Management of tracheal lesions in Hurler syndrome.
Hurler syndrome is a rare mucopolysaccharide storage disease that becomes clinically apparent during early childhood as mucopolysaccharide deposits form in skeletal and soft tissues. Progressive mucopolysaccharide deposition in the oropharynx and tracheal connective tissues leads to airway obstruction if untreated. Tonsillectomy, adenoidectomy, and tracheostomy have been utilized to provide symptomatic relief of upper airway obstruction. Treatment of tracheal lesions by laser excision is a recent innovative method for the management of airway obstruction in children with Hurler syndrome. We describe two boys with Hurler syndrome whose mucopolysaccharide tracheal lesions were excised by utilization of the carbon dioxide laser.
Adachi
K
K
Department of Otolaryngology, University of California, Davis School of Medicine.
Chole
R A
RA
D002648:Child; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D053685:Laser Therapy; D008297:Male; D008059:Mucopolysaccharidosis I / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D014132:Trachea / Q000378:metabolism / Q000473:pathology; D014135:Tracheal Stenosis / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D014139:Tracheostomy
D006025:Glycosaminoglycans