Abstract
Purpose :
Bietti’s crystalline dystrophy (BCD) is a degenerative retinal disease characterized by crystalline deposits in the retina. Choroidal neovascularization (CNV) is rare in cases of BCD, and no treatment method has been established. Herein we report a case of CNV with BCD treated with the anti-vascular endothelial growth factor (VEGF) agent aflibercept.
Methods :
The patient was a 68-year-old woman who visited our hospital due to night blindness. Her best-corrected visual acuity (BCVA) was 1.2 in both eyes, and crystalline deposits were observed in the posterior fundus. There was no glistening limbic crystal in the superficial corneal stroma. Optical coherence tomography revealed choroidal atrophy and thinning of the outer retinal layer (Fig. 1a), and in full-field electroretinography the rod response was absent and there was a marked decrease in the cone response. BCD was diagnosed based on these observations. Genetic analysis via Sanger sequencing revealed known homozygous mutations—c.802-8_810delinsGC in the CYP4V2 gene. Nine months later her left BCVA had decreased to 0.15. CNV with retinal hemorrhage was detected in the macular region. After obtaining informed consent from the patient and approval from the hospital’s ethics committee, intravitreal injections of the anti-VEGF agent aflibercept were administered.
Results :
After three aflibercept injections the CNV disappeared (Fig. 1b). At the most recent follow up examination her left BCVA was 0.2 and no recurrence was evident. No side effects of aflibercept were detected.
Conclusions :
If there is a rapid reduction in visual acuity in a patient with BCD, the occurrence of CNV should be suspected. Intravitreal injections of aflibercept were effective for CNV in the present patient with BCD.
This is a 2020 ARVO Annual Meeting abstract.