Indocyanine green angiography abnormality of the periphery in vitelliform macular dystrophy

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).

Observational case report series.

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.

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.

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.