included with:
[my link to pubmed](http://pubmed.com)
- Images can be included with:

- For footnotes use
[^1](This is a footnote.)
inline. - Or use an inline reference
[^1]
to refer to a longer footnote elseweher in the document[^1]: This is a long footnote.
.