Acute Presentation of Undiagnosed Hereditary Angioedema of the Larynx: Averting Death

Abstract

Hereditary angioedema (HAE) differs from histamine-mediated angioedema in that it is resistant to steroids and antihistamines. Laryngeal attacks of this condition, if not diagnosed timely, carry a mortality rate up to 34%. Rarely, this disease goes undiagnosed until late adulthood and presents a life-threatening episode that poses a management challenge to the emergency physician. We report the case of a 48-yearold man who presented to the emergency department with progressive breathing difficulty two hours after consuming a carbonated drink. Clinical examination revealed supraglottic edema. He did not respond to steroids or antihistamines and required emergency tracheostomy to secure the airway due to failed intubation. Absence of symptoms such as itching or urticaria and inadequate response to steroids pointed to hereditary angioedema. Low complement factor 4 levels with low C1 esterase inhibitor functionality confirmed the diagnosis. This case report highlights the fact that delayed presentation of HAE can be life threatening and the diagnosis should be considered in all non-atopic adult patients with angioedema.

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

“…HAE attacks of the upper airways can result in asphyxiation 94–97 . Abdominal attacks are painful and debilitating 98–100 .…”

“…HAE attacks of the upper airways can result in asphyxiation. [94][95][96][97] Abdominal attacks are painful and debilitating. 98-10 0 Peripheral attacks such as those of hands or feet result in impaired function.…”

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

“…HAE attacks of the upper airways can result in asphyxiation. 94 , 95 , 96 , 97 Abdominal attacks are painful and debilitating. 98 , 99 , 100 Peripheral attacks such as those of hands or feet result in impaired function.…”