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Year : 2022  |  Volume : 60  |  Issue : 1  |  Page : 128-129

Macular cysts in bietti crystalline dystrophy

Department of Retina and Vitreous, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India

Date of Submission06-Mar-2021
Date of Decision12-Nov-2021
Date of Acceptance07-Dec-2021
Date of Web Publication22-Mar-2022

Correspondence Address:
Dr. Jahnara Jaffar
Department of Retina and Vitreous, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli - 627 001, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_23_21

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Bietti crystalline dystrophy (BCD) is a rare autosomal disorder characterized by the presence of intraretinal crystalline deposits and later chorioretinal degeneration. We describe a case of BCD with macular cysts. Macular cysts have been reported in many retinal dystrophies such as Juvenile X-linked retinoschisis, Goldmann-Favre Syndrome, and fenetrated sheen dystrophy. BCD with macular cysts is rarely reported in the literature.

Keywords: Bietti crystalline dystrophy, macular cysts, optical coherence tomography

How to cite this article:
Abdul Khadar SM, Jaffar J, Shetty AP. Macular cysts in bietti crystalline dystrophy. TNOA J Ophthalmic Sci Res 2022;60:128-9

How to cite this URL:
Abdul Khadar SM, Jaffar J, Shetty AP. Macular cysts in bietti crystalline dystrophy. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Jun 29];60:128-9. Available from: https://www.tnoajosr.com/text.asp?2022/60/1/128/340360

  Introduction Top

Bietti crystalline dystrophy (BCD) was first described by Bietti in 1937 as an autosomal disorder characterized by intraretinal crystalline deposits associated with sclerosis of choroidal vessels and retinal pigment epithelial atrophy. Few case reports have reported the association of macular edema in BCD. We report the case of a middle-aged female with macular cysts in BCD.

  Case Report Top

A 39-year-old female presented to our clinic with complaints of gradual diminution of vision in both eyes for the past 2 years. On examination, her best-corrected visual acuity was 20/120 in the right eye and 20/30 in the left eye. Anterior segment examination was normal in both eyes. Fundus examination showed the presence of multiple crystalline deposits over the posterior pole extending till mid periphery in both eyes. There was associated retinal thinning as well [Figure 1]a and [Figure 1]b.
Figure 1: Fundus photo of the right eye (a) and left eye (b) showing multiple intraretinal crystalline deposits

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Standard deviation optical coherence tomography imaging showed altered foveal contour in the right eye and a relatively well preserved fovea in the left eye [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. There was atrophy of the rating of perceived exertion (RPE), choriocapillaries, and loss of ellipsoid zone in both eyes. Multiple hyperreflective spots at the level of RPE-Bruchs complex corresponding to the crystalline deposits were noted. There were multiple outer retinal tubulations. In addition, we noticed multiple cystoid spaces at the level of inner nuclear layer and ganglion cell layer. These cystic spaces were more localized to those areas where there was severe outer retinal atrophy. Based on the fundus and OCT findings, a diagnosis of BCD was made. Other causes of retinal crystalline retinopathy were ruled out by history and blood investigations. The visual prognosis was explained to the patient and she was advised to come for periodic follow-up.
Figure 2: (a and b) Standard deviation optical coherence tomography images of the right eye, (b and c) Standard deviation optical coherence tomography images of the left eye. Blue arrows show the inner retinal cysts. Note the marked outer retinal layer atrophy in area of these cysts. Red arrows show hyperreflectivity at the level of Bruch rating of perceived exertion complex representing crystals. White arrows show outer retinal tubulations. Note the marked atrophy of choriocapillary layer and retinal pigment epithelium

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  Discussion Top

RPE atrophy and photoreceptor loss are the major causes of vision loss in patients with BCD. Subretinal choroidal neovascular membrane, subfoveal neurosensory detachment, macular hole, and macular edema are the other rare associations that has been reported in the literature.[1],[2],[3]

Ganesh et al.[4] have described an entity called non-CME macular cysts in retinal dystrophies such as Goldman Favre syndrome, X-linked juvenile retinoschisis, fenestrated sheen dystrophy, and many other conditions. They have differentiated it from macular edema by the absence of leakage on fundus fluorescein angiography. The formation of these cysts can be attributed to tissue loss and disruption in the normal retinal architecture rather than vascular leakage. This corroborates our finding of the localization of these cysts in areas with severe atrophy of the outer layers.

The cysts in our case were parafoveal and were not the cause of vision loss. No treatment was given for our case.

  Conclusion Top

Macular cysts can occur in BCD like many other dystrophies. There are no clear guidelines regarding the management of these macular changes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Saatci AO, Yaman A, Berk AT, Söylev MF. Macular hole formation in bietti's crystalline retinopathy. A case report. Ophthalmic Genet 1997;18:139-41.  Back to cited text no. 1
Li Q, Li Y, Zhang X, Xu Z, Zhu X, Ma K, et al. Utilization of fundus autofluorescence, spectral domain optical coherence tomography, and enhanced depth imaging in the characterization of bietti crystalline dystrophy in different stages. Retina 2015;35:2074-84.  Back to cited text no. 2
Khojasteh H, Roohipoor R, Riazi-Esfahani H, Ghasempour M. Topical Dorzolamide For Cystoid Macular Edema In Bietti Crystalline Retinal Dystrophy. Retin Cases Brief Rep. 2021 May 1;15(3):306-309.  Back to cited text no. 3
Ganesh A, Stroh E, Manayath GJ, Al-Zuhaibi S, Levin AV. Macular cysts in retinal dystrophy. Curr Opin Ophthalmol 2011;22:332-9.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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