May 2008
Volume 49, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2008
In vivo Visualization of Photoreceptor Layer and Lipofuscin Accumulation in Stargardt’s Disease / Fundus Flavimaculatus by High Definition Optical Coherence Tomography
Author Affiliations & Notes
  • G. Querques
    University of Foggia, Foggia, Italy
    Ophthalmology, Ophthalmology,
    University of Paris XII, Creteil, France
  • R. Prato
    University of Foggia, Foggia, Italy
    Hygiene,
  • C. Iaculli
    University of Foggia, Foggia, Italy
    Ophthalmology, Ophthalmology,
  • A. V. Bux
    University of Foggia, Foggia, Italy
    Ophthalmology, Ophthalmology,
  • C. Angulo Bocco
    Ophthalmology, Ophthalmology,
    University of Paris XII, Creteil, France
  • N. Delle Noci
    University of Foggia, Foggia, Italy
    Ophthalmology, Ophthalmology,
  • G. Soubrane
    Ophthalmology, Ophthalmology,
    University of Paris XII, Creteil, France
  • E. H. Souied
    Ophthalmology, Ophthalmology,
    University of Paris XII, Creteil, France
  • Footnotes
    Commercial Relationships  G. Querques, None; R. Prato, None; C. Iaculli, None; A.V. Bux, None; C. Angulo Bocco, None; N. Delle Noci, None; G. Soubrane, None; E.H. Souied, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science May 2008, Vol.49, 2970. doi:https://doi.org/
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      G. Querques, R. Prato, C. Iaculli, A. V. Bux, C. Angulo Bocco, N. Delle Noci, G. Soubrane, E. H. Souied; In vivo Visualization of Photoreceptor Layer and Lipofuscin Accumulation in Stargardt’s Disease / Fundus Flavimaculatus by High Definition Optical Coherence Tomography. Invest. Ophthalmol. Vis. Sci. 2008;49(13):2970. doi: https://doi.org/.

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

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Abstract

Purpose: : To assess photoreceptor (PR) morphology in patients with Stargardt’s disease (STGD) and fundus flavimaculatus (FFM) using high definition optical coherence tomography (HD-OCT, OCT 4000 Cirrus, Humphrey-Zeiss, San Leandro, California).

Methods: : This was a prospective observational case series. Eight consecutive patients with STGD and FFM were prospectively examined by HD-OCT, a high-speed OCT system using spectral/Fourier domain detection, with an axial image resolution of 5 µm.

Results: : A total of 15 eyes were included in the study. HD-OCT was capable of visualizing regions of transverse PR loss in the foveal region. Moreover, we observed hyperreflective deposits which we classified in two types: type 1 lesions located in the inner part of the retinal pigment epithelium layer and type 2 lesions located at the level of the outer nuclear layer. Presence of type 1 lesion alone was associated with absence of complete loss of the PR layer in the foveal region in all eyes; presence of type 2 lesion was always associated with presence of type 1 lesions, and often (4/6 eyes) associated with complete loss of the PR layer within the foveal region. In addition, BCVA impairment showed a statistically significant correlation to the presence of complete loss of the PR layer in the foveal region (p=.0001), as well as to presence of type 2 flecks (p=.03).

Conclusions: : The possibility of in vivo visualization of the PR layer and of hyperreflective deposits in STGD/FFM might yield insight into genotypic/ phenotypic correlation, disease progression, and possibly treatment monitoring.

Keywords: imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • degenerations/dystrophies • retinal degenerations: hereditary 
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