Abstract
IntroductionProgressive night blindness and constriction of the visual fields are features of Bietti crystalline corneoretinal dystrophy, but here we report a case with the most probable diagnosis of Bietti crystalline dystrophy and good central visual acuity and severely decreased electroretinogram.Case presentationThe patient was a 28-year-old woman with complaint of decreased vision without night blindness. Her both eyes visual acuity were 20/25 with plano refraction. Fundus examination showed intraretinal crystals distributed in the posterior pole and also midperiphery. Fullfield electroretinogram showed decreased scotopic a and b-wave amplitudes.ConclusionIn our patient central foveal region was relatively intact; and this can explain subnormal visual acuity. Although visual acuity was nearly spared, electroretinogram was extremely affected.
Highlights
Introduction: Progressive night blindness and constriction of the visual fields are features of Bietti crystalline corneoretinal dystrophy, but here we report a case with the most probable diagnosis of Bietti crystalline dystrophy and good central visual acuity and severely decreased electroretinogram
Fundus examination showed intraretinal crystals distributed in the posterior pole and midperiphery
Bietti crystalline corneoretinal dystrophy (BCD) is an autosomal recessive disorder of retinal degeneration characterized by numerous tiny sparkling yellow-white spots at the posterior pole of the fundus
Summary
Bietti crystalline corneoretinal dystrophy (BCD) is an autosomal recessive disorder of retinal degeneration characterized by numerous tiny sparkling yellow-white spots at the posterior pole of the fundus. We reported a case with the most probable diagnosis of Bietti crystalline dystrophy (BCD). To our knowledge, this is the first case report of this denegation in Iran. Case presentation A 28-year-old Iranian Caucasian woman referred to our clinic with complaints of decreased vision without night blindness and visual acuity of both eyes was 20/25 with plano refraction. Her slit lamp examination revealed clear cornea and clear lens, and 1+ pigment in vitreous. Visual field testing with program SITA fast 24 -2 was performed and showed constriction of visual field bilaterally (Figure 3)
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