Retinal Vascular Abnormalities in Different Types of Inherited Retinal Dystrophies Assessed by Optical Coherence Tomography Angiography.

Purpose: To investigate the retinal vascular characteristics among patients with different types of inherited retinal dystrophies (IRDs).

Methods: This comparative cross-sectional study was conducted on 59 genetically confirmed cases of IRD including 37 patients with retinitis pigmentosa (RP) (74 eyes), 13 patients with Stargardt disease (STGD) (26 eyes), and 9 patients with cone-rod dystrophy (CRD) (18 eyes). Both eyes of 50 age- and sex-matched healthy individuals were investigated as controls. All participants underwent optical coherence tomography angiography to investigate the vascular densities (VDs) of superficial and deep capillary plexus (SCP and DCP) as well as foveal avascular zone area.

Results: In RP, significantly lower VD in whole image ( = 0.001 for DCP), fovea ( = 0.038 for SCP), parafovea ( < 0.001 for SCP and DCP), and perifovea ( < 0.001 for SCP and DCP) was observed compared to controls. In STGD, VD of parafovea ( = 0.012 for SCP and = 0.001 for DCP) and fovea ( = 0.016 for DCP) was significantly lower than controls. In CRD, the VD of parafovea ( = 0.025 for DCP) was significantly lower than controls. Whole image density was significantly lower in RP compared to STGD ( < 0.001 for SCP) and CRD ( = 0.037 for SCP). VD in parafovea ( = 0.005 for SCP) and perifovea ( < 0.001 for SCP and DCP) regions was significantly lower in RP compared with STGD. Also, foveal VD in STGD was significantly lower than RP ( = 0.023 for DCP).

Conclusion: Our study demonstrated lower VDs in three different IRDs including RP, STGD, and CRD compared to healthy controls. Changes were more dominant in RP patients.