Indocyanine green angiography in Fundus flavimaculatus

To describe the characteristic findings of fundus flavimaculatus (Stargardt disease) as seen on indocyanine green angiography.

Twelve eyes of 6 consecutive patients with fundus flavimaculatus were studied by fundus color photographs, fluorescein angiography and indocyanine green angiography.

Indocyanine green angiography allowed visualization of small, clearly demarcated areas in which hypofluorescence increased over time, leading eventually to a large reticular pattern with small areas of normal-appearing choroid encircled by a well-defined network of hypofluorescent curvilinear lesions. These hypofluorescent flecks were present in all 12 eyes but corresponded only partially to the yellow flecks visible on biomicroscopy of the fundus. The peripapillary area was well preserved on indocyanine green angiography and the periphery did not show any visible abnormalities.

The hypofluorescent curvilinear areas visualized on indocyanine green angiography form a reticular pattern that is similar to the polygonal shape of the watershed zones between terminal choroidal arterioles, which supply the choriocapillaris. These dark areas may reflect choriocapillaris defects secondary to lysis of lipofuscin-engorged retinal pigment epithelial cells. The typical lesions of fundus flavimaculatus thus seem to be situated in areas of least vascular supply. Their absence in the peripapillary area, which benefits from anastomotic vascular connections, would support this hypothesis.