Dr.Ajit Kumar Sinha, Dr. NEHA KHANNA, Dr.Nangia Vinay Kumar B, Dr.Pooja Mokashi,Tuhina kunwar
Background: A 29 year old female, diagnosed case of Marfans syndrome came with gradual progressive diminution of vision in BE since 2 years. On examination, BCVA was 6/9 in BE with -14 DS / -2 DC at 90’. Slit lamp revealed supero-temporal subluxated clear lens (7 clock hours) with intact, stretched zonules. She refused intervention then. 2 years later, she presented with reduced VA of 6/36 in BE. Clear lens extraction by phaco-aspiration with cionni’s ring fixation, intra-ocular lens implantation in the bag was done in BE.
Result: Post-operatively she attained VA of 6/6 in BE with well centred stable intraocular lens.
Discussion: Marfans syndrome is an autosomal dominant disorder, involving the fibrillin gene on chromosome 15, associated with ocular, cardiac, skeletal manifestations. Crystalline lens is subluxated supero-temporally, with intact zonules. In our case the subluxated lens induced a significant refractive error, hence, surgical intervention was justified.