Linderman, RE;
Georgiou, M;
Woertz, EN;
Cava, JA;
Litts, KM;
Tarima, S;
Rajendram, R;
... Carroll, J; + view all
(2020)
Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit.
Investigative Ophthalmology & Visual Science
, 61
(10)
, Article 52. 10.1167/iovs.61.10.52.
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Abstract
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 mm2, 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.
| Type: | Article |
|---|---|
| Title: | Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1167/iovs.61.10.52 |
| Publisher version: | https://doi.org/10.1167/iovs.61.10.52 |
| Language: | English |
| Additional information: | This work is licensed under a Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/). |
| Keywords: | foveal avascular zone, achromatopsia, retinal development, optical coherence tomography angiography |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10109630 |
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