Anaesthesetic considerations in the perioperative management of patients with hereditary angioedema-FXII.

Abstract

Hereditary angioedema (HAE) is of interest to anaesthesiologists due to its impact on perioperative management. HAE manifests as episodic, self-limiting oedema in the deep subcutaneous or submucosal tissue that can be fatal if it involves the airway. It is usually caused by C1 esterase inhibitor deficiency. HAE episodes can be triggered by trauma, including surgical and medical procedures associated with mechanical trauma. Our understanding of HAE has evolved in recent years following the development of new genetic sequencing technologies that have made it possible to differentiate variants with normal C1 esterase inhibitor levels. In Spain, the most frequent of these is HAE due to a mutation in FXII, previously known as HAE type III. There are as yet no good quality studies on perioperative management and treatment of these patients. The admission of a patient with FXII-HAE for labour induction has given us the opportunity to review the perioperative management of these cases.