INTRODUCTIONAngioedema (AE) is described as edema due to an allergic, hereditary or acquired condition. Hereditary AE (HAE) accounts for 2% of clinical AE cases and affects roughly 1 in 50,000 people. HAE is transmitted in... [ view full abstract ]
INTRODUCTION
Angioedema (AE) is described as edema due to an allergic, hereditary or acquired condition. Hereditary AE (HAE) accounts for 2% of clinical AE cases and affects roughly 1 in 50,000 people. HAE is transmitted in an autosomal dominant pattern and results in episodic edema of the skin and mucosa of the respiratory and gastrointestinal tracts. In HAE, dysfunction of the C1 esterase inhibitor (C1-INH) leads to elevated bradykinin levels, which increases vascular permeability and causes edema. The most dreaded complication is edema of the upper airway resulting in life-threatening airway compromise and asphyxia. Type I HAE accounts for 85% of HAE and is characterized by deficient levels of C1-INH. Type II HAE is seen in 15% of cases and involves normal or elevated levels of dysfunctional C1-INH. AE development can be unprovoked or triggered by minor things, and the perioperative period is considered high risk for triggering AE.
CASE REPORT
A 69-year-old female with a past medical history of HAE Type II, HTN, DLD, GERD presented for C2-C6 laminoplasty and fenestration of syringohydromyelia causing progressive left upper extremity weakness and sensory deficits. Patient noted that she had three prior tracheostomies, two related to URIs in the 1960’s and the third in 2004 following surgery for an ischemic bowel. She was on long-term prophylaxis with danazol 200mg daily for the past 30 years. On the day of surgery, she received 2000u of IV plasma-derived C1-INH (Berinert) one hour prior to surgery. She then underwent uneventful induction of general anesthesia and intubation utilizing a GlideScope and 6.0 ETT. A CVC and arterial line were placed and the patient underwent successful laminoplasty and fenestration. The patient was extubated in the OR at the end of surgery and taken to the ICU for careful observation overnight. She was continued on her home dose of danazol throughout her stay and did not experience any airway AE during her hospitalization but did complain of abdominal pain for which she received 1500u plasma-derived C1-INH on POD 2, 4, and 6.
DISCUSSION
No routinely administered anesthetic drugs are considered contraindicated in patients with HAE, but estrogen contraceptives, hormone replacement therapy, and ACE inhibitors are known to potentially worsen the disease. Management focuses on increasing C1-INH levels and blocking the actions of kallikrein, bradykinin, and plasmin. This is accomplished by short-term or long-term prophylaxis or with rescue therapy. Long-term therapy is indicated in those experiencing >1 attack per month that is unresponsive to rescue therapy or when rescue therapy is not readily available. This is accomplished with C1-INH, attenuated androgens, and antifibrinolytics. Short-term prophylaxis for procedures requiring airway manipulation calls for C1-INH 1 hour before surgery with an additional dose available in case of emergency. Androgens are another option if C1-INH is not available. Rescue therapy for acute AE can be treated with C1-INH, ecallantide, or icatibant. HAE is a rare disease that can pose a significant challenge to anesthesiologists in the perioperative period; therefore one must be familiar with the prophylaxis and treatment algorithms currently available.
