TONSILLECTOMY IN A PATIENT WITH HEREDITARY ANGIOEDEMA AFTER PROPHYLAXIS WITH C1 INHIBITOR CONCENTRATE

Background: A 15-year-old young man with a history of recurrent strept ococcal pharyngitis and hereditary angioedema presented for tonsillect omy. Preoperative physical examination was normal with the exception o f enlarged pharyngeal tonsils with crypts and pustules; there was no e vidence of angioedema. Laboratory studies were remarkable for a C4 lev el of 8 mg/dL (normal 20-50 mg/dL) and C1 inhibitor (C1 INH) level of 4 mg/dL (normal 11-26 mg/dL). Objective: To report the use of C1 INH c oncentrate as prophylactic treatment for a patient with hereditary ang ioedema who required tonsillectomy. Methods: The patient was treated w ith stanozolol 4 mg po qid and clindamycin 150 mg po tid during the we ek before the procedure. Two hours prior to surgery, he received 2300 plasma units of intravenous C1-inhibitor (Human) Vapor Heated, IMMUNO (IMMUNO Clinical Research Corporation, New York, NY). Results: Approxi mately eight hours after an uncomplicated tonsillectomy, the patient b egan to experience crampy abdominal pain, typical of his hereditary an gioedema. Beginning 22 hours after surgery, he had facial swelling and complained of difficulty swallowing and the sensation of throat swell ing. The symptoms resolved over the next eight hours. Serial laborator y examinations revealed:[GRAPHICS] Conclusions: We believe that the oc currence of abdominal pain, facial swelling, and difficulty swallowing suggests that this patient may have experienced a mild, generalized f lare of hereditary angioedema during the postoperative period in spite of prophylactic therapy with both anabolic Steroids and C1 INH concen trate. This serves as a reminder that patients with hereditary angioed ema require close observation following invasive procedures even after premedication with stanozolol and C1 INH concentrate.