Abstract

BACKGROUND:

Hereditary angioedema (HAE) is extremely rare and clinically characterized by recurrent and self-limiting skin, intestinal and life threatening laryngeal edema. Because of the danger of asphyxiation, it is vital that acute attacks of laryngeal edema be interrupted immediately. The aim of this study is to provide information on the incidence, diagnosis and treatment of laryngeal edema with upper airway obstruction in general and due to HAE.

METHODS:

102 patients with swelling of the larynx and difficulty in breathing (stridor) and 21 patients with a manifest HAE were surveyed at the Department of Otorhinolaryngology, Head and Neck Surgery of the Ludwig-Maximilians-University of Munich from 2001 to 2004.

RESULTS:

63% of the laryngeal swellings with airway obstruction were due to radiotherapy in cancer and the intake of ACE-Inhibitors. Just 22% were due to allergic reactions. Only one patient (< 1%) suffered from HAE. All patients were treated with intravenous steroids and anti-histamines successfully, except the HAE patient. After emergency coniotomy the patient was treated with purified C1 inhibitor concentrate (C1-INH).

CONCLUSIONS:

According to our experience almost all patients with laryngeal swelling and upper airway obstruction react well to the standard therapy with steroids and anti-histamines. In contrast these drugs have no effect on HAE. Replacement therapy with C1-INH has proved to be effective. The challenge is to realize whether an airway obstruction is due to HAE or not. In this case, careful observation, the right medication and an appropriate airway management are lifesaving. The possibility of sudden airway obstruction and asphyxiation must be discussed with these patients and their relatives.