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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 427-428

The bejewelled retinal fireworks of flecked retina syndrome: A multiplexed retinal imaging


1 Medical Officer, Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
2 Medical Officer, Department of Glaucoma and Research, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
3 Head of the Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
4 Chief Medical Officer, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
5 Medical Officer, Department of Vitreo- Retinal Services, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India

Date of Submission22-Aug-2020
Date of Acceptance29-Jan-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Prasanna Venkatesh Ramesh
Mahathma Eye Hospital Private Limited, No 6, Tennur, Seshapuram, Trichy - 620 017, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2738_20

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  Abstract 


Keywords: Flecked retina syndrome, flecked retina, multi-modal retinal imaging


How to cite this article:
Ramesh SV, Ramesh PV, Ramesh MK, Rajasekaran R, Balamurugan A. The bejewelled retinal fireworks of flecked retina syndrome: A multiplexed retinal imaging. Indian J Ophthalmol Case Rep 2021;1:427-8

How to cite this URL:
Ramesh SV, Ramesh PV, Ramesh MK, Rajasekaran R, Balamurugan A. The bejewelled retinal fireworks of flecked retina syndrome: A multiplexed retinal imaging. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:427-8. Available from: https://www.ijoreports.in/text.asp?2021/1/3/427/320010



A 31-year-old male patient came to the hospital for routine evaluation. On examination, his best-corrected visual acuity in OS was 20/20. He had no history of defective vision in dim lighting. Anterior segment examination was normal. There was no history of consanguineous marriage between his parents. Confocal fundus evaluation (Eidon, CenterVue, Italy) revealed porcelain-white spots extending anterior and posterior to the vasculature sparing the macula in both eyes [Figure 1]. The optic discs and retinal vessels were normal and there were no peripheral pigmentary degenerative changes. Fundus autofluorescence (FAF) shows diffuse foci of hyper-autofluorescence scattered throughout the posterior pole associated with the white spots [Figure 2]. Infrared (IR) reflectance image showing numerous white spots which appeared to be partially or totally reflective on IR imaging extending anterior and posterior to the vasculature sparing the macula [Figure 3]. Spectral-domain optical coherence tomography SD-OCT (Spectralis, Heidelberg Engineering, Germany) revealed normal macula anatomy with homogenous dome-shaped lesions originating from the retinal pigment epithelial (RPE) layer [Figure 4]. These lesions extend from the RPE level into the inner segment/outer segment (IS/OS) junction of the photoreceptors, external limiting membrane, and into the outer nuclear layer. Posterior pole FAF and IR images [Figure 5] were also obtained with Spectralis optical coherence tomography (OCT).
Figure 1: (a and b) Mosaic view of confocal colour fundus photograph of OD and OS respectively showing white dots scattered throughout the posterior pole sparing the macula

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Figure 2: (a and b) Mosaic view of fundus autofluorescence image of OD and OS respectively showing multiple hyper-autofluorescent spots scattered throughout the posterior pole, which were associated with the white spots seen on the colour fundus photograph

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Figure 3: (a and b) Mosaic view of infrared fundus image of OD and OS respectively showing numerous spots with partial or total reflectivity along the posterior pole sparing the macula

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Figure 4: (a and b) SD-OCT of OD in normal and inverse colour respectively, showing healthy macula with dome shaped elevations arising from the retinal pigment epithelium (RPE) into the IS/OS junction, external limiting membrane and sometimes even extending into the outer nuclear layer in few regions (red arrows). (c and d) SD-OCT of OS in normal and inverse colour respectively, showing healthy macula with dome shaped elevations arising from the retinal pigment epithelium (RPE) into the IS/OS junction, external limiting membrane and sometimes even extending into the outer nuclear layer in few regions (red arrows)

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Figure 5: Fundus autofluorescence (FAF) and infrared (IR) image taken using spectralis OCT. (a and b) FAF image of OD and OS respectively showing hyper-autofluorescence spots along the posterior pole sparing the macula. (c and d) IR image of OD and OS respectively showing numerous spots with partial or total reflectivity along the posterior pole sparing the macula

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  Discussion Top


Flecked retina syndromes encompass a group of diseases that include Stargardt's macular dystrophy, fundus albipunctatus, retinitis punctata albescens, Leber congenital amaurosis, pseudoxanthoma elasticum, Kjellin's syndrome, Alport's syndrome, Sjögren-Larsson syndrome, Bietti's crystalline dystrophy, oxalosis, and cystinosis.[1],[2],[3] The use of multimodal imaging are of important value in monitoring these patients for structural changes over time; and also for monitoring their changes during future treatment trials.[4]

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Genead MA, Fishman GA, Lindeman M. Spectral-domain optical coherence tomography and fundus autofluorescence characteristics in patients with fundus albipunctatus and retinitis punctata albescens. Ophthalmic Genet 2010;31:66-72.  Back to cited text no. 1
    
2.
Garcia JMBB, Isaac DLC, Sardeiro T, Aquino É, Avila M. Benign familial fleck retina: Multimodal imaging including optical coherence tomography angiography. Arq Bras Oftalmol 2017;80:321-3.  Back to cited text no. 2
    
3.
Sergouniotis PI, Davidson AE, Mackay DS, Lenassi E, Li Z, Robson AG, et al. Biallelic mutations in PLA2G5, encoding group V phospholipase A2, cause benign fleck retina. Am J Hum Genet 2011;89:782-91.  Back to cited text no. 3
    
4.
Bin NJ, Heng HM, Poh R, Noor SM, Subrayan V. Phospholipase A2 group v in benign familial fleck retina in a set of triplets. Retina 2015;35:1266-72.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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