ACUTE AIRWAY-OBSTRUCTION IN HUNTER-SYNDROME

Hunter syndrome is one of the mucopolysaccharidoses, characterized by a deficiency of the lysosomal enzyme iduronate sulfatase. Among its ph ysical manifestations, there are numerous head and neck signs, includi ng characteristic facial features, macroglossia and short neck. The ac cumulation of glycosaminoglycans in the soft tissues of the head and n eck can be associated with acute airway obstruction. We report a 7 yea r old boy with Hunter syndrome who developed acute airway compromise r equiring an emergency tracheotomy. A review of the literature of airwa y management in patients with this disease is also presented. (C) 1998 Elsevier Science Ireland Ltd. All rights reserved.