Tonsillectomy in a patient with hereditary angioedema after prophylaxis with C1 inhibitor concentrate.

Ann Allergy. 1994 Nov;73(5):435-8.

Tonsillectomy in a patient with hereditary angioedema after prophylaxis with C1
inhibitor concentrate.

Maves KK, Weiler JM.

Department of Internal Medicine, University of Iowa, Iowa City.

BACKGROUND: A 15-year-old young man with a history of recurrent streptococcal
pharyngitis and hereditary angioedema presented for tonsillectomy. Preoperative
physical examination was normal with the exception of enlarged pharyngeal tonsils
with crypts and pustules; there was no evidence of angioedema. Laboratory studies
were remarkable for a C4 level 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 concentrate as prophylactic treatment for a patient with hereditary
angioedema who required tonsillectomy. METHODS: The patient was treated with
stanozolol 4 mg po quid and clindamycin 150 mg po tid during the week 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: Approximately eight hours after an
uncomplicated tonsillectomy, the patient began to experience crampy abdominal
pain, typical of his hereditary angioedema. Beginning 22 hours after surgery, he
had facial swelling and complained of difficulty swallowing and the sensation of
throat swelling. The symptoms resolved over the next eight hours. Serial
laboratory examinations revealed: [table: see text]

CONCLUSIONS: We believe that
the occurrence of abdominal pain, facial swelling, and difficulty swallowing
suggests that this patient may have experienced a mild, generalized flare of
hereditary angioedema during the postoperative period in spite of prophylactic
therapy with both anabolic steroids and C1 INH concentrate. This serves as a
reminder that patients with hereditary angioedema require close observation
following invasive procedures even after premedication with stanozolol and C1 INH
concentrate.