Hereditary retinal dystrophies and choroidal neovascularization

Abstract 

Background: Choroidal neovascularization infrequently occurs in patients affected by hereditary retinal dystrophies. Methods: We studied eight patients suffering from different hereditary retinal dystrophies (Best’s disease, reticular dystrophy, butterfly-shaped dystrophy, gyrate atrophy, and retinitis pigmentosa) who developed choroidal neovascularization. All patients underwent complete ophthalmic evaluation, electrophysiology, colour vision testing, and fluorescein angiography. In some patients, ICG video-angio- graphy was also performed. Laser treatment was carried out in only one patient. Results: The mean duration of follow-up was 41.7 months (range 6–148 months). At CNV diagnosis, the mean VA was 0.23 (range 0.02–0.6). At the last follow-up, mean VA was 0.34 (range HM to 0.9). At the last follow-up, fluorescein angiography showed a focal, atrophic scar in seven eyes, a fibrotic membrane in two eyes and a still active membrane in two cases. Conclusion: We emphasize the relatively favourable visual prognosis in patients suffering from inherited retinal dystrophies complicated with choroidal neovascularization. Therapeutic approaches other than laser treatment could be attempted in these patients.