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Hereditary angioneurotic oedema is an autosomal dominant disease associated with serum deficiency of functional C1-inhibitor. It is characterized by periodic swelling of subcutaneous tissues, abdominal viscera and upper airways. Lethal acute episodes of oedema can occur during anaesthesia and surgery. It is essential to prepare such patients before surgery. This article describes a case and the various preventive measures used to avoid acute episodes during anaesthesia for urgent surgery for mesenteric ischemia. In emergency situations where C1 inhibitor concentrate is not available, fresh frozen plasma (FFP) can be used as an alternative, as it also contains C1 inhibitor, corticosteroids, antihistamines, and epinephrine can be useful adjuncts but typically are not efficacious in aborting acute attacks. Prophylactic management involves long-term use of attenuated androgens or antifibrinolytic agents (Tranexamic acid).Their various indications are discussed.
Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: New findings concerning symptoms, affected organs, and course. Am J Med. 2006;119:267-74.
AGOSTINI A, CICARDI M. Hereditary and acquired C1 inhibitor deficiency. In: biological and clinical characteristic in 235 patients. Medicine. 1992;71:206-215.
ARVIEUX CC, ALIBEU JP, DROUET N_ Hereditary angioneurotic edema: Behavior to be taken in pediatric surgery. Chir Péd 1990;31:52-56.
Boccon-Gibod I. Hereditary angioedema: treatment and educational therapeutic program. Medical Press. 2015;44:78-88.
Sheffer AL, Austen KF, Rosen FS. Tranexamic acid therapy in hereditary angioneurotic edema. N Engl J Med. 1972; 287(9):452-454.
Gelfand JA, Sherins RJ, Alling DW, Frank MM. Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities. N Engl J Med. 1976;295(26):1444-1448.
Koeberlé P, Pequenot-Jeannin C, Douge C, Barale F. Angioneurotic edema and anesthesia: Preparation and perioperative monitoring Angioneurotic edema and anesthesia: preparation and perioperative monitoring. Ann Fr Anesth Reanim. 1994; 13 (3):417-420.
Mertes PM, Floccard B, Javaud N, Goichot B, Bouillet. Management of angioneurotic edema. The Doctors Congress. Update Conference © 2016 Sfar.
Hopkinson RB, Sutcliffe AJ. Hereditary angioneurotic edema. Anaesthesia. 1979;34(2):183- 186.