title: Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema.
abstract: BACKGROUND
  Recurrent angioedema is the hallmark of various inherited or acquired angioedema diseases. 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.
  
  
  METHODS
  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 edema induced by tooth extraction. In patients with HAE, dental surgery--including tooth extraction--may be followed by self-limiting edema episodes, including lip swelling, facial swelling, tongue edema and laryngeal edema with upper airway obstruction. Preoperative prophylaxis has been performed 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.
  
  
  CONCLUSIONS
  Before undergoing 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.
authors.affiliation: Universitäts-Hautklinik, Mainz, Germany. bork@hautklinik.klinik.uni-mainz.de
authors.forename: Konrad
authors.initials: K
authors.lastname: Bork
authors.forename: Sven-Erik
authors.initials: SE
authors.lastname: Barnstedt
mesh_terms: D000328:Adult; D000402:Airway Obstruction / Q000209:etiology; D000799:Angioedema / Q000150:complications / Q000209:etiology*; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D017742:Dental Care for Chronically Ill / Q000009:adverse effects*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015843:Serpins / Q000172:deficiency; D014081:Tooth Extraction / Q000009:adverse effects*
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human; D015843:Serpins