Perioperative Management and Postoperative Outcomes in Patients with Hereditary Angioedema

Rationale: This retrospective study collected postoperative outcomes data for patients with HAE, and analyzed these data based on demographics, disease specific variables, and surgical variables. The goal was to determine optimal perioperative management of this population.

Methods: A retrospective chart review of 30 patients who underwent 56 procedures at Hershey Medical Center from 2002-2015 with a diagnosis of HAE, identified by database query. Data collected included demographics, disease related data, and data regarding perioperative management and complications.

Results: Four patients, each on one occasion after a single procedure, had postoperative HAE attacks. Two of these patients did not receive preoperative prophylaxis. The other 2 received a prophylactic dose of 1500 units of C1-inhibitor (based on 20 units/kg dosing), preoperatively. In spite of this one patient experienced attacks in multiple locations including the airway after thyroidectomy, and the other experienced an abdominal attack after port placement. Neither required reintubation or tracheotomy. Whether or not patients received prophylaxis was significantly related to the anesthetic induction technique (p < 0.01). Patients were more likely to receive prophylaxis if receiving general anesthesia as opposed to conscious sedation. Two patients remained intubated postoperatively; neither was discharged on the day of surgery. 

Conclusions: These findings have perioperative management implications for patients with HAE. Specifically, even patients that received adequate doses of prophylactic C1-inhibitor may experience postoperative attacks suggesting some patients may need higher doses of C1-inhibitor or danazol in addition to C1-inhibitor to prevent poor outcomes.  Furthermore, preparation to treat an attack is essential despite prophylaxis.