Journal article
Using Goldmann visual field volume to track disease progression in choroideremia
Ophthalmology science (Online), Vol.3(4), 100397
12/2023
DOI: 10.1016/j.xops.2023.100397
PMCID: PMC10630671
PMID: 38025158
Abstract
Choroideremia is an X-linked choroidopathy caused by pathogenic variants in the CHM gene. It is characterized by early appearance of multiple scotomas in the peripheral visual field that spread and coalesce, usually sparing central vision until late in disease. These features make quantitative monitoring of visual decline particularly challenging. Here we describe a novel computational approach to convert Goldmann visual field (GVF) data into quantitative volumetric measurements. With this approach we analyzed visual field loss in a longitudinal, retrospective cohort of patients with choroideremia.
Single-center, retrospective, cohort study.
We analyzed data from 238 clinic visits of 56 molecularly-confirmed male patients with choroideremia from 41 families (range 1–27 visits per patient). Patients had a median follow up of 4 years (range 0–56 years) with an age range of 5 to 76 years at the time of their visits.
Clinical data from molecularly-confirmed patients with choroideremia, including GVF data, were included for analysis. GVF records were traced using a tablet-based application, and the three-dimensional hill-of-vision was interpolated for each trace. This procedure allowed quantification of visual field loss from data collected over decades with differing protocols, including different or incomplete isopters. Visual acuity data were collected and converted to LogMAR values. A delayed exponential mixed effects model was used to evaluate the loss of visual field volume over time.
Visual acuity and Goldmann visual field volume.
The estimated mean age at disease onset was 12.6 years (SD 9.1 years; 95% quantile interval 6.5-36.4 years). Mean field volume loss was 6.8% per year (SD 4.5%; 95% quantile interval 1.9%-18.8%) based on exponential modeling. Field volume was more strongly correlated between eyes (r2 = 0.935) than best-corrected visual acuity (r2 = 0.285).
Volumetric analysis of GVF data enabled quantification of peripheral visual function in patients with choroideremia and evaluation of disease progression. The methods presented here may facilitate the analysis of historical GVF data from patients with inherited retinal disease and other diseases associated with visual field loss. This work informs the creation of appropriate outcome measures in choroideremia therapeutic trials, particularly in trial designs.
Details
- Title: Subtitle
- Using Goldmann visual field volume to track disease progression in choroideremia
- Creators
- Adam P. DeLuca - University of IowaS. Scott Whitmore - University of IowaNicole J. Tatro - University of IowaJeaneen L. Andorf - University of IowaBen P. Faga - University of IowaLaurel A. Faga - University of IowaMalia M. Colins - University of IowaMeagan A. Luse - University of IowaBeau J. Fenner - Singapore National Eye CenterEdwin M. Stone - University of IowaTodd E. Scheetz - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Ophthalmology science (Online), Vol.3(4), 100397
- DOI
- 10.1016/j.xops.2023.100397
- PMID
- 38025158
- PMCID
- PMC10630671
- NLM abbreviation
- Ophthalmol Sci
- ISSN
- 2666-9145
- eISSN
- 2666-9145
- Publisher
- Elsevier Inc
- Grant note
- DOI: 10.13039/100000053, name: National Eye Institute, award: P30EY025580; DOI: 10.13039/100000002, name: National Institutes of Health
- Language
- English
- Electronic publication date
- 09/2023
- Date published
- 12/2023
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; The University of Iowa Institute for Vision Research; Iowa Neuroscience Institute; John and Marcia Carver Nonprofit Genetic Testing Laboratory; Center for Bioinformatics and Computational Biology; Ophthalmology and Visual Sciences
- Record Identifier
- 9984466697302771
Metrics
12 Record Views