Anaesthesia for a Patient with B-thalassaemia

Abstract

Β-Thalassaemia is a rare hereditary disease caused by partial or complete deficiency of β-haemoglobin chain synthesis. There is a lot of literature regarding anaesthetic management in other haemoglobinopathies (i.e. sickle cell disease), especially in the paediatric population, but there is scarce information regarding β-thalassaemia major in adults. With current medical management, β-thalassaemia major patients survive to adulthood and may present for a variety of surgical procedures, even unrelated to their disease process. It is important for the anaesthetist to be familiar with the pathophysiology of β-thalassaemia major and how the disease itself and its treatment or complications (iron deposition from multiple transfusions) can affect anaesthesia. A case of a 51-year-old woman with severe β-thalassaemia undergoing laparoscopic cholecystectomy is presented. The anaesthetic management and systematic review of the perioperative concerns in severe β-thalassaemia are discussed.

Keywords: Anaesthesia, B-thalassaemia, Laparoscopic Cholecystectomy, Non-cardiac Surgery, Pathophysiology