Macular Telangiectasia Type 2: Visual Acuity, Disease End Stage, and the MacTel Area
| Autor: | Marcus Fruttiger, Konstantinos Balaskas, Catherine A Egan, Roy Schwartz, Traci E Clemons, Tjebo F. C. Heeren, Emily Y. Chew, Peter Charbel Issa |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Visual acuity genetic structures 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Atrophy Foveal Ophthalmology medicine Stage (cooking) Macular hole 030304 developmental biology Macular telangiectasia 0303 health sciences business.industry Retinal medicine.disease eye diseases medicine.anatomical_structure chemistry 030221 ophthalmology & optometry sense organs medicine.symptom business Optic disc |
| Zdroj: | Ophthalmology. 127:1539-1548 |
| ISSN: | 0161-6420 |
| Popis: | Purpose To report the visual acuity measures from the macular telangiectasia type 2 (MacTel) registry and to investigate and describe phenotypic findings in eyes with substantial vision loss resulting from MacTel. Design Cross-sectional multicenter study. Participants Participants in the MacTel Natural History Observation Registration Study. Methods Best-corrected visual acuity (BCVA) data, retinal imaging data, and clinical data were accessed from the MacTel Study databases in May 2019. Main Outcome Measures Frequency distribution of BCVA and its relationship to age; morphologic changes in eyes with very late disease stages, defined by a BCVA of 20/200 or worse; average retinal thickness of macular subfields on OCT; and dimensions of the area affected by MacTel (i.e., the MacTel area). Results Best-corrected visual acuity was 20/50 or worse in 37.3% and 20/200 or worse in 3.8% of 4449 eyes of 2248 patients; 18.4% and 0.7% of all patients showed bilateral BCVA of 20/50 or worse and 20/200 or worse, respectively. Asymmetry between right and left eyes was present (median BCVA, 71 letters vs. 74 letters), a finding supported by more advanced morphologic changes in right eyes. Participant age correlated with BCVA, but the effect size was small. If a neovascularization or macular hole were present, bilateral occurrence was frequent (33% or 17%, respectively), and BCVA was better than 20/200 (79% or 78%, respectively) or 20/50 or better (26% or 13%, respectively). Eyes with advanced disease (BCVA, ≤20/200) showed the following characteristics: (1) atrophy of the foveal photoreceptor layer with or without associated subretinal fibrosis; (2) an affected area, termed MacTel area, limited to a horizontal diameter not exceeding the distance between the temporal optic disc margin and foveal center, and the vertical diameter not exceeding approximately 0.8 times this distance (exceptions were eyes with large active or inactive neovascular membranes); (3) reduced retinal thickness measures within the MacTel area; and (4) less frequent retinal greying and more frequent hyperpigmentations compared with eyes that have better BCVA. Conclusions Severe vision loss is rare in MacTel and is related to photoreceptor atrophy in most people. Results indicate disease asymmetry with slightly worse vision and more advanced disease manifestation in right eyes. MacTel-related neurodegeneration does not spread beyond the limits of the MacTel area. |
| Databáze: | OpenAIRE |
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