American Journal of Ophthalmology
Volume 149, Issue 4 , Pages 656-660.e2 , April 2010

Peripapillary Dark Choroid Ring as a Helpful Diagnostic Sign in Advanced Stargardt Disease

  • Thiran Jayasundera

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • ,
  • William Rhoades

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • ,
  • Kari Branham

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • ,
  • Leslie M. Niziol

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • ,
  • David C. Musch

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • Department of Epidemiology, School of Public Health, University of Michigan, Ann Arbor, Michigan
    • ,
  • John R. Heckenlively

      Affiliations

    • Department of Ophthalmology and Visual Sciences, Medical School, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan
    • Corresponding Author InformationInquiries to John R. Heckenlively, Kellogg Eye Center, University of Michigan, 1000 Wall Street, Ann Arbor, MI 48105

,Accepted 1 November 2009.

References 

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  2. Westerfeld C, Mukai S. Stargardt's disease and the ABCR gene. Semin Ophthalmol. 2008;23:59–65
  3. Beharry S, Zhong M, Molday RS. N-retinylidene-phosphatidylethanolamine is the preferred retinoid substrate for the photoreceptor-specific ABC transporter ABCA4 (ABCR). J Biol Chem. 2004;279:53972–53979
  4. Cideciyan AV, Aleman TS, Swider M, et al. Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence. Hum Mol Genet. 2004;13:525–534
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  6. Wu Y, Fishkin NE, Pande A, Pande J, Sparrow JR. Novel lipofuscin bisretinoids prominent in human retina and in a model of recessive Stargardt disease. J Biol Chem. 2009;284:20155–20166
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  13. Rotenstreich Y, Fishman GA, Anderson RJ. Visual acuity loss and clinical observations in a large series of patients with Stargardt disease. Ophthalmology. 2003;110:1151–1158
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  17. Gelisken O, De Laey JJ. A clinical review of Stargardt's disease and/or fundus flavimaculatus with follow-up. Int Ophthalmol. 1985;8:225–235
  18. Cideciyan AV, Swider M, Aleman TS, et al. ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina. Invest Ophthalmol Vis Sci. 2005;46:4739–4746
  19. Curcio CA, Sloan KR, Kalina RE, Hendrickson AE. Human photoreceptor topography. J Comp Neurol. 1990;292:497–523
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PII: S0002-9394(09)00807-1

doi: 10.1016/j.ajo.2009.11.005

American Journal of Ophthalmology
Volume 149, Issue 4 , Pages 656-660.e2 , April 2010

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