Natural course of ocular function in pigmented paravenous retinochoroidal atrophy

PURPOSE: To estimate mean rates of change of ocular function in patients with pigmented paravenous retinochoroidal atrophy (PPRCA). DESIGN: Retrospective observational case series. METHODS: Fifteen patients aged 8 to 67 years with PPRCA were followed for 3 to 35 years (average follow-up time, 13 years) with measures of visual acuity, visual field area, and full,field electroretinogram amplitude. Mean annual exponential rates of change were quantified by repeated measures longitudinal regression. RESULTS: Estimated mean annual rates of change were -2.0% for Visual acuity, +0.3% for visual field area, and -3.4% and -6.7% for 0.5 Hz and 30 Hz electroretinogram amplitudes, respectively. CONCLUSION: Patients with PPRCA have a slowly progressive disease with respect to the loss of peripheral vision.