This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on HighWire  • Citing articles on ISI (6)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Airway Obstruction  • Alert me on articles by topic

Shai Y. Shinhar, MD; Hedy Zablocki, RN; David N. Madgy, DO

Arch Otolaryngol Head Neck Surg. 2004;130:233-237.

Mucopolysaccharidoses (MPS), a group of disorders caused by a genetic disruption, create a special challenge for the otolaryngologist. With the rare types of MPS IV and MPS 1 S, a skilled practitioner is required to abate airway management complications. The erratic deposits of mucopolysaccharides throughout the trachea should be taken into account when decisions to stent the airway are made. Proper management requires to provide an airway that is custom-made to meet the patient's needs. This is a case-by-case presentation of 3 patients with MPS who presented to the Children's Hospital of Michigan with progressive respiratory embarrassment. Discussed are the various issues revolving around our ability to provide proper airway management, from intubation to tracheostomy tube placement.

From the Department of Pediatric Otolaryngology, Children's Hospital of Michigan, Detroit (Dr Shinhar and Ms Zablocki), and Michigan Pediatric Ear Nose and Throat, Detroit (Dr Madgy). The authors have no relevant financial interest in this article.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Mucopolysaccharidosis I: Management and Treatment Guidelines
Muenzer et al.
Pediatrics 2009;123:19-29.
ABSTRACT | FULL TEXT