Hereditary angioedema (HAE) is an autosomal dominant hereditary disorder in which edema occurs spontaneously because of a decrease and dysfunction of the complement inhibitor C1-inhibitor (C1-INH), developing and disappearing repeatedly in various parts of the body. The edema can be triggered by physical stress, such as emotional stress and overwork, as well as by dental procedures, such as tooth extraction. In addition, stimulation arising from tracheal intubation and extubation under general anesthesia can induce laryngeal edema, which may lead to a fatal upper airway obstruction, thereby requiring strict attention during perioperative management. In the present case, dental treatment under general anesthesia with nasal intubation was performed in an HAE patient with autistic spectrum disorder and an extreme phobia of dental treatment. Reportedly, adrenaline and felypressin, which are added to local anesthetic preparations, should be avoided in dental treatment because they can trigger seizures by tensing the adrenal gland and sympathetic nerves and increasing the fibrinolytic system. In the present case, since there were no plans for tooth extraction or other invasive procedures, 3％ mepivacaine hydrochloride was used.
We report our experience providing good perioperative management that did not lead to an edema attack in response to stimulation during the dental treatment or nasal intubation ; this success was achieved by collaborating with a medical doctor, preoperative supplementation with C1-INH and tranexamic acid, and thorough preparation.
