Hereditary angioedema presenting as epiglottitis.

ABSTRACT

Hereditary angioedema is a disorder characterized by decreased levels or function of complement C1 esterase inhibitor. Symptoms in children generally consist of recurrent episodes of soft tissue swelling. On rare occasion, it can cause airway edema which may lead to airway obstruction. A case is presented of a child presenting with epiglottitis requiring intubation. Initial management of this rare complication should be directed at establishing an adequate airway and ensuring good oxygenation and ventilation. In addition, prompt administration of C1 esterase inhibitor concentrate is the most effective means of stopping progression of laryngeal edema and other forms of swelling. Commonly used agents for airway edema such as glucocorticoids, antihistamines, H1-blockers, and epinephrine tend not to be effective for reducing swelling related to hereditary angioedema.