Hereditary angioedema (HAE), caused by C1-inhibitor (C1-INH) deficiency or dysfunction, is a rare and potentially life-threatening disease that leads to unpredictable recurrent attacks of angioedema in localized regions, including the larynx. As medical or dental procedures can trigger laryngeal edema, resulting in asphyxiation, major global guidelines recommend short-term prophylaxis prior to invasive procedures and long-term prophylaxis to prevent acute attacks and achieve near-normal lives. Here, we report the case of a 63-year-old male who experienced asphyxiation after tooth extraction. Emergency tracheotomy had saved his life at the age of 40 years, before the diagnosis of HAE. At the age of 63, when he had another opportunity for tooth extraction, he was definitively diagnosed with HAE. Administering short-term prophylaxis with ongoing long-term prophylaxis for HAE and perioperative multidisciplinary management for tooth extraction helped prevent recurrent fatal angioedema due to dental procedures and this can be useful when managing patients with HAE.
Keywords: acute attack; hereditary angioedema; laryngeal edema; prophylaxis; recurrence; tooth extraction; trigger.
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