Natural course of ocular function in pigmented paravenous retinochoroidal atrophy.

Abstract

To estimate mean rates of change of ocular function in patients with pigmented paravenous retinochoroidal atrophy (PPRCA). Retrospective observational case series. 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. 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. Patients with PPRCA have a slowly progressive disease with respect to the loss of peripheral vision.