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.