AF in Myriad Retinal Degenerations- Part 1 – Page 6 of 57

Case 2: 17 yo WF presented for follow up of Lebers Congenital Amarosis (LCA). Visual problems were noticed when the patient was 4 months old and the patient was diagnosed with LCA at 7 months old. The patient has had long standing reduced vision and BCVA are now 20/50-2 OD and 20/60-2 OS, which are surprisingly good in LCA. Flash ERGs a decade earlier were flat in both eyes. (Flat ERGs are always a poor prognostic sign). Goldmann visual fields were restricted both OD and OS paralleling the patients peripheral visual complaints. Optos Ultra-Widefield confirmed mid-peripheral bone spicule pigmentation noted by previous DFEs.