Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II

Abstract

Mucopolysaccharidosis type II (MPS II) is a rare, X-linked recessive disease characterized by deficiency of lysosomal iduronate-2-sulfatase. Progressive upper airway obstruction is common in patients with MPS II. Tracheostomy is an effective way of managing airway obstruction when less invasive interventions are no longer adequate. Here, we report a patient with MPS II who developed sudden respiratory distress in the emergency department and required tracheostomy as endotracheal intubation and laryngeal mask airway (LMA) failed to secure the airway.

Keywords

• Mucopolysaccharidosis
• Hunter syndrome
• airway
• tracheostomy