DR. MOAZZAM PARVEZ

DR. ZEENAL DABRE, Dr. MD. PARVEZ ALAM, DR. SNEHA JAIN

Abstract

Best disease or vitelliform macular dystrophy is a rare autosomal dominant disease with bilateral subretinal accumulation of yellowish material around macula.

We report 4 cases of Best disease in siblings complaining of reduced visual acuity in both eyes for several months.Two of them were males and 2 females.Both Parents had normal visual acuity and fundus. BCVA of all 4 patients was between 6/60 and 6/24.Fundus examination of all 4 patients showed macular lesions suggestive of Best Disease.ERG was normal,however EOG of all four patients showed striking reduction(Arden index <1.5) . OCT of both eyes revealed deposits of homogenous hyper-reflective material beneath retinal pigment epithelium at fovea.FFA showed blocked fluorescence at the site of vitelliform lesion of both eyes.Routine blood investigation,physician consultation were within normal limits.We only observe these patients every 6 months as no treatment is available at present.

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