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Insights into autofluorescence patterns in Stargardt macular dystrophy using ultra-wide-field imaging

  • Retinal Disorders
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To characterize autofluorescence (AF) patterns occurring in Stargardt macular dystrophy (STGD1) using ultra-wide-field (UWF) imaging.

Methods

This paper is a cross-sectional observational study of 22 eyes of 11 patients (mean age 23.44 years) with Stargardt disease-fundus flavimaculatus who presented with decrease of vision at a tertiary eye care center. UWF short-wave AF images were obtained from all the patients using an Optos TX200 instrument. The main outcome measures were to assess patterns of AF changes seen on UWF AF imaging.

Results

All eyes showed a central area of hypoautofluorescence at the macula along with retinal flecks extending centrifugally as well as to the nasal side of the optic disc. Peripapillary sparing was seen in 100% of the eyes. Flecks were seen to be hypoautofluorescent in the center and hyperautofluorescent in the periphery in 77.8% eyes and were only hyperfluorescent in 27.2%. A background-increased fluorescence was visible in 100% of eyes, the outer boundary of which was marked by distribution of flecks in 81.9% eyes. A characteristic inferonasal vertical line was seen separating the nasal hypoautofluorescent area from the temporal hyperautofluorescent area in all the eyes.

Conclusions

UWF AF changes in STGD1 are not limited to the posterior pole and may extend more peripherally. UWF imaging is a useful tool for the assessment of patients with Stargardt macular dystrophy.

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References

  1. Roosing S, Thiadens AA, Hoyng CB, Klaver CC, den Hollander AI, Cremers FP (2014) Causes and consequences of inherited cone disorders. Prog Retin Eye Res 42:1–26

    Article  CAS  PubMed  Google Scholar 

  2. Allikmets R, Singh N, Sun H et al (1997) A photoreceptor cell specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy. Nat Genet 15:236–246

    Article  CAS  PubMed  Google Scholar 

  3. Mata NL, Weng J, Travis GH (2000) Biosynthesis of a major lipofuscin fluorophore in mice and humans with ABCR-mediated retinal and macular degeneration. Proc Natl Acad Sci U S A 97:7154–7159

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Ablonczy Z, Higbee D, Anderson DM, Dahrouj M, Grey AC, Gutierrez D et al (2013) Lack of correlation between the spatial distribution of A2E and lipofuscin fluorescence in the human retinal pigment epithelium. Invest Ophthalmol Vis Sci 54:5535–5542

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Smith RT, Gomes NL, Barile G, Busuioc M, Lee N, Laine A (2009) Lipofuscin and autofluorescence metrics in progressive STGD. Invest ophthalmol Vis Sci 50:3907–3914

    Article  PubMed  PubMed Central  Google Scholar 

  6. Hwang JC, Chan JW, Chang S, Smith RT (2006) Predictive value of fundus autofluorescence for development of geographic atrophy in age-related macular degeneration. Invest Ophthalmol Vis Sci 47:2655–2661

    Article  PubMed  PubMed Central  Google Scholar 

  7. Burke TR, Duncker T, Woods RL, Greenberg JP, Zernant J, Tsang SH, Smith RT, Allikmets R, Sparrow JR, Delori FC (2014) Quantitative fundus autofluorescence in recessive Stargardt disease. Invest Ophthalmol Vis Sci 55:2841–2852

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Fujinami K, Lois N, Mukherjee R et al (2013) A longitudinal study of Stargardt disease: quantitative assessment of fundus autofluorescence, progression, and genotype correlations. Invest Ophthalmol Vis Sci 54:8181–8190

    Article  PubMed  Google Scholar 

  9. Choudhry N, Giani A, Miller JW (2010) Fundus autofluorescence in geographic atrophy: a review. Semin Ophthalmol 25:206–213

    Article  PubMed  Google Scholar 

  10. von Ruckmann A, Fitzke FW, Bird AC (1995) Distribution of fundus autofluorescence with a scanning laser ophthalmoscope. Br J Ophthalmol 79:407–412

    Article  Google Scholar 

  11. Gomes NL, Greenstein VC, Carlson JN et al (2009) A comparison of fundus autofluorescence and retinal structure in patients with Stargardt disease. Invest Ophthalmol Vis Sci 50:3953–3959

    Article  PubMed  PubMed Central  Google Scholar 

  12. Cukras CA, Wong WT, Caruso R, Cunningham D, Zein W, Sieving PA (2012) Centrifugal expansion of fundus autofluorescence patterns in Stargardt disease over time. Arch Ophthalmol 130:171–179

    Article  PubMed  Google Scholar 

  13. Duncker T, Marsiglia M, Lee W et al (2014) Correlations amongst near-infrared and short-wavelength autofluorescence and spectral domain optical coherence tomography in recessive Stargardt disease. Invest Ophthalmol Vis Sci 55:8134–8143

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Patel M, Kiss S (2014) Ultra-wide-field fluorescein angiography in retinal disease. Curr Opin Ophthalmol 25:213–220

    Article  PubMed  Google Scholar 

  15. Eagle RC Jr, Lucier AC, Bernardino VB Jr, Yanoff M (1980) Retinal pigment epithelial abnormalities in fundus flavimaculatus: a light and electron microscopic study. Ophthalmology 87:1189–1200

    Article  PubMed  Google Scholar 

  16. Delori FC, Staurenghi G, Arend O, Dorey CK, Goger DG, Weiter JJ (1995) In vivo measurement of lipofuscin in Stargardt’s disease-fundus flavimaculatus. Invest Ophthalmol Vis Sci 36:2327–2331

    CAS  PubMed  Google Scholar 

  17. von Ruckmann A, Fitzke FW, Bird AC (1997) In vivo fundus autofluorescence in macular dystrophies. Arch Ophthalmol 115:609–615

    Article  Google Scholar 

  18. Duncker T, Lee W, Tsang SH, Greenberg JP, Zernant J, Allikmets R, Sparrow JR (2013) Distinct characteristics of Inferonasal fundus autofluorescence patterns in Stargardt disease and retinitis Pigmentosa. Invest Ophthalmol Vis Sci 54:6820–6826

    Article  PubMed  PubMed Central  Google Scholar 

  19. Duncker T, Greenberg JP, Sparrow JR, Smith RT, Quigley HA, Delori FC (2012) Visualization of the optic fissure in short-wavelength autofluorescence images of the fundus. Invest Ophthalmol Vis Sci 53:6682–6686

    Article  PubMed  PubMed Central  Google Scholar 

  20. Sparrow JR, Marsiglia M, Allikmets R, Tsang S, Lee W, Duncker T, Zernant J (2015) Flecks in recessive Stargardt disease: short-wavelength autofluorescence, near-infrared autofluorescence, and optical coherence tomography imaging of flecks. Invest Ophthalmol Vis Sci 56:5029–5039

    Article  PubMed  PubMed Central  Google Scholar 

  21. Burke TR, Rhee DW, Smith RT, Tsang SH, Allikmets R, Chang S, Lazow MA, Hood DC, Greenstein VC (2011) Quantification of peripapillary sparing and macular involvement in Stargardt disease (STGD1). Invest Ophthalmol Vis Sci 52:8006–8015

    Article  PubMed  PubMed Central  Google Scholar 

  22. Klufas MA, Tsui I, Sadda SR, Hosseini H, Schwartz SD (2017) Ultrawidefield autofluoresence in ABCA4 Stargardt disease. Retina. doi:10.1097/IAE.0000000000001567

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Authors and Affiliations

  1. Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, 57, Sadar Apartments, Mayur Vihar Phase 1 extension, New Delhi, 110091, India

    Vinod Kumar

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  1. Vinod Kumar
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Correspondence to Vinod Kumar.

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No funding was received for this research.

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All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

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All procedures performed in this study were in accordance with the ethical standards of the institution and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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For this type of study, formal consent is not required.

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Kumar, V. Insights into autofluorescence patterns in Stargardt macular dystrophy using ultra-wide-field imaging. Graefes Arch Clin Exp Ophthalmol 255, 1917–1922 (2017). https://doi.org/10.1007/s00417-017-3736-4

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  • DOI: https://doi.org/10.1007/s00417-017-3736-4

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