Hereditary angioedema (HAE) is an inherited disease characterized by angioedema, that is caused by a genetic abnormality of C1-inhibitor (C1-INH). Decrease or functional abnormality of the C1-INH protein leads to excessive production of bradykinin, which causes increased vascular permeability. Patients with HAE present with edema of the face, arms and legs, as also of the larynx and gastrointestinal tract. Notably, airway stenosis caused by laryngeal edema can be fatal. Upper airway surgery under general anesthesia in patients with HAE carries the risk of airway stenosis caused by surgical site edema and by laryngeal edema due to tracheal intubation. In one guideline for the management of HAE, administration of C1-INH (Berinert®P) prior to surgery is recommended as prophylaxis against the development of angioedema.
A 68-year-old female patient with HAE (type 1) was referred to our department with the chief complaint of pain in the right maxilla. She had undergone Caldwell-Luc surgery for sinusitis as a 13-year-old. CT showed two cysts in the right maxilla, and she was diagnosed as having maxillary cysts and scheduled for surgery. As she suffered from frequent attacks of HAE, we administered C1-INH to the patient prior to surgery, according to a guideline for the management of HAE, and the patient underwent endoscopic sinus surgery (ESS) under general anesthesia without HAE attacks during the perioperative period.
ESS under general anesthesia in a patient with HAE carries the risk of angioedema developing at the surgical site and laryngeal edema developing as a result of tracheal intubation. We could safely perform ESS without angioedema developing in the perioperative period, by prophylactic administration of C1-INH according to a guideline for the management of HAE.
