Background: Perioperative management of a patient with HAE type I.
Case report: 60years old female, 75 kg is admitted for total hip replacement. During the preoperative screening, HAE is mentioned with a first appearance at the age of 15 with a clinical presentation of bilateral lower limb oedema and abdominal pain. Since then, there have been no symptoms and no medication has been received.
Full laboratory analysis tests were ordered and low levels of C4 and C1-Inh were detected. The rest of the personal medical history was clear.
During the perioperative period, benzodiazepines, tranexamic acid and androgens were administered. C1- Inh 500 IU was given 2 hours pre-surgery. Patient in full monitoring. Spinal anaesthesia with 15 mg of bupibacaine and 27G needle was conducted. lidocaine cream was applied at the sites of cutaneous access.
The patient underwent the surgery with her consent. Surgical operation was 80 min. After surgery, the patient was observerd at the Post Operative Unit Care for 3 hours without complications. Tramadol iv was gived for post-operative analgesia.
Discussion: HAE is a rare disorder (1/50000-1/10000) of autosomal dominant inheritance. HAE patients have a defect in the gene that controls C1-inh. As a result either inadequate(type 1) or non-fuctionining C1-inh(type 2) are produced. Its role is to regulate activation of complement, fibrinolytic, coagulation and kinin systems and finally the release of bradykinine. It is characterized by swelling of limbs, face, airway and abdominal pains caused by trauma, cold and stress.The mortality rate is up to 35% due to laryngeal oedema therefore spinal anesthesia was preferred. Laboratory tests that set the diagnosis are low levels of C1 Inh1, C4 factor but C3 is normal. Treatment includes androgens and antifibrinolytic agents. Adrenaline, corticosteroids and antihistamines are ineffective.
Jerrold H. Levy, MD, FAHA, * Douglas J. Freiberger, MD,* J. Roback, MD, PhD, HAE: Current and Emerging Treatment Options, Society of Cardiovascular Anesthesiologists, May 2010, Vol.110, Number 5, pg1271-1280
Learning points: Even though the world literature is poor and it suggests as few invasive manipulations as possible, there were no complications in our case. The proper perioperative preparation remains the cornerstone.