ArticleInvestigative ophthalmology & visual science2020
Preservation of the Foveal Avascular Zone in Achromatopsia Despite the Absence of a Fully Formed Pit.
Article in Investigative ophthalmology & visual science, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 13 citations in OpenAlex.
- Axial Length Estimation Using Metadata From Swept-Source Optical Coherence Tomography Angiography.Investigative ophthalmology & visual science · 2026Observational
- Skin Melanin Content and the Foveal Avascular Zone Correlation in a Healthy White Population.Investigative ophthalmology & visual science · 2026Article
- Assessing the Relationship between Foveal Cone Density, Outer Nuclear Layer Thickness and Foveal Morphology.Ophthalmology science · 2026Article
- Multimodal phenotyping of foveal hypoplasia in albinism and albino-like conditions: a pediatric case series with adaptive optics insights.Scientific reports · 2024Article
- In Vivo Assessment of Retinal Phenotypes in Axenfeld-Rieger Syndrome.Investigative ophthalmology & visual science · 2024Article
- Cone Density Is Correlated to Outer Segment Length and Retinal Thickness in the Human Foveola.Investigative ophthalmology & visual science · 2023Article
- Genotypic and Phenotypic Spectrum of Foveal Hypoplasia: A Multicenter Study.Ophthalmology · 2022Observational
- Human Foveal Cone and RPE Cell Topographies and Their Correspondence With Foveal Shape.Investigative ophthalmology & visual science · 2022Article
- Assessing Foveal Structure in Individuals withOphthalmology science · 2021Article
- Examining Whether AOSLO-Based Foveal Cone Metrics in Achromatopsia and Albinism Are Representative of Foveal Cone Structure.Translational vision science & technology · 2021Article
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Authors and funding
10 authors at 3 institutions in 3 countries.
Funding
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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.