case of homocystinuria: anesthetic implications

We report a case of 12 yr. old male child who presented with diminshed vision for 3-4 years and was found to have supranasal subluxation of lens. He was suspected to have homocystinuria and confirmed by high serum homocysteine level. Homocystinuria is second most common inherited amino acid metabolic disorder, associated with cystathionine-beta-synthetase deficiency. After reducing his serum homocysteine level with medication, patient was taken up for surgery under general anesthesia. During perioperative period prolonged fasting hours and use of nitrous oxide were avoided and use of pneumatic compressions was taken to avoid the various complications and mortality associated with this syndrome