Abstract

A 12 year old female child presented with complaints of defective vision in RE)for past 4 weeks. History of seizures present 7 yrs ago followed which she was diagnosed as Moya Moya disease in association with Neurofibromatosis 1 . History of NF 1 in parents. Further Bypass graft surgery done between MCA and Superficial temporal artery 5 years ago.On examination RE) Anterior segment normal LE) Lisch nodules present otherwise normal.UCVA RE)6/36NIP LE)6/12 with pH 6/6 .IOP in BE) 12 mmhg. Fundus in RE) shows white sheathing of vessels in IT quadrant with extensive collaterals between superior and inferior arcade diagnosis as old vascular occlusive sequlae with extensive collaterals.
FFA shows RE)Delayed phase patchy filling of retinal vessels with capillary nonperfusion temporal fovea and extensive collaterals .
Since extensive collaterals with ischemic changes sparing vision loss, capillary nonperfusion areas planned for targeted retinal photocoagulation and patient is on followup