May 2006
Volume 47, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2006
Corrugations of the RPE in Juvenile X– Linked Macular Retinoschisis
Author Affiliations & Notes
  • A. Agarwal
    Ophthalmology, Vanderbilt, Nashville, TN
  • Footnotes
    Commercial Relationships  A. Agarwal, None.
  • Footnotes
    Support  Challenge grant from Research to Prevent Blindness
Investigative Ophthalmology & Visual Science May 2006, Vol.47, 2793. doi:
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      A. Agarwal; Corrugations of the RPE in Juvenile X– Linked Macular Retinoschisis . Invest. Ophthalmol. Vis. Sci. 2006;47(13):2793.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : To describe a peculiar "Sea Gull" like corrugations of the retinal pigment epithelium (RPE) in 2 siblings with juvenile X– linked retinoschisis.

Methods: : Two siblings aged 10 and 18 at the time of their initial evaluation underwent clinical examination, fluorescein angiography and optical coherence tomography (OCT). The fundus appearance was correlated with the angiographic and OCT findings.

Results: : Both brothers presented with typical macular schisis. The OCT demonstrated cyst like spaces at the level of the outer plexiform layer and within the photoreceptor layer. Peculiar hitherto undescribed corrugations were seen temporal to the macula in both eyes of each patient. Fluorescein angiogram and OCT through these corrugations confirm their location to be at the level of the RPE.

Conclusions: : Whether forces generated by the progressive enlargement of the macular schisis imparts localized folds in the RPE temporal to it.

Keywords: macula/fovea • retinal detachment • inner retina dysfunction: hereditary 
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