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
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