Dataset Information

Detailed Evaluation of Chromatic Pupillometry and Full-Field Stimulus Testing to Assess Ultralow Vision in Retinitis Pigmentosa.

ABSTRACT:

Purpose

Novel therapeutic options, such as regenerative medicine and gene therapy, are now emerging as viable treatment options for patients with severe visual impairments, such as retinitis pigmentosa (RP). Gradable assessment of patients' visual function is essential to consider treatment options and to evaluate treatment outcomes; however, evaluation of visual function in patients with advanced low vision is often challenging because of patients' poor and sometimes unpredictable responses. In this study, we attempted to accurately assess visual capabilities and disease stage in patients with RP with a visual acuity (VA) of ≤ 0.01.

Design

Retrospective analysis of visual function indicators, including VA, retinal thickness, full-field stimulus testing (FST), and chromatic pupillometry.

Subjects

Overall, 43 patients (84 eyes) with advanced RP with a VA of ≤ 0.01 visited Kobe City Eye Hospital from 2019 to 2021.

Methods

Hierarchical (multilevel) Bayesian modeling was used to estimate individual eye's pupil response and FST threshold, taking into account the ambiguity and randomness often observed in patients with ultralow vision. Using the estimated ability obtained from each test, the correlation between each test and retinal thickness was further analyzed to make a comprehensive assessment of the data.

Main outcome measures

Visual acuity, retinal thickness, FST threshold, and pupil diameter change to different light stimuli.

Results

Full-field stimulus testing and pupillometry measurements were moderately correlated with VA but exhibited a wide range of values within the same VA groups. Full-field stimulus testing was not correlated with central retinal thickness at counting fingers/hand motion VA range and seemed to reflect overall remaining photoreceptor function, including peripheral retina. Pupillometry may be able to distinguish between different levels of inner retinal function.

Conclusions

The combination of pupillometry and FST allowed for graded evaluation of visual function within patients grouped in the same VA groups in patients with advanced RP with ultralow vision.

Financial disclosures

Proprietary or commercial disclosure may be found after the references.

SUBMITTER: Yamamoto M

PROVIDER: S-EPMC10618823 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature

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Publications

Detailed Evaluation of Chromatic Pupillometry and Full-Field Stimulus Testing to Assess Ultralow Vision in Retinitis Pigmentosa.

Yamamoto Midori M Matsuyama Take T Maeda Tadao T Takagi Seiji S Motozawa Naohiro N Sakai Daiki D Hirami Yasuhiko Y Maeda Akiko A Kurimoto Yasuo Y Takahashi Masayo M Mandai Michiko M

Ophthalmology science 20230508 4

<h4>Purpose</h4>Novel therapeutic options, such as regenerative medicine and gene therapy, are now emerging as viable treatment options for patients with severe visual impairments, such as retinitis pigmentosa (RP). Gradable assessment of patients' visual function is essential to consider treatment options and to evaluate treatment outcomes; however, evaluation of visual function in patients with advanced low vision is often challenging because of patients' poor and sometimes unpredictable respo ...[more]

PMID: 37920419

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Project description:PurposeThere have been few systematic reports of vision-related activity limitations of people with retinitis pigmentosa (RP). We report a merging of data from the National Eye Institute Visual Function Questionnaire (NEI-VFQ) obtained in five previous studies. We asked whether the Vision Function Scale (VFS; Pesudovs et al., 2010) which was developed for cataract patients would apply in this new population (condition).MethodsFive hundred ninety-four individuals completed a total of 1753 questionnaires, with 209 participants providing responses over at least 4 years. Rasch analysis showed that the 15-item VFS was poorly targeted. A new instrument created by adding four driving-related items to the VFS had better targeting. As an indirect validation, VFS-plus person scores were compared to visual field area measured using a Goldmann perimeter, to the summed score for the combined 30-2 and 30/60-1 Humphrey Field Analyzer programs (HFA), to 30-Hz full-field cone electroretinogram (ERG) amplitude, and to ETDRS visual acuity. Changes in VFS-plus person scores with age and between four common heredity groups were also examined.ResultsThe Rasch model of responses to the 19 VFS-plus items had person and item separation of 2.66 and 24.43 respectively. The VFS-plus person scores were related to each vision measure (p < 0.001). Over a five-year period, there was a reduction in person scores of 0.5 logits (p < 0.001). Person scores fell by an average of 0.34 logits per decade (p < 0.0001). Participants with an X-linked hereditary pattern had, on average, lower person scores (p < 0.001).ConclusionsThe VFS-plus instrument quantified a highly-significant annual reduction in perceived vision-related ability over a five-year period. The outcome was consistent with clinical measures of vision, and detected lower perceived vision-related ability in participants with X-linked disease. It may be of use in future studies, but this needs to be tested in a representative population sample.

Dataset Information

Detailed Evaluation of Chromatic Pupillometry and Full-Field Stimulus Testing to Assess Ultralow Vision in Retinitis Pigmentosa.

Purpose

Design

Subjects

Methods

Main outcome measures

Results

Conclusions

Financial disclosures

Publications

Detailed Evaluation of Chromatic Pupillometry and Full-Field Stimulus Testing to Assess Ultralow Vision in Retinitis Pigmentosa.

Similar Datasets

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