Using Goldmann visual field volume to track disease progression in choroideremia.

PurposeChoroideremia is an X-linked choroidopathy caused by pathogenic variants in the CHM gene. It characterized early appearance of multiple scotomas peripheral visual field that spread and coalesce, usually sparing central vision until late disease. These features make quantitative monitoring decline particularly challenging. Here we describe a novel computational approach to convert Goldmann (GVF) data into volumetric measurements. With this analyzed loss longitudinal, retrospective cohort patients with choroideremia.DesignSingle-center, retrospective, study.ParticipantsWe from 238 clinic visits 56 molecularly-confirmed male choroideremia 41 families (range 1–27 per patient). Patients had median follow up 4 years 0–56 years) age range 5 76 at time their visits.MethodsClinical choroideremia, including GVF data, were included for analysis. records traced using tablet-based application, three-dimensional hill-of-vision was interpolated each trace. This procedure allowed quantification collected over decades differing protocols, different or incomplete isopters. Visual acuity converted LogMAR values. A delayed exponential mixed effects model used evaluate volume time.Main Outcome MeasuresVisual volume.ResultsThe estimated mean disease onset 12.6 (SD 9.1 years; 95% quantile interval 6.5-36.4 years). Mean 6.8% year 4.5%; 1.9%-18.8%) based on modeling. Field more strongly correlated between eyes (r2 = 0.935) than best-corrected 0.285).ConclusionsVolumetric analysis enabled function evaluation progression. The methods presented here may facilitate historical inherited retinal other diseases associated loss. work informs creation appropriate outcome measures therapeutic trials, trial designs.