An anesthetic experience of hereditary angioedema type I patient undertook total laparoscopic hysterectomy - A case report -

Abstract

Background Hereditary angioedema (HAE) is an autosomal dominant disorder. The characteristic of HAE is recurrent angioedema episodes due to low C1 esterase inhibitor (C1-INH) level. HAE symptoms, especially those affecting oropharynx or larynx may develop respiratory distress syndrome due to impaired airway, which can be potentially fatal. Case We report a clinical case of a 57 year-old female patient, with type I HAE, scheduled for total laparoscopic hysterectomy under general endotracheal anesthesia, which was done successfully without inducing airway edema. Danazol, which increases liver synthesis of C1-INH, was administered and fresh frozen plasma (FFP), which contained C1-INH, was transfused after induction. Conclusions For HAE patients, the greatest concern is that general anesthesia can induces upper airway edema by direct mucosal irritation by the endotracheal tube. The perioperative management should include both prophylactic increase of C1-INH production and on-demand administration of C1-INH or FFP.

Keywords

• anesthesia
• complement c1 inhibitor protein
• danazol
• fresh frozen plasma
• hereditary angioedema
• hysterectomy