Multimodal Imaging in Retinitis Pigmentosa: Correlations among Microvascular Changes, Macular Function and Retinal Structure

PURPOSE: To analyze microvascular changes in patients with retinitis pigmentosa (RP) with relatively preserved visual acuity (VA), using swept source optical coherence tomography (SS-OCT) angiography to correlate results to macular function and structure. METHODS: This was a case–control study conducted over 70 eyes of 35 RP patients with relatively preserved VA. All patients underwent a complete ophthalmic examination, including SS-OCT, OCT angiography (OCT-A), fundus autofluorescence (FAF), and multifocal electroretinogram (mfERG). Thirty-four eyes of 34 healthy controls of age-, sex-, and axial length-matched (control group), were also analyzed. The main outcome measures were foveal and parafoveal vascular densities (FVDs and PFVDs) in the superficial capillary plexus (SCP) and deep capillary plexus (DCP), foveal avascular zone (FAZ) and its enlargement coefficient and their correlation with macular function (by means of VA and mfERG), and structure (by means of FAF and SS-OCT). RESULTS: In the RP group, PFVD was 25.99 ± 5.2% in the SCP and 34.47 ± 2.37% in the DCP and were significantly lower as compared to control group (P < 0.0001; P = 0.0026, respectively). Enlargement coefficient of FAZ was 1.78 ± 0.79. We found a statistically significant correlation between VA and PFVD in the DCP (P < 0.0001), FAZ disruption in the SCP (P = 0.006) and enlargement coefficient of FAZ (P = 0.01). The parafoveal DCP density was significantly correlated with P1 amplitude (P = 0.005) in rings 2, 3, 4, and 5 of the mfERG. We found a statistically significant correlation between parafoveal density in the DCP, thickness of ganglion cell complex (GCC) (P = 0.001), and the width of ellipsoid band (P = 0.041). Parafoveal SCP density was also correlated to GCC (P = 0.033). CONCLUSIONS: We showed that vascular alteration in RP begins at the level of the DCP, which affects the outer retina and leads to a narrowing of the ellipsoid. The alteration of the SCP would occur later in the evolution of the disease. Vascular changes occur early during RP and were highly correlated to retinal function and structure. OCT-A seems to be a good tool to quantify vascular network loss and could play a central role in staging, prognosis, and monitoring disease progression.