Chronic upper airway obstruction has been shown to cause secondary reversible pulmonary hypertension. Many pathophysiologic processes can produce such obstruction. A 3-month-old child with Beckwith-Wiedemann syndrome who manifested chronic upper airway obstruction secondary to macroglossia is reported. Early recognition and therapy of airway compromise may decrease morbidity and mortality in this syndrome and others in which macroglossia is present.

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