Homocystinuria is due to deficiency of cystathionine bet synthetase. It is an autosomal recessive disorder of methionine metabolism with an increase of 1 in 52 544 births. Ocular manifestations in untreated cases are Ectopia lentis Dislocation of lens which is down and in Sec glaucoma Optic atrophy Retinal detachment The treatment is dietary treatment and vitamin supplementation.

Ocular complications can be reduced substantially if treatment is started within 6 weeks of birth. Share your views.