Modern preoperative and intraoperative management of hereditary angioedema

Abstract

Hereditary angioedema (HAE), deficiency of C1 esterase inhibitor, poses a risk of airway compromise during trauma, including surgery, due to activation of the complement cascade. Classical surgical management includes emergent/slash tracheostomy and cricothyrotomy, associated with high complication rates. We provide here an evidence-based review of available medical literature to construct guidelines for managing patients with HAE pre- and intra-operatively. We also describe our experience with a patient for whom we cared using these guidelines. Our objective was to explain new preventive measures to prevent airway compromise in HAE and their level of evidence for averting potential therapeutic misadventure. We analyzed PUBMED literature regarding airway management and etiology of angioedema and its prevention, followed by application of guidelines based on these data in a patient with HAE undergoing inguinal hernia repair. An analysis of contemporary literature yielded key points: (1) using a Cook Exchange catheter to facilitate re-intubation, (2) measuring cuff leak pressure to verify whether airway pressure had increased during surgery, (3) visualizing the airway directly using a fiberoptic laryngoscope connected to a digital flat-screen monitor for real-time assessment, (4) following conventional dictum to double stanozolol dosages 2 weeks before admission, (5) administering fresh frozen plasma pre- and intraoperatively, and (6) preparing recombinant C1 esterase inhibitor for instantaneous intraoperative use; and using FDA-approved human-derived C1-esterase inhibitor prophylactically. Biotechnology in the form of novel but currently available and in-practice medical devices, as well as new therapeutic agents, have expanded the armamentarium for safely managing patients with HAE pre- and intraoperatively.

2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

“…Since anabolic androgens such as Danazol are more efficacious in the short term compared to antifibrinolytics such as Tranexamic acid (TA; Cyklokapron ® ) or epsilon aminocaproic acid (EACA; Amicar ® ), anabolic steroids are more often used for short term prophylaxis in the setting where pdC1INH is not available [8,19,29,31]. The recommended dose for oral TA (not fully established) is 25 mg/kg two to three times daily with maximum 3 to 6 g daily; IV dose 10 mg/kg two to three times daily adjusting the dose for renal impairment [19,29,31,33,45-48]. …”

“…If pdC1INH is not available, then Danazol or Stanozolol are recommended as in V.1 (see figure 2; androgens preferred to TA; TA in doses as above; [19,29,31,33,45,47]. ) Solvent/detergent treated plasma (SDP; 10 ml/kg; 2 to 4 units, 400 to 800 ml per adult infusion) is an option one to six hours presurgery (fresh frozen plasma or frozen plasma is less safe than SDP; [8,9,19,28,29,31,33,48]; Dr. Mike Frank's group has reported using two units fresh frozen plasma the night before, [49-51]).…”

“…PdC1INH has been the first line therapy for several decades in Europe and elsewhere and used for many years in Canada under Special Access Program [8,12,17-19,29,31,35,37,38,48,52-54]. Berinert ® from CSL Behring was licensed by USA FDA October 9 th , 2009 for therapy of HAE events and licensed in many other countries for many years.…”

Management of hereditary angioedema in pregnant women: a review

“…If pdhC1INH is not available, virally inactivated fresh frozen plasma can be administered 92. pdhC1INH should also be available for acute angioedema treatment if necessary.…”

Angioedema: Perioperative management

“…It affects up to two-thirds of patients with hereditary angioedema (HAE) or acquired angioedema (AAE) during their lifetimes with a 15%–33% mortality rate. 1,2 …”