General anaesthesia in an adult patient with Morquio syndrome with emphasis on airway issues

Abstract

Patients with Morquio syndrome possess a number of characteristics which may complicate an anaesthetic procedure. The most important is that a deposition of mucopolysaccharides in the soft tissues of the oro-pharynx distorts the airway, making the airway management difficult, while the atlanto-axial instability puts these patients at risk of subluxation and quadriparesis. As the endotracheal intubation in Morquio syndrome patients may be difficult or even impossible, we recommend the technique of awake fiberoptic intubation to be considered. Our approach to awake fiberoptic intubation in an adult patient is described in this case report.

Keywords

• Morquio syndrome
• mucopolysaccharidosis
• neck instability
• general anesthesia
• fiberoptic intubation