Anaesthesia challenges in Wilson’s disease with multisystem involvement

Abstract

Wilson’s disease is a rare genetic disorder with a prevalence rate of 1:10000-1:30000. It is a multi- system disorder with a defect in the copper transporter protein; thereby resulting in extortionate copper deposition in organs of the body; mainly liver, brain and cornea. Osteo-muscular involvement may also be seen, presenting as dystonia and muscle rigidity. The case discussed below is that of a 13 year old patient, ASA III E, diagnosed with Wilson’s disease 7 years back and now is posted for emergency tracheostomy under anaesthesia in view of chest rigidity, difficulty in breathing and repeated episodes of bronchospasm. The patient presented in late stage of the disorder with associated derangements of multiple systems of the body. This case report provides details of successful administration of Anaesthesia to a patient with Wilson's in late phase. The management protocol can be used as a guide in other surgeries of patients with this disorder, as precise titration of anaesthesia is needed.