Perioperative Management of a Paediatric Patient with Wilson’s Disease for Cardiac Surgery

Wilson’s disease is a rare autosomal recessive disorder of copper metabolism due to a deficiency of the copper transportation protein, resulting in toxic accumulation of copper in the liver, brain, and other organs. An excess of copper deposition in the liver can cause chronic liver disease and cirrhosis. Anaesthesia management during open-heart surgery in such children is challenging, as most of the anaesthesia agents are metabolised in the liver. We herewith present a 6-year-old child with Wilson’s disease who underwent successful closure of the Ventricular Septal Defect (VSD).