A multimodal imaging approach to investigate retinal oxygen and vascular dynamics, and neural dysfunction in bietti crystalline dystrophy.

BACKGROUND

This study aimed to explore retinal changes in Bietti crystalline dystrophy (BCD) patients, including retinal metabolism, blood flow, vascular remodeling, and pupillary light reflex (PLR) abnormalities.

METHODS

This cross-sectional study included 120 eyes from BCD patients and 120 eyes from healthy controls, utilizing a multimodal imaging system (MEFIAS 3200, SYSEYE, Chongqing, China) to evaluate retinal oxygenation, blood flow, vascular structure, and PLR. Measurements included oxygen saturation, blood flow velocity, vessel diameters, and pulsatility metrics. PLR parameters were assessed under specific light stimuli.

RESULTS

BCD patients demonstrated significantly higher retinal oxygen saturation and content, but lower oxygen utilization and metabolism compared to controls, with more pronounced declines in those over 40 years old. Vascular parameters revealed smaller external diameters and larger lumen diameters, indicating vascular remodeling. Retinal blood flow was lower, while the resistivity index was higher in BCD patients. Additionally, PLR abnormalities were noted, including reduced constriction amplitude, pupil constriction ratio, constriction duration, and maximum constriction velocity, along with prolonged latency were observed in BCD patients.

CONCLUSION

BCD patients had significant retinal and vascular changes, along with PLR impairments, especially in patients over 40. More targeted interventions should be focused in future research.