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 physical manifestations, there are numerous head and neck signs, including characteristic facial features, macroglossia and short neck. The accumulation of glycos...

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Bibliographic Details
Published in:International journal of pediatric otorhinolaryngology 1998-08, Vol.44 (3), p.273-278
Main Authors: Yoskovitch, Adi, Tewfik, Ted L, Brouillette, Robert T, Schloss, Melvin D, Der Kaloustian, Vazken M
Format: Article
Language:English
Subjects:
Airway Obstruction - etiology
Airway Obstruction - surgery
Biological and medical sciences
Child
Humans
Hunter syndrome
Lysosomal enzyme
Male
Medical sciences
Mucopolysaccharidosis II - complications
Non tumoral diseases
Otorhinolaryngology. Stomatology
Tracheotomy
Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology
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Summary:Hunter syndrome is one of the mucopolysaccharidoses, characterized by a deficiency of the lysosomal enzyme iduronate sulfatase. Among its physical manifestations, there are numerous head and neck signs, including characteristic facial features, macroglossia and short neck. The accumulation of glycosaminoglycans in the soft tissues of the head and neck can be associated with acute airway obstruction. We report a 7 year old boy with Hunter syndrome who developed acute airway compromise requiring an emergency tracheotomy. A review of the literature of airway management in patients with this disease is also presented.
ISSN:0165-5876
1872-8464
DOI:10.1016/S0165-5876(98)00063-9