ANAESTHETIC MANAGEMENT OF A CASE OF HOMOCYSTINURIA

Homocystinuria is an inherited metabolic disorder associated with cystathionine - beta-synthase deficiency. A 6 yr. old male child presented with the complaints of diminshed vision and was found to have ectopia lentis. He was suspected to have homocystinuria due to his clinical findings and this was confirmed by his serum homocysteine level. He was started on medication to reduce homocysteine levels and then was taken up for surgery i. e. pars plana vitrectomy with lensectomy with scleral fixation under general anaesthesia. Precautions were taken in the perioperative period to avoid the various complications and mortality associated with this syndrome. Nitrous oxide was avoided in the anaesthetic technique intraoperatively The patient was fine postoperatively.