Abstract
Bradykinin-mediated angioedema (AE) is a rare disease characterized by recurrent cutaneous or mucosal angioedema. This hereditary or acquired disease is of rapid installation, non-pruritic, usually painless and can affect the face, lips, larynx, gastrointestinal tract or extremities. When the affected area involves the upper respiratory tract, laryngeal angioedema can lead to imminent death by asphyxia. This is the reason for the high mortality rate (30 %) in undiagnosed or improperly managed patients. High-risk situations in oral and maxillofacial surgery procedures should be identified preoperatively. Short-term prophylaxis must be carried-out prior to any procedure that may trigger an attack. A multi-site reference center (CREAK) has been created to help clinicians to manage this disease. This article reviews the pathophysiologic mechanisms, the clinical presentations, the possible treatments, the acute strategies for attacks and different prophylactic possibilities in oral and maxillofacial surgery.
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