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A 7-year-old boy with a history of Hunter syndrome presented to the emergency department with a 3-day history of difficulty breathing. His parents noted that he had been in his usual state of health until 3 days before presentation, when he developed a slight cough, which worsened and was described as “barking” in nature approximately 24 hours before admission.
Physical examination revealed an afebrile, intermittently sedated child who became agitated and combative when aroused. His oral cavity was notable for macroglossia and copious secretions. His oropharynx was normal in appearance, and there was mucopurulent drainage from his nose. He had a short neck, growth delay, and an abnormal facies. He was initially treated with multiple courses of albuterol sulfate, which he used at home for his asthma. He did not improve with this treatment, and a chest x-ray film was obtained (Figure 1and Figure 2). He was admitted to the pediatric intensive care unit and treated aggressively with continuous positive airway pressure and intravenous steroids.
Chen B, Wilhelm M, Boseley M. Radiology Quiz Case 1. Arch Otolaryngol Head Neck Surg. 2010;136(1):95. doi:10.1001/archoto.2009.182-a