Optical coherence tomography angiography in best vitelliform macular dystrophy
Ahmad Mirshahi1, Alireza Lashay1, Ahmad Masoumi1
1Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Journal of Current Ophthalmology
|December 18, 2019
Summary
Best vitelliform macular dystrophy (BVMD) shows unusual vascular patterns on optical coherence tomography angiography (OCTA). A bridging vessel in the foveal avascular zone (FAZ) was observed in all patients, potentially linked to bestrophin protein effects.
Area of Science:
- Ophthalmology
- Medical Imaging
- Genetics
Background:
- Best vitelliform macular dystrophy (BVMD) is an inherited retinal disease.
- Characteristic macular lesions and electrophysiologic abnormalities are typical.
- Optical coherence tomography angiography (OCTA) offers detailed vascular imaging.
Purpose of the Study:
- To describe unique vascular flow patterns in BVMD using OCTA.
- To investigate the foveal avascular zone (FAZ) in BVMD patients.
- To correlate OCTA findings with clinical presentation and electrophysiology.
Main Methods:
- Evaluation of four eyes from two BVMD patients.
- Ophthalmic examination including best-corrected visual acuity (BCVA).
- Spectral domain optical coherence tomography (SD-OCT) and OCTA imaging.
- Confirmation of diagnosis via electroretinography (ERG) and electrooculography (EOG).
Main Results:
- All eyes confirmed BVMD electrophysiologically.
- Patients aged 18 and 35 presented with varying stages of macular lesions.
- OCTA revealed an absent distinct FAZ with a bridging vessel in all eyes.
- Choroidal neovascularization (CNV) was observed in one patient's eye.
Conclusions:
- A bridging vessel within the FAZ is a notable OCTA finding in BVMD.
- This vascular anomaly may be influenced by bestrophin protein's effect on RPE cells.
- Further research into bestrophin's role in retinal vascularization is warranted.
