Abstract
Purpose:
To analyze the retinal structure and autofluorescence (AF) patterns in retinitis pigmentosa (RP).
Methods:
Fifty eyes of 25 patients with RP were included. Mean age of 51.92 years. Best corrected visual acuity (BCVA) ranged from light perception to 20/25. Spectral-domain optical coherence tomography (SD-OCT) and AF imaging of the macular area were obtained using Spectralis HRA+OCT (Heidelberg Engineering). Patients were divided into 4 groups according to their fundus AF pattern. Correlation with structural abnormalities was performed.
Results:
Sixteen eyes showed a hyperautofluorescent ring (group 1), 8 eyes showed preserved retinal AF (group 2), 6 eyes showed abnormal hyperautofluorescence (group 3) and 20 eyes showed increased hypoautofluorescence inside vascular arcades (group 4). In this last group, all patients showed an abnormal foveal contour due to generalized atrophy and different levels of disorganization of retinal layers. These changes correlated with the BCVA obtained for group 4, which was counting fingers or worse for all patients. An epiretinal membrane (ERM) was found in all groups: 75% of patients in group 2 and 66.6% of patients in group 3, while in groups 1 and 4 it was present in less than 50% of the cases. Cystoid macular edema (CME) was seen in half of the patients in groups 1 and 2, in only 2 patients of group 4 and it was absent in patients of group 3. Eleven out of 14 patients with CME had a BCVA of 20/40 or better. The inner and outer segment of the photoreceptors (IS/OS junction) was clearly detected centrally in all patients of group 1. A diffuse hyperreflective granular material was observed in the inner and outer retinal layers among all groups. In patients of group 4, dense and irregular clumping of hyperreflective material was also noted. Group 1 had the best visual acuity with a mean logMAR score of 0.38.
Conclusions:
An increased hypoautofluorescence pattern was associated with retinal atrophy and low visual acuity. Two of the SD-OCT features that were associated with better visual acuity included CME and a well defined IS/OS junction. An ERM and hyperreflective granular material were common findings in this group of RP patients regardless of AF pattern or retinal structure.
Keywords: 696 retinal degenerations: hereditary •
552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) •
702 retinitis