ABSTRACT

Homocystinuria is an autosomal recessive metabolik disorder. Patients with homocystinuria, a rare inborn error of aminoacid metabolism, may present with subluxation of the lens or glaucoma. These patients are susceptible to thromboembolic complications during general anesthesia. Hyperinsulinemia and hypoglycemic convulsions are also common. In this case we report a 11 year old boy with homocystinuria who required an ocular surgery including lentectomy under general anesthesia because of lens subluxation.