Perioperative Approach to Patients with Hereditary Angioedema

Abstract

Introduction: Hereditary angioedema (HAE) is a rare, autosomal dominant disease manifested by the sudden, recurrent and variable severity of subcutaneous and submucosal edema. These episodes may occur spontaneously or in response to triggers. Three types of HAE are known. The disease is conditioned by a decrease in the plasma level of C1 inhibitor and increase of bradykinin, with increased vascular permeability and consequent edema.

The aim of this study is to present a protocol for the perioperative management of patients with hereditary angioedema.

Methods: We carried out a bibliographic review in 3 databases, with the terms “hereditary angioedema” AND “anaesthesia”. We included articles under 10 years old and in English. In total, 58 articles were found, 22 were selected.

Results: There are a number of drugs available for the hereditary angioedema approach: C1 inhibitor concentrate, human plasma / fresh frozen plasma, attenuated androgens, antifibrinolytics and modulators of the contact system. Prophylaxis of crisis may be performed in the long term, in patients with frequent or severe symptoms, and in the short term before dental, medical or surgical procedures. The treatment of acute attacks is carried out intra or extra-hospital.

Discussion: Most of the articles included are descriptions of individual clinical cases or review of an institution’s cases. Several measures must be taken in the perioperative period in order to avoid an acute crisis. Prophylaxis should be performed during pregnancy prior to any surgical procedure, in dental procedures (except dental cleaning or simple restoration), when surgery involves airway manipulation, in patients with previous episodes of angioedema affecting the airway and when there is an estimate of significant volume changes. Due to the risk of angioedema in the postoperative period, these patients should remain in a unit with permanent surveillance.

Conclusions: One of the potential triggers for a hereditary angioedema crisis is anaesthetic and surgical trauma. It is essential to adopt measures to prevent angioedema and decrease morbidity and mortality. Any suspected case should be referred to the Immunoallergology Service of one of the reference centers.