Journal
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
Volume 61, Issue 10, Pages -Publisher
ASSOC RESEARCH VISION OPHTHALMOLOGY INC
DOI: 10.1167/iovs.61.10.52
Keywords
foveal avascular zone; achromatopsia; retinal development; optical coherence tomography angiography
Categories
Funding
- National Eye Institute of the National Institutes of Health (NIH) [R01EY024969, R01EY017607, F32EY029148, T35AG029793, T32GM080202, TL1TR001437, UL1TR001436]
- National Institute of Aging of the National Institutes of Health (NIH) [R01EY024969, R01EY017607, F32EY029148, T35AG029793, T32GM080202, TL1TR001437, UL1TR001436]
- National Institute of General Medical Sciences of the National Institutes of Health (NIH) [R01EY024969, R01EY017607, F32EY029148, T35AG029793, T32GM080202, TL1TR001437, UL1TR001436]
- National Center for Advancing Translational Sciences of the National Institutes of Health (NIH) [R01EY024969, R01EY017607, F32EY029148, T35AG029793, T32GM080202, TL1TR001437, UL1TR001436]
- National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust
- National Institute for Health Research Biomedical Research Centre at UCL Institute of Ophthalmology
- Onassis Foundation
- Leventis Foundation
- Moorfields Eye Hospital Special Trustees
- Moorfields Eye Charity
- Retina UK
- Foundation Fighting Blindness (USA)
- National Center for Research Resources, NIH [C06RR016511]
- Thomas M. Aaberg, Sr. Retina Research Fund
- AchromaCorp.
- NATIONAL EYE INSTITUTE [F32EY029148] Funding Source: NIH RePORTER
Ask authors/readers for more resources
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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available