Anaesthesia Management of a Case with Hereditary Angioedema for Whom Tracheal Dilatation was Planned.

Abstract

Hereditary angioedema (HAE) causes recurrent angioedema attacks in the oropharynx, larynx, face, and other regions due to bradykinin overproduction as a result of C1 esterase inhibitor deficiency. Surgical interventions requiring general anaesthesia might trigger HAE attacks. Laryngeal angioedema is the most important cause of perioperative mortality. Tracheal dilatation was performed by rigid bronchoscopy in our patient with type 1 HAE, because of tracheal stenosis due to prolonged intubation, which occurred after the attack. The patient was administered 2x500 IU C1-esterase inhibitor approximately 24 hours before rigid bronchoscopy. No complication developed after the first procedure. Two months later, tracheal dilatation was repeated and 2x500 IU C1 esterase inhibitor was administered. While the patient was followed up in the intensive care unit, significant oedema developed in the facial area, especially the tongue and lips, approximately 10 hours after the procedure. Our patient also had stridor due to airway obstruction. The patient was treated with 1000 IU C1 esterase inhibitor and 3 units of fresh frozen plasma (FFP). After FFP, edema started to regress. The patient was discharged after symptoms improved. The patient should be monitored in the intensive care unit for a minimum of 48 hours to monitor for postoperative laryngeal oedema.