Case Report

Anaesthetic Approach for Patient with Hereditary Angioedema

10.5152/TJAR.2019.90522

  • Maize Cordeiro Melo
  • Walkiria Wingester Vilas Boas
  • Marcos Daniel Faria
  • Alyne Andrade Lima
  • Rafaela Souto Souza

Received Date: 28.12.2018 Accepted Date: 21.04.2019 Turk J Anaesthesiol Reanim 2020;48(1):68-70

Hereditary Angioedema (HEA), a disease caused by a mutation in the gene that encodes for the production of the fraction C1 in the complement (C1-INH), is a rare pathology (1/50.000) that causes swelling of the skin and submucosa in various organs, either naturally triggered or provoked by physical and psychological traumas, infections, or by the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and angiotensin-converting enzyme inhibitors (ACEIs). Surgical trauma may spur the HEA crisis, leading to complications such as the swelling of the respiratory tracts and hemodynamic instability. Thus, the pre-surgical approach to HEA patients requires a specific plan that ensures short term prophylaxis, careful intra-operative management, rescue therapy and intensive post-surgery care. We present a report on a video-laparoscopic cholecystectomy approach for a 28-year-old woman diagnosed with asthma and HEA with symptomatic choledocholithiasis. We opted for short-term prophylaxis and immunology with the intravenous application of C1-INH. Ultrasonography imaging showed arterial wall oedema, which could correspond to a manifestation of C1-INH deficiency in the wall of the manipulated arteries during ultrasonography-guided puncture. Once the patient recovered consciousness, she was transferred to the intensive care unit and was discharged on the 6th day of hospitalisation.

Keywords: Anaesthesia, C1 complement inhibitory protein, hereditary angioedema