Indocyanine green angiography features of central areolar choroidal dystrophy

Central areolar choroidal dystrophy is an inherited autosomal dominant macular disease characterized by a central atrophy of the retinal pigment epithelium and choriocapillaris. Our purpose was to describe fluorescein angiography and confocal indocyanine green angiography features of central areolar choroidal dystrophy.

We performed a complete ophthalmologic examination including best corrected visual acuity, fundus examination, color fundus photographs, red free frames, fluorescein angiography, infrared, confocal indocyanine green, and electroretinography in a cohort of patients with a family history of central areolar choroidal dystrophy.

Eleven patients (22 eyes) affected were prospectively included. Indocyanine green differentiated two distinct phenotypes. In 9/11 patients, atrophy area was hyperfluorescent or normofluorescent. In the two other patients, the lesion was hypofluorescent from early to late phases and pinpoints were observed on the late phases.

In our small series, indocyanine green angiography distinguished two phenotypes of central areolar choroidal dystrophy, correlated with fluorescein angiography features.