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Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit

PURPOSE: To examine the foveal avascular zone (FAZ) in patients with congenital achromatopsia (ACHM). METHODS: Forty-two patients with genetically confirmed ACHM were imaged either with Optovue's AngioVue system or Zeiss's Plex Elite 9000, and the presence or absence of a FAZ was determine...

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Autores principales: Linderman, Rachel E., Georgiou, Michalis, Woertz, Erica N., Cava, Jenna A., Litts, Katie M., Tarima, Sergey, Rajendram, Ranjan, Provis, Jan M., Michaelides, Michel, Carroll, Joseph
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Association for Research in Vision and Ophthalmology 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7463179/
https://www.ncbi.nlm.nih.gov/pubmed/32866266
http://dx.doi.org/10.1167/iovs.61.10.52
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author Linderman, Rachel E.
Georgiou, Michalis
Woertz, Erica N.
Cava, Jenna A.
Litts, Katie M.
Tarima, Sergey
Rajendram, Ranjan
Provis, Jan M.
Michaelides, Michel
Carroll, Joseph
author_facet Linderman, Rachel E.
Georgiou, Michalis
Woertz, Erica N.
Cava, Jenna A.
Litts, Katie M.
Tarima, Sergey
Rajendram, Ranjan
Provis, Jan M.
Michaelides, Michel
Carroll, Joseph
author_sort Linderman, Rachel E.
collection PubMed
description PURPOSE: To examine the foveal avascular zone (FAZ) in patients with congenital achromatopsia (ACHM). METHODS: Forty-two patients with genetically confirmed ACHM were imaged either with Optovue's AngioVue system or Zeiss's Plex Elite 9000, and the presence or absence of a FAZ was determined. For images where a FAZ was present and could be confidently segmented, FAZ area, circularity index, and roundness were measured and compared with previously published normative values. Structural optical coherence tomography images were acquired to assess the degree of foveal hypoplasia (number and thickness of inner retinal layers present at the fovea). RESULTS: A FAZ was present in 31 of 42 patients imaged (74%), although no determination could be made for 11 patients due to poor image quality (26%). The mean ± SD FAZ area for the ACHM retina was 0.281 ± 0.112 mm(2), which was not significantly different from the previously published normative values (P = 0.94). However, their FAZs had decreased circularity (P < 0.0001) and decreased roundness (P < 0.0001) compared to the normative cohort. In the patients with ACHM examined here, the FAZ area decreased as the number and thickness of the retained inner retinal layers increased. CONCLUSIONS: Our data demonstrate that despite the presence of foveal hypoplasia, patients with ACHM can have a FAZ. This is distinct from other conditions associated with foveal hypoplasia, which generally show an absence of the FAZ. In ACHM, FAZ formation does not appear to be sufficient for complete pit formation, contrary to some models of foveal development.
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spelling pubmed-74631792020-09-14 Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit Linderman, Rachel E. Georgiou, Michalis Woertz, Erica N. Cava, Jenna A. Litts, Katie M. Tarima, Sergey Rajendram, Ranjan Provis, Jan M. Michaelides, Michel Carroll, Joseph Invest Ophthalmol Vis Sci Retina PURPOSE: To examine the foveal avascular zone (FAZ) in patients with congenital achromatopsia (ACHM). METHODS: Forty-two patients with genetically confirmed ACHM were imaged either with Optovue's AngioVue system or Zeiss's Plex Elite 9000, and the presence or absence of a FAZ was determined. For images where a FAZ was present and could be confidently segmented, FAZ area, circularity index, and roundness were measured and compared with previously published normative values. Structural optical coherence tomography images were acquired to assess the degree of foveal hypoplasia (number and thickness of inner retinal layers present at the fovea). RESULTS: A FAZ was present in 31 of 42 patients imaged (74%), although no determination could be made for 11 patients due to poor image quality (26%). The mean ± SD FAZ area for the ACHM retina was 0.281 ± 0.112 mm(2), which was not significantly different from the previously published normative values (P = 0.94). However, their FAZs had decreased circularity (P < 0.0001) and decreased roundness (P < 0.0001) compared to the normative cohort. In the patients with ACHM examined here, the FAZ area decreased as the number and thickness of the retained inner retinal layers increased. CONCLUSIONS: Our data demonstrate that despite the presence of foveal hypoplasia, patients with ACHM can have a FAZ. This is distinct from other conditions associated with foveal hypoplasia, which generally show an absence of the FAZ. In ACHM, FAZ formation does not appear to be sufficient for complete pit formation, contrary to some models of foveal development. The Association for Research in Vision and Ophthalmology 2020-08-31 /pmc/articles/PMC7463179/ /pubmed/32866266 http://dx.doi.org/10.1167/iovs.61.10.52 Text en Copyright 2020 The Authors http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License.
spellingShingle Retina
Linderman, Rachel E.
Georgiou, Michalis
Woertz, Erica N.
Cava, Jenna A.
Litts, Katie M.
Tarima, Sergey
Rajendram, Ranjan
Provis, Jan M.
Michaelides, Michel
Carroll, Joseph
Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title_full Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title_fullStr Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title_full_unstemmed Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title_short Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit
title_sort preservation of the foveal avascular zone in achromatopsia despite the absence of a fully formed pit
topic Retina
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7463179/
https://www.ncbi.nlm.nih.gov/pubmed/32866266
http://dx.doi.org/10.1167/iovs.61.10.52
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