Anesthetic management of a patient with acquired angioedema submitted to broncofibroscopy: a case report

Abstract

Acquired angioedema with C1 inhibitor deficiency (AAE-C1INH) is a very rare condition of bradykinin-mediated angioedema. One of its major complications is potentially life-threatening, laryngeal edema. We report a 53-year-old woman with AAE-C1INH proposed for an elective broncofibroscopy. The direct stimulation caused by broncofibroscopy poses a high risk of angioedema, thus presenting an anesthetic challenge. Due to the risk of death, it is essential to adopt preventive measures. Short-term prophylaxis was performed, and the acute treatment was readily available. A well-structured multidisciplinary periprocedural plan makes it possible to safely approach the airway, in a remote area of the hospital.

Keywords: Airway Management; Angioedema; Bronchoscopy; C1 Esterase Inhibitor.

Figure 1

The function of C1 esterase inhibitor (C1-INH) includes inhibition of (1) activated factor XII (FXIIa)-mediated cleavage of prekallikrein to kallikrein; (2) kallikrein-mediated conversion of highmolecular-weight kinin (HMWK) to bradykinin; (3) plasmin-mediated fibrinolysis; (4) C1 esterase-mediated activation of C4 in the classical complement pathway. In the presence of C1-INH disfunction or deficiency, there is no inhibition of the contact activation, fibrinolysis and complement cascade, leading to overproduction of bradykinin and consequently edema. Management of bradykinin-mediated angioedema can include increasing C1-INH levels with C1-INH concentrate and attenuated androgens (e.g., danazol) and inhibition of bradykinin with Icatibant and plasmin with antifibrinolytics (e.g., tranexamic acid). C1-INH, C1 esterase inhibitor; FXII, Factor XII; HMWK, highmolecular-weight kinin. Figure redrawn based on “MacBeth LS, Volcheck GW, Sprung J, Weingarten TN. Perioperative course in patients with hereditary or acquired angioedema. J Clin Anesth. 2016;34:385-91.” with authors’ permission.