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Hunter's syndrome: A study in airway obstruction
Author(s) -
Sasaki Clarence T.,
Ruiz Richard,
Kirchner John A.,
Gaito Raymond,
Seshi Beerelli
Publication year - 1987
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1288/00005537-198703000-00005
Subject(s) - medicine , pharynx , autopsy , airway obstruction , airway , thickening , tongue , anatomy , pathology , larynx , surgery , chemistry , polymer science
Hunter's disease is a genetically transmitted defect known to produce mucopolysaccharide infiltration of multiple organ systems. Upper airway obstruction is caused by an enlarged tongue, deformed pharynx, and short, thick neck. Its eventual lethal outcome by the second decade of life is known to result from an infiltrative cardiomyopathy leading to irreversible heart failure. Instead, our recent experience in the care of five patients with this disorder suggests the lethal event is related to progressive obstruction sequentially involving the upper, mid, and lower airway characterized by gradual deformation and collapse of the trachea. Autopsy and histopathologic whole organ sections demonstrate anteroposterior flattening of the trachea and bronchi with submucosal thickening producing structural alterations known only to this disease.