Purpose: To analyze fixation location and stability in best vitelliform macular dystrophy (BVMD) and test their association with best-corrected visual acuity (BCVA).Design: Observational, cross-sectional study.Participants: Thirty patients (55 eyes) affected by genetically confirmed BVMD were followed up at the Retinal Heredodystrophies Unit of IRCCS San Raffaele Scientific Institute, Milan.Methods: Patients underwent testing with macular integrity assessment (MAIA) microperimeter. Fixation location was measured as distance in degrees (& DEG;) between preferred retinal locus (PRL) and estimated fovea location (EFL); fixation was defined as eccentric when the distance between PRL and EFL exceeded 2 & DEG;. Fixation stability was graded as stable, relatively unstable, or unstable and expressed as bivariate contour ellipse area (BCEA, & DEG;2).Main Outcome Measures: Fixation location and stability.Results: The median distance of the PRL from the anatomic fovea was 0.7 & DEG;, and fixation location was eccentric in 27% of eyes. Fixation was graded as stable in 64% of eyes, relatively unstable in 13%, and unstable in 24%, with a median 95% BCEA of 6.2 & DEG;2. The atrophic/fibrotic stage was associated with worse fixation pa-rameters (all P < 0.01). Both PRL eccentricity and fixation stability were linearly associated with BCVA: every 1 & DEG; increase in PRL eccentricity was associated with a 0.07 logarithm of the minimum angle of resolution (logMAR) worse BCVA (P < 0.0001) while every 1 & DEG;2 increase in 95% BCEA was associated with a 0.01 logMAR worse BCVA (P < 0.001). No significant intereye correlation was found for PRL eccentricity and fixation stability, as well as no association between the patient's age and fixation parameters.Conclusions: We demonstrated that most eyes affected by BVMD retain a central stable fixation and pro-vided evidence that both fixation eccentricity and stability are strongly associated with visual acuity in BVMD. These parameters may serve as secondary end points for future clinical trials.Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. Ophthalmology Science 2023;3:100329 & COPY; 2023 by the American Academy of Ophthalmology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Fixation Location and Stability in Best Vitelliform Macular Dystrophy / Bianco, Lorenzo; Arrigo, Alessandro; Marchese, Alessandro; Antropoli, Alessio; Aragona, Emanuela; La Franca, Lamberto; Mauro, Luca; Pina, Adelaide; Hassan Farah, Rashid; Basile, Giulia; Bandello, Francesco; Battaglia Parodi, Maurizio. - In: OPHTHALMOLOGY SCIENCE. - ISSN 2666-9145. - 3:4(2023), p. 100329. [10.1016/j.xops.2023.100329]

Fixation Location and Stability in Best Vitelliform Macular Dystrophy

Bianco, Lorenzo
Primo
;
Arrigo, Alessandro
Secondo
;
Marchese, Alessandro;Antropoli, Alessio;Aragona, Emanuela;La Franca, Lamberto;Bandello, Francesco
Penultimo
;
Battaglia Parodi, Maurizio
Ultimo
2023-01-01

Abstract

Purpose: To analyze fixation location and stability in best vitelliform macular dystrophy (BVMD) and test their association with best-corrected visual acuity (BCVA).Design: Observational, cross-sectional study.Participants: Thirty patients (55 eyes) affected by genetically confirmed BVMD were followed up at the Retinal Heredodystrophies Unit of IRCCS San Raffaele Scientific Institute, Milan.Methods: Patients underwent testing with macular integrity assessment (MAIA) microperimeter. Fixation location was measured as distance in degrees (& DEG;) between preferred retinal locus (PRL) and estimated fovea location (EFL); fixation was defined as eccentric when the distance between PRL and EFL exceeded 2 & DEG;. Fixation stability was graded as stable, relatively unstable, or unstable and expressed as bivariate contour ellipse area (BCEA, & DEG;2).Main Outcome Measures: Fixation location and stability.Results: The median distance of the PRL from the anatomic fovea was 0.7 & DEG;, and fixation location was eccentric in 27% of eyes. Fixation was graded as stable in 64% of eyes, relatively unstable in 13%, and unstable in 24%, with a median 95% BCEA of 6.2 & DEG;2. The atrophic/fibrotic stage was associated with worse fixation pa-rameters (all P < 0.01). Both PRL eccentricity and fixation stability were linearly associated with BCVA: every 1 & DEG; increase in PRL eccentricity was associated with a 0.07 logarithm of the minimum angle of resolution (logMAR) worse BCVA (P < 0.0001) while every 1 & DEG;2 increase in 95% BCEA was associated with a 0.01 logMAR worse BCVA (P < 0.001). No significant intereye correlation was found for PRL eccentricity and fixation stability, as well as no association between the patient's age and fixation parameters.Conclusions: We demonstrated that most eyes affected by BVMD retain a central stable fixation and pro-vided evidence that both fixation eccentricity and stability are strongly associated with visual acuity in BVMD. These parameters may serve as secondary end points for future clinical trials.Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. Ophthalmology Science 2023;3:100329 & COPY; 2023 by the American Academy of Ophthalmology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
2023
BEST1
Best vitelliform macular dystrophy
Fixation eccentricity
Fixation stability
Mircoperimetry
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/148716
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