Patients with Hunter syndrome may have symptoms of hoarseness, stridor and breathing difficulties as a result of laryngeal and tracheal involvement. In planning their evaluation, we must prefer non-invasive methods as X-ray or CT scan, and avoid doing endotracheal intubation or bronchoscopies. Review of adult cases in the literature and description of the only case of a child with Hunter syndrome having life-threatening complications of his upper airways is discussed in this report. In this case and in the literature we cannot exclude intubation or bronchoscopy as a serious aggravating factor, causing further narrowing of the larynx.
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