Purpose: To report the peripheral abnormalities seen only with indocyanine green angiography in patients with vitelliform macular dystrophy (Best disease, caused by a mutation in the bestrophin gene).
Design: Observational case report series.
Methods: Eight eyes of four patients, two with only a central macular lesion and two with multifocal lesions, were studied. Results of indocyanine green angiography were compared with findings from ophthalmoscopy and fluorescein angiography.
Results: Throughout the fundus periphery, indocyanine green angiography demonstrated a number of hyperfluorescent spots in all eight eyes. The spots were observed in the midperiphery and the periphery in areas with no abnormality visible by ophthalmoscopy or fluorescein angiography.
Conclusions: Although Best disease generally causes lesions visible in the posterior pole, the extensive distribution of the hyperfluorescent spots is consistent with the wide-ranging abnormalities of the retinal pigment epithelium, Bruch membrane, and the choroid as seen histopathologically.