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Dark choroid sign in Stargardt disease

Eye (2025)Cite this article

Subjects

A 36-year-old female presented with defective vision OU for three years. Her best-corrected vision was 6/36 and the anterior segment was normal OU. The fundus evaluation revealed diffuse pigment mottling, retinal pigment epithelium (RPE) changes at the centre of the fovea, and fleck-like hypopigmented lesions throughout fundus OU. Optical coherence tomography (OCT) revealed RPE degeneration at the centre fovea. Fundus fluorescein angiography (FFA) revealed hyper-fluorescence in the centre of the fovea, a diffuse pisciform fleck-like hyper-fluorescence throughout the fundus, peripapillary dark choroid ring, and an absence of the choroidal fluorescence (dark choroid or silent choroid) OU (Fig. 1). The presence of these FFA findings helped in the diagnosis of Stargardt disease, in which the deposition of lipofuscin pigments in the retinal pigment epithelium causes the masking of choroidal fluorescence on FFA and the RPE atrophy causes the hyperfluorescence [1, 2]. The patient was advised guarded visual prognosis and was managed conservatively.

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Fig. 1: FFA of OU Stargardt disease.

References

  1. Jayasundera T, Rhoades W, Branham K, Niziol LM, Musch DC, Heckenlively JR. Peripapillary dark choroid ring as a helpful diagnostic sign in advanced Stargardt disease. Am J Ophthalmol. 2010;149:656–660.e2. https://doi.org/10.1016/j.ajo.2009.11.005.

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Authors and Affiliations

  1. Department of Vitreo-Retina, Aravind Eye Hospital, Madurai, Tamil Nadu, India

    Meri Debbarma, Muthukrishnan Vallinayagam, Chitaranjan Mishra & Naresh Babu Kannan

Authors
  1. Meri Debbarma
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  2. Muthukrishnan Vallinayagam
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  3. Chitaranjan Mishra
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  4. Naresh Babu Kannan
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CM drafted the manuscript. MD, MV, and NBK revised the manuscript. All authors have read and approved the final version of the manuscript.

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Correspondence to Chitaranjan Mishra.

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The authors declare no competing interests.

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Debbarma, M., Vallinayagam, M., Mishra, C. et al. Dark choroid sign in Stargardt disease. Eye (2025). https://doi.org/10.1038/s41433-025-03676-7

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