Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema

Background. Recurrent angioedema is the hallmark of various inherited or acquired angioedema disease. Hereditary angioedema, or HAE, due to C1 inhibitor, or C1NH deficiency has considerable implications for dental health care providers because dental surgery may trigger distressing and even life-threatening episodes. Case Description. The authors reviewed the literature, focusing on the pathogenesis, clinical signs and treatment of HAE. They also provided case reports of four patients who died from laryngeal endema induced by tooth extraction. In patient with HAE, dental surgery - including tooth extraction - may be followed by self-limiting edema episodes, including lips swelling, facial swelling, tongue edema and laryngeal edema with upper airway obstruction. Preoperative prophylaxis has been preformed with attenuated androgens, fresh frozen plasma, C1NH concentrate and antifibrinolytics. The four patients described underwent tooth extraction which, after a symptom-free latency of four to 30 hours, provoked laryngeal edema. Three of the patients died of asphyxiation the night after surgery, and the fourth died on the second night. In three of the patients, laryngeal edema had not occurred previously. Clinical Implication. Before under-going dental surgery, patients with a history of recurrent angioedema should be evaluated for C1NH deficiency. If it is present, they are at risk of developing life-threatening laryngeal edema.