Dr. Saranya D. V. , S17933, Dr. (Mrs ) P S Maheswari
An 8 years old boy presented with complaints of defective vision BE. He is a third born child of second degree consanguineous marriage. On examination vision in RE: 1/60 LE: 3/60 with RE dislocated lens in anterior chamber and LE inferior subluxation of lens. Patient had records of previous herniotomy surgery and treated for megaloblastic anemia. Investigations were done to confirm diagnosis of homocystinuria. Investigations showed elevated serum homocystine levels and positive cyanide nitroprusside test. UBM LE showed lens subluxation with zonular dialysis. CT spine showed D4-D5 intervertebral disc prolapse. Planned for RE lens removal with IOL implantation and LE pars plana vitrectomy with lensectomy with IOL implantation . This case is presented to emphasis the importance of correlation of clinical findings with other systemic features to clinch the etiology of ectopia lentis.