Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity - 21/10/20

Doi : 10.1016/j.ajo.2020.05.024

Jacque L. Duncan ^a, Wendi Liang ^b, Maureen G. Maguire ^c, Isabelle Audo ^d,^e, Allison R. Ayala ^b,^⁎ , David G. Birch ^f, Joseph Carroll ^g, Janet K. Cheetham ^h, Simona Degli Esposti ⁱ, Todd A. Durham ^h, Laura Erker ^j, Sina Farsiu ^k, Frederick L. Ferris ^l, Elise Heon ^m, Robert B. Hufnagel ⁿ, Alessandro Iannaccone ^k, Glenn J. Jaffe ^k, Christine N. Kay ^o, Michel Michaelides ⁱ, Mark E. Pennesi ^j, José-Alain Sahel ^d,^e,^p
on behalf of the
Foundation Fighting Blindness Consortium Investigator Group¹
The comprehensive list of FFB Consortium Investigator Group members participating in this protocol is included in the Acknowledgement section.
^a University of California, San Francisco, San Francisco, CA, USA
^b Jaeb Center for Health Research, Tampa, FL, USA
^c University of Pennsylvania, Philadelphia, PA, USA
^d Institut de la Vision, Sorbonne Université, INSERM, CNRS, Paris, France
^e Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, INSERM-DGOS CIC1423, Paris, France
^f Retina Foundation of the Southwest, Dallas, TX, USA
^g Medical College of Wisconsin Eye Institute, Milwaukee, WI, USA
^h Foundation Fighting Blindness, Columbia, MD, USA
ⁱ Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
^j Casey Eye Institute–Oregon Health & Science University, Portland, OR, USA
^k Department of Ophthalmology, Duke University, Durham, NC, USA
^l Ophthalmic Research Consultants, Waxhaw, NC, USA
^m Departments of Ophthalmology and Vision Sciences, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada
ⁿ National Eye Institute, Bethesda, MD, USA
^o Vitreoretinal Associates, Gainesville, FL, USA
^p Department of Ophthalmology, The University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

^∗Inquiries to Allison R. Ayala, 15310 Amberly Dr, Ste 350, Tampa, FL 33647, USA15310 Amberly Dr, Ste 350TampaFL33647USA

Abstract

Purpose

To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.

Design

Cross-sectional study within a natural history study.

Methods

Setting: multicenter, international. Study population: Usher syndrome type 2 (USH2) (n = 80) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP) (n = 47) associated with biallelic disease-causing sequence variants in USH2A. Observation procedures: Repeatability of full-field static perimetry (SP) and between-eye symmetry of kinetic perimetry (KP) were evaluated with intraclass correlation coefficients (ICCs). The association of demographic and clinical characteristics with total hill of vision (V_TOT) was assessed with general linear models. Associations between V_TOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests. Main outcome measures: V_TOT (SP) and III4e isopter area (KP).

Results

USH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean V_TOT measures among 3 repeat tests were 32.7 ± 24.1, 31.2 ± 23.4, and 31.7 ± 23.9 decibel-steradians (intraclass correlation coefficient [ICC] = 0.96). Better VA, greater photopic ERG 30-Hz flicker amplitudes, higher mean microperimetry sensitivity, higher central subfield thickness, absence of macular cysts, and higher III4e seeing area were associated with higher V_TOT (all r > .48; P < .05). Mean III4e isopter areas for left (4561 ± 4426 squared degrees) and right eyes (4215 ± 4300 squared degrees) were concordant (ICC = 0.94).

Conclusions

USH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. V_TOT was repeatable and correlated with other functional and structural metrics, suggesting it may be a good summary measure of disease severity in patients with USH2A-related retinal degeneration.

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Highlights

•	Disease duration and age are associated with visual field loss in USH2A-related retinal degeneration.
•	Visual field loss is more severe in Usher syndrome type 2 (USH2) than autosomal recessive nonsyndromic retinitis pigmentosa (ARRP).
•	Total hill of vision is repeatable.
•	Total hill of vision correlates with function and structure.

Le texte complet de cet article est disponible en PDF.

Plan

Functional and Structural Measures at Baseline

Variability of Static Perimetry Testing

Association of Baseline Characteristics With Total Hill of Vision (V_TOT)

Association of V_TOT With Other Measures of Function and Structure

Discussion

Supplemental Material available at AJO.com.

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Vol 219

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Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity - 21/10/20

Abstract

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Design

Methods

Results

Conclusions

Highlights

Plan

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