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

Microvasc Res. 2025 Jan:157:104762. doi: 10.1016/j.mvr.2024.104762. Epub 2024 Nov 8.

Abstract

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.

Keywords: Bietti crystalline dystrophy (BCD); Hemodynamic dysfunction; Multimodal imaging; Pupillary light reflex defects; Retinal oxygenation changes.

MeSH terms

  • Adult
  • Aged
  • Blood Flow Velocity
  • Case-Control Studies
  • Corneal Dystrophies, Hereditary* / diagnostic imaging
  • Corneal Dystrophies, Hereditary* / metabolism
  • Corneal Dystrophies, Hereditary* / physiopathology
  • Cross-Sectional Studies
  • Female
  • Humans
  • Male
  • Middle Aged
  • Multimodal Imaging*
  • Oxygen / blood
  • Oxygen Saturation
  • Predictive Value of Tests
  • Reflex, Pupillary
  • Regional Blood Flow*
  • Retinal Diseases / diagnostic imaging
  • Retinal Diseases / physiopathology
  • Retinal Vessels* / diagnostic imaging
  • Retinal Vessels* / metabolism
  • Retinal Vessels* / physiopathology
  • Vascular Remodeling*
  • Young Adult

Substances

  • Oxygen

Supplementary concepts

  • Bietti Crystalline Dystrophy