Risk of angioedema following invasive or surgical procedures in HAE type I and II : the natural history

  • Background: Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications. As surgery can be a trigger for edema episodes, current guidelines recommend preoperative prophylaxis with C1-INH or attenuated androgens in patients with HAE undergoing surgery. However, the risk of an HAE attack in patients without prophylaxis has not been quantified. Objectives: This analysis examined rates of perioperative edema in patients with HAE not receiving prophylaxis. Methods: This was a retrospective analysis of records of randomly selected patients with HAE type I or II treated at the Frankfurt Comprehensive Care Centre. These were examined for information about surgical procedures and the presence of perioperative angioedema. Results: A total of 331 patients were included; 247 underwent 700 invasive procedures. Of these procedures, 335 were conducted in 144 patients who had not received prophylaxis at the time of surgery. Categories representing significant numbers of procedures were abdominal (n = 113), ENT (n = 71), and gynecological (n = 58) procedures. The rate of documented angioedema without prophylaxis across all procedures was 5.7%; in 24.8% of procedures, the presence of perioperative angioedema could not be excluded, leading to a maximum potential risk of 30.5%. Predictors of perioperative angioedema could not be identified. Conclusion: The risk of perioperative angioedema in patients with HAE type I or II without prophylaxis undergoing surgical procedures ranged from 5.7% to 30.5% (CI 3.5–35.7%). The unpredictability of HAE episodes supports current international treatment recommendations to consider short-term prophylaxis for all HAE patients undergoing surgery.

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Author:Emel Aygören-PürsünORCiDGND, Inmaculada Martinez SaguerGND, Wolfhart Kreuz, Thomas KlingebielORCiDGND, Dirk Schwabe
Parent Title (English):Allergy
Publisher:Blackwell Munksgaard
Place of publication:Oxford
Document Type:Article
Date of Publication (online):2013/08/23
Date of first Publication:2013/08/23
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2013/10/08
Tag:C1-inhibitor deficiency; HAE; hereditary angioedema; preprocedure prophylaxis; surgical procedures
Page Number:6
First Page:1034
Last Page:1039
© 2013 University Hospital Frankfurt, Goethe University. Allergy published by John Wiley & Sons Ltd This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License http://creativecommons.org/licenses/by-nc-nd/3.0/, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
Institutes:Medizin / Medizin
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - Namensnennung-Nicht kommerziell-Keine Bearbeitung 3.0