Successful management of hereditary angioedema during tonsillectomy: a case report

Blood Coagul Fibrinolysis. 2012 Mar;23(2):155-7. doi: 10.1097/MBC.0b013e32834ee10d.

Abstract

Hereditary angioedema (HAE) is a rare genetic disorder causing a deficiency in C1 esterase inhibitor (C1-INH) that is manifested through unpredictable oedema. We describe a case of a patient with HAE who had previously been refused surgery for tonsillitis due to the potential for oedema, in whom regular monitoring of C1-INH levels combined with intensified therapy with danazol, tranexamic acid and C1-INH concentrate enabled an uncomplicated procedure with no oedema crisis. However, clinicians should be alerted that higher overall dosages of C1-INH concentrate for perioperative prophylaxis may be required than those typically used to treat acute HAE attacks.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Angioedemas, Hereditary / complications*
  • Angioedemas, Hereditary / drug therapy*
  • Complement C1 Inhibitor Protein / therapeutic use
  • Danazol / therapeutic use
  • Edema / etiology
  • Edema / prevention & control
  • Female
  • Humans
  • Tonsillectomy / adverse effects
  • Tonsillectomy / methods*
  • Tonsillitis / complications*
  • Tonsillitis / surgery*
  • Tranexamic Acid / therapeutic use

Substances

  • Complement C1 Inhibitor Protein
  • Tranexamic Acid
  • Danazol