A Case of Airway Obstruction in a Patient with Hunter Syndrome

Abstract

Patients with Hunter syndrome have progressive deposition of glycosaminoglycans in the soft tissue of the throat and trachea, and tend to have a big tongue and coarse facial features. The deposition is thought to be responsible for airway obstruction. We report herein on a 20-year-old man with Hunter syndrome with dyspnea, whose airway obstruction became severe with stenosis of his trachea and bronchus. He was treated with insertion of a metallic stent which improved his airway symptoms for several months. Unfortunately, although he had planned to have a stent operation for his narrow airway, he suddenly died from cardio-respiratory arrest before he could have the operation.