2. The importance of cervicofacial anatomical location in C1-INH-HAE angioedema attacks

Oestrogens, trauma, infections or stress has been described as triggers for AE attacks in 21 patients with C1-INH-HAE [6]. Microtrauma can precipitate the onset of acute AE attacks, and thus, dental-oral procedures carry a high risk of triggering them and also an increased risk of death from asphyxiation due to the AE location [7]. Treatment with adrenaline, antihistamines or glucocorticoids is not effective in this type of BK-mediated AE [8].

In the past, without proper specific treatment, overall mortality after dental surgery in patients with C1-INH-HAE was up to 30–40% [9–12]. Some dental-oral, medical and/or surgical procedures are susceptible to receive "short-term prophylaxis" (STP) (also called "pre-procedural prophylaxis") [8, 13–15] in order to reduce the risk of AE. Such prophylaxis in patients with C1-INH-HAE usually consists of introducing oral antifibrinolytics or attenuated androgens (AAs) or administering intravenous pdhC1INH before the procedure [8, 13]. There are currently two brands of pdhC1INH available: Berinert® (CSL-Behring, Marburg, Germany) and Cinryze® (Shire-HGT, Zug, Switzerland). Since upper airway AE can cause death from asphyxiation [13, 16], adequate monitoring of upper airway permeability is imperative, so that appropriate emergency treatment (endotracheal intubation and/or tracheotomy) is performed if the upper airway is threatened despite medical treatment [8, 17]. Nevertheless, the availability of specific drugs for the treatment of acute AE attacks and of plasma-derived human C1-inhibitor concentrates (pdhC1INH) (Berinert®, CSL Behring, Marburg, Germany and Cinryze®, Shire HGT, Zug, Switzerland) for STP, together with the increased awareness of pre-procedural prophylaxis, has reduced the prevalence of upper airway respiratory AE and death from asphyxiation after dental procedures [15, 18].
