Vessel density and choroidal vascularity index in patients with Bietti crystalline dystrophy and retinitis pigmentosa.

OBJECTIVE

To evaluate and compare the vessel density (VD) using swept-source optical coherence tomography angiography (OCT-A) and the choroidal vascularity index (CVI) using spectral-domain optical coherence tomography (SD-OCT) in patients with Bietti crystalline dystrophy (BCD) and retinitis pigmentosa (RP).

MATERIALS AND METHODS

A cross-sectional retrospective study was conducted on 26 eyes of 13 BCD patients, 26 eyes of 13 RP patients, and 26 eyes of 13 age- and gender-matched healthy individuals. BCD patients were further staged as having early, intermediate, and advanced disease. VD was assessed in five quadrants of the macula (superior, temporal, inferior, nasal, and center) using a modified ETDRS technique with OCT-A. SD-OCT scans were binarized using Niblack's autolocal threshold, and CVI was determined as the ratio of the luminal area to the total choroidal area.

RESULTS

A significant difference was found in VD in all quadrants of the superficial capillary plexus (SCP) and the deep capillary plexus (DCP) slabs among the three groups (p < 0.001). A statistically significant difference was noted in the mean VD of temporal and inferior quadrants of the SCP and between the BCD and RP groups (p = 0.005, p = 0.015, respectively). A statistically significant difference was observed in the mean VD of the temporal, inferior, and nasal quadrants between the BCD and RP groups on DCP slabs (p = 0.002, p = 0.003, p = 0.003, respectively). The mean central choroidal thickness was 214.65±87.10 μm in the BCD group, 351.69±67.94 μm in the RP group, and 320.92±59.26 μm in the control group (p < 0.001). We found that CVI was significantly higher in the control group than BCD group (p < 0.001), and it was significantly lower in the BCD group when compared to the RP group (p < 0.001).There was no difference in CVI between RP and control groups (p = 0.948). Furthermore, the CVI was significantly lower in the intermediate and advanced disease stages than the early disease stage in the subgroup analysis of BCD patients (p < 0.001, p < 0.001, respectively).

CONCLUSION

CVI is a novel investigative tool to monitor disease progression. The CVI value was lower in BCD and RP patients than in the healthy subjects, and lower CVI values seem to be related to the disease severity in BCD patients. VD was also significantly lower in BCD patients when compared to RP patients, and VD analysis may help clinicians better understand the disease pathophysiology.