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Clinical Note
February 2004

Airway Management in Mucopolysaccharide Storage Disorders

Author Affiliations

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.

Arch Otolaryngol Head Neck Surg. 2004;130(2):233-237. doi:10.1001/archotol.130.2.233
Abstract

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.

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