Management of patients with hereditary angio-oedema in dental, oral, and maxillofacial surgery: a review.

Hereditary angio-oedema (HEA) is an autosomal dominant, life-threatening genetic disorder that is caused by insufficiency or dysfunction of the C1 esterase inhibitor that develops coincidentally with recurrent oedema in the skin, internal organs, and upper respiratory tract. Increased production of bradykinin secondary to increased plasma kallikrein activity is the primary cause of attacks. Dental procedures cause emotional stress and mechanical trauma and may also initiate attacks. The most feared complication is asphyxiation as a result of laryngeal oedema. Cases that resulted in death after tooth extraction have been reported, so dentists and oral and maxillofacial surgeons should take maximum care in the treatment of patients with HAO, consult with the patient's doctor, and ensure that prophylaxis is given before the procedure. They should work as atraumatically as possible and use procedures to minimise stress. In the event of an attack of HAO, despite all the correct measures having been taken, the procedure should be terminated immediately and treatment of the attack started as soon as possible. The first drugs for the treatment of acute attacks are C1-INH (C1 inhibitor), ecallantide, or icatibant.