Linking the Presence of Macular Oedema to Structural and Functional Alterations in Retinitis Pigmentosa.

OBJECTIVE: To investigate the association between the central retinal thickness (CRT), the retinal nerve fibre layer thickness (RNFL), and the functional alterations in retinitis pigmentosa (RP) patients. METHODS: Forty-three patients with typical RP and nineteen age-matched controls, who underwent SD-OCT (macular and optic disc OCT protocols) and electrophysiology, were included. The RP group was divided into two subgroups: with clinical appearance of macular oedema (ME-RP; 30 eyes) and without macular oedema (no-ME; 44 eyes). Central retinal thickness OCT data were averaged in three zones (zone 1 [0°-3°], zone 2 [3°-8°], and zone 3 [8°-15°]) and were evaluated in relation to the RNFL thickness and electrophysiological data. RESULTS: The ME-RP group showed increased CRT (zone 1) and RNFL thickness compared to the controls and no-ME-RP (p ≤ 0.002). The no-ME-RP group had reduced CRT thickness (all zones; p ≤ 0.018) compared to the controls and ME-RP, whereas the RNFL thickness in the no-ME-RP group was reduced only compared to the ME-RP group (p < 0.001). The ME-RP group showed significantly more attenuated functional responses than the no-ME-RP patients. A significant positive interaction was found between the CRT (zones 1 and 2) and the RNFL thickness within ME-RP (p ≤ 0.010). Significant negative interactions were found between CRT, RNFL thickness, and functional findings within ME-RP (p ≤ 0.049). CONCLUSION: The presence of macular oedema correlated well with increased RNFL thickness and residual function in RP patients. Such association provides evidence of an underlying transneuronal mechanism of retinal degeneration. Simultaneous monitoring of CRT and RNFL thickness may help in the future to evaluate the progression of the disease and the efficacy of treatments in RP patients.

Retinal Oxygenation in Inherited Diseases of the Retina.

(1) Background: The aim of our study was to investigate the relationship between retinal metabolic alterations (retinal vessel oximetry, RO) and structural findings (retinal vessel diameter, central retinal thickness and retinal nerve fiber layer thickness, RNFL) in patients with inherited retinal diseases (IRDs). (2) Methods: A total of 181 eyes of 92 subjects were examined: 121 eyes of 62 patients with IRDs were compared to 60 eyes of 30 healthy age-matched controls. The retinal vessel oximetry was performed with the oxygen saturation measurement tool of the Retinal Vessel Analyser (RVA; IMEDOS Systems UG, Jena, Germany). The oxygen saturation in all four major peripapillary retinal arterioles (A-SO2; %) and venules (V-SO2; %) were measured and their difference (A-V SO2; %) was calculated. Additionally, retinal vessel diameters of the corresponding arterioles (D-A; µm) and venules (D-V; µm) were determined. The peripapillary central retinal thickness and the RNFL thickness were measured using spectral domain optical coherence tomography (SD-OCT) (Carl Zeiss Meditec, Dublin, CA, USA). Moreover, we calculated the mean central retinal oxygen exposure (cO2-E; %/µm) and the mean peripapillary oxygen exposure (pO2-E; %/µm) per micron of central retinal thickness and nerve fiber layer thickness by dividing the mean central retinal thickness (CRT) and the RNFL thickness with the mean A-V SO2. (3) Results: Rod-cone dystrophy patients had the highest V-SO2 and A-SO2, the lowest A-V SO2, the narrowest D-A and D-V and the thickest RNFL, when compared not only to controls (p ≤ 0.040), but also to patients with other IRDs. Furthermore, in rod-cone dystrophies the cO2-E and the pO2-E were higher in comparison to controls and to patients with other IRDs (p ≤ 0.005). Cone-rod dystrophy patients had the lowest cO2-E compared to controls and patients with other IRDs (p ≤ 0.035). Evaluated in central zones, the cO2-E was significantly different when comparing cone-rod dystrophy (CRD) against rod-cone dystrophy (RCD) patients in all zones (p < 0.001), whereas compared with controls and patients with inherited macular dystrophy this was observed only in zones 1 and 2 (p ≤ 0.018). The oxygen exposure was also the highest in the RCD group for both the nasal and the temporal peripapillary area, among all the evaluated groups (p ≤ 0.025). (4) Conclusions: The presented metabolic-structural approach enhances our understanding of inherited photoreceptor degenerations. Clearly demonstrated through the O2-E comparisons, the central and the peripapillary retina in rod-cone dystrophy eyes consume less oxygen than the control-eyes and eyes with other IRDs. Rod-cone dystrophy eyes seem to be proportionally more exposed to oxygen, the later presumably leading to more pronounced oxidative damage-related remodeling.

Peripapillary retinal vessel diameter correlates with mfERG alterations in retinitis pigmentosa.

PURPOSE: To investigate relationship between the peripapillary retinal vessel diameter and the residual retinal function, measured by mfERG, in patients with retinitis pigmentosa (RP). PATIENTS AND METHODS: A cross-sectional study based on 23 patients with RP (43 eyes) and 20 controls (40 eyes) was performed. Retinal vessel diameters were measured using a computer-based program of the retinal vessel analyser (RVA; IMEDOS Systems UG, Jena, Germany). We evaluated the mean diameter in all four major retinal arterioles (D-A) and venules (D-V) within 1.0-1.5 optic disc diameters from the disc margin. The data were compared with the N1 amplitudes (measured from the baseline to the trough of the first negative wave), with the N1P1 amplitudes (measured from the trough of the first negative wave to the peak of the first positive wave) of the mfERG overall response and with the mfERG responses averaged in zones [zone 1 (0°-3°), zone 2 (3°-8°), zone 3 (8°-15°) and zone 4 (15°-24°)]. RESULTS: Mean (±SD) D-A and D-V were narrower in patients with RP [84.86 µm (±13.37 µm) and 103.35 µm (±13.65 µm), respectively] when compared to controls [92.81 µm (±11.49 µm) and 117.67 µm (±11.93 µm), respectively; the p-values between groups were p = 0.003 for D-A and p < 0.001 for D-V, linear mixed-effects model]. The RP group revealed clear differences compared to the controls: D-A and D-V became narrower with reduced mfERG responses. D-V correlated significantly with the overall mfERG N1 amplitudes (p = 0.013) and with N1P1 amplitudes (p = 0.016). D-V correlated with the mfERG amplitudes averaged in zones: (zone 2, 3 and 4; p ≤ 0.040) and N1P1 mfERG amplitudes (zones 1, 2, 3 and 4; p ≤ 0.013). CONCLUSIONS: Peripapillary retinal vessel diameter is reduced in RP proportionally to functional alterations.