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Published on: May 26, 2023
Natural course of ocular function in pigmented paravenous retinochoroidal atrophy
John Y Choi1, Michael A Sandberg, Eliot L Berson
1Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
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.
