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Handbook of Pediatric Retinal Disease pp 96–134Cite as

Heritable Disorders of RPE, Bruch’s Membrane, and the Choriocapillaris

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Abstract

This chapter covers disorders characterized by ophthalmoscopically visible changes in structures deep to the neurosensory retina. A prominent component of many of these conditions is the accumulation of yellowish material within and beneath the retinal pigment epithelium (RPE) associated with a progressive loss of macular RPE cells. A number of toxic and inflammatory conditions can also cause dots and spots at the level of the RPE, but these conditions can usually be distinguished by history from those in this chapter and are considered elsewhere in this volume (Chapter 11). Choroideremia, gyrate atrophy, and some forms of congenital stationary night blindness are also associated with ophthalmoscopically visible abnormalities in structures beneath the photoreceptors. However, these diseases share some psychophysical, electrophysiological, and symptomatic features with the photoreceptor degenerations and are discussed in Chapter 5. Last, myopia (Chapter 12) can be associated with several abnormalities at the level of the RPE that can be ophthalmoscopically similar to the entities discussed in this chapter.

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Authors
  1. Arlene V. Drack
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Editors and Affiliations

  1. Wright Foundation for Pediatric Ophthalmology, Los Angeles, CA, USA

    Kenneth W. Wright MD (Director and Clinical Professor of Ophthalmology) (Director and Clinical Professor of Ophthalmology)

  2. Pediatric Ophthalmology, Cedars-Sinai Medical Center, Los Angeles, CA, USA

    Kenneth W. Wright MD (Director and Clinical Professor of Ophthalmology) (Director and Clinical Professor of Ophthalmology)

  3. University of Southern California—Keck School of Medicine, Los Angeles, CA, USA

    Kenneth W. Wright MD (Director and Clinical Professor of Ophthalmology) (Director and Clinical Professor of Ophthalmology)

  4. Focus On You, Inc., Palm Desert, CA, USA

    Peter H. Spiegel MD

  5. Inland Eye Clinic, Murrieta, CA, USA

    Peter H. Spiegel MD

  6. Children’s Eye Institute, Upland, CA, USA

    Peter H. Spiegel MD

  7. Stroger Hospital of Cook County, Chicago, IL, USA

    Lisa S. Thompson MD (Attending Physician) (Attending Physician)

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Drack, A.V. (2006). Heritable Disorders of RPE, Bruch’s Membrane, and the Choriocapillaris. In: Wright, K.W., Spiegel, P.H., Thompson, L.S. (eds) Handbook of Pediatric Retinal Disease. Springer, New York, NY. https://doi.org/10.1007/0-387-27933-4_4

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  • DOI: https://doi.org/10.1007/0-387-27933-4_4

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