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Hereditary angioedema (HAE) results from an underlying absence or dysfunction of the C1 esterase inhibitor and concurrent exposure to stressors, including surgery. Type I and II are caused by a mutation in the C1NH gene. Type I is due to deficiency of the C1 inhibitor; Type II is due to the dysfunction of the C1 inhibitor. This results in diffuse swelling and possible airway catastrophe, which can persist when incorrectly treated as anaphylaxis or allergic angioedema. We report the successful management of a HAE patient with a previously identified difficult airway undergoing elective surgery.

Perioperative Management of a Patient with C1 Esterase Deficiency and a Known Difficulty Airway, a Case Report with Mini Review