Abstract :
Wilson’s disease is a rare genetic disorder with a prevalence rate of 1:10000-1:30000. 1 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. 2 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.
Keyword :
Wilson’s, Multi-system, Bronchospasm, Muscle rigidity, Liver, Osteo-muscular.