|Year : 2022 | Volume
| Issue : 1 | Page : 329
Multimodal imaging of Bietti's retinal dystrophy
Sashwanthi Mohan, Namitha Mohan, Sujatha Mohan
Department of Vitreoretina, Rajan Eye Care Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||07-Jan-2022|
Dr. Sashwanthi Mohan
Department of Vitreoretina, Rajan Eye Care Hospital, No. 5, Vidyodaya East 2nd Street, T. Nagar, Chennai - 600 017, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mohan S, Mohan N, Mohan S. Multimodal imaging of Bietti's retinal dystrophy. Indian J Ophthalmol Case Rep 2022;2:329
Bietti's crystalline retinal dystrophy is an autosomal recessive disease caused by a mutation in the CYP4V2 gene. The left eye of a patient is featured here. The fundus showed glistening, yellow crystals deposited in the retina [Figure 1]a. Fundus autofluorescence showed hypoautofluorescence suggestive of retinal pigment epithelium (RPE) atrophy with surrounding granular hyperautofluorescence indicative of distressed retinal pigment epithelial cells [Figure 1]b. Optical coherence tomography (OCT) showed disruption of the outer retinal layers, outer retinal tubulations, RPE, and choroidal atrophy [Figure 1]c. OCT angiography of the choriocapillaris slab showed flow voids. Vessels from the deeper choroidal layer were also seen here due to increased penetration secondary to atrophy of RPE and choriocapillaris (arrow) [Figure 1]d.
|Figure 1: (a) Left eye fundus photo showing glistening, yellow crystals deposited in the retina. (b) Left eye fundus autofluorescence showing hypoautofluorescence in the area corresponding to the retinal crystals. (c) OCT showed disruption of the outer retinal layers, outer retinal tubulations, RPE and choroidal atrophy. (d) OCT (optical coherence tomography) angiography of the choriocapillaris slab showing flow voids. Vessels from the deeper choroidal layer are also seen here (arrow)|
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| References|| |
Lin J, Nishiguchi KM, Nakamura M, Dryja TP, Berson EL, Miyake Y. Recessive mutations in the CYP4V2 gene in East Asian and Middle Eastern patients with Bietti crystalline corneoretinal dystrophy. J Med Genet 2005;42:e38.