Perioperative Management for Patients with Hereditary Angioedema

Abstract

Hereditary angioedema (HAE) is a rare autosomal dominant disease that results from mutations in the C1-esterase inhibitor (C1-INH) gene. HAE is characterized by recurrent episodes of angioedema of the skin (face, extremities, genitalia, trunk), the gastrointestinal tract, and respiratory tract. Symptoms experienced can be debilitating, may impact quality of life, and can be life threatening. Preventing attacks particularly for patients undergoing procedures is critical. Patients with HAE may now treat acute attacks or prevent attacks with medications that have recently become available in the United States; however, these same medications can be used for perioperative management for patients undergoing medical, surgical, and dental procedures. Periprocedural planning is important for patients to reduce the incidence of acute attacks. Education is critical and increasing awareness of short-term prophylaxis options will allow providers to develop an appropriate action plan for their patients. The goal of this review is to increase awareness for HAE treating physicians, surgeons, anesthesia, and emergency room physicians by examining the available treatment options, researching the literature, and summarizing available data for periprocedural management. The availability of treatment options has increased over the past few years, expanding options for physicians and patients living with HAE and improve safety during the perioperative period and at the time of other procedures.

Preventing Hereditary Angioedema Attacks in Children Using Cinryze®: Interim Efficacy and Safety Phase 3 Findings

“…Hereditary angioedema (HAE), a rare disease with an estimated prevalence of 1 in 50,000 [1], is characterized by episodic swelling of the skin, abdomen, and larynx [2,3]. HAE types I and II are identified by low total levels and nonfunctionality of the C1 inhibitor (C1-INH), respectively, accounting for approximately 85 and 15% of cases [4].…”

Angioedema: Perioperative management

“…2,9–12 Dosing depends on the manufacturer. The dose may be repeated in more complex surgeries, those of extended duration and those with significant blood loss.…”

“…Published recommended therapeutic durations vary from 4–7 days preoperatively through 3–5 days post-operatively. Dosing ranges from 4 mg of danazol every 6 h 2 to 200 mg every 8 h 9 with or without the addition of prophylactic C1-INH administration. 8,11 FFP is described most consistently as a rescue agent and as third-line prophylactic agent after androgens.…”

“…A volume of 10 mL/kg or 2–4 units should be administered 1–6 h preoperatively. 1,9,10 Though FFP replenishes C1-INH, it can theoretically worsen an attack by replacing depleted complement factors. Cardiac function should be assessed as FFP administration contributes to intravascular volume.…”

Prophylaxis in hereditary angioedema (HAE) with C1 inhibitor deficiency

“…Mechanical trauma may trigger local swelling attacks . Short‐term preventive treatment is therefore recommended for all interventions associated with any mechanical impact to the upper aerodigestive tract . One to six hours (according to the SmPC of Cinryze ® even 24 hours) prior to an intervention in the upper aerodigestive tract, prior to intubation, or also prior to bronchoscopy or esophagogastroduodenoscopy, human C1‐INH concentrate should be given.…”