Indocyanine Green Angiography Features of Central Areolar Choroidal Dystrophy

Purpose:Central areolar choroidal dystrophy (CACD) 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 (FA) and confocal indocyanine green angiography (ICGA) features of CACD.

Methods:We performed a complete ophthalmologic examination including best corrected visual acuity (BCVA), fundus examination, color fundus photographs, red free frames, FA, infrared, confocal ICG and electroretinography, in a cohort of patients with family history of CACD.

Results:Eleven patients (22 eyes) affected were prospectively included. ICG 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 pin points were observed on the late phases.

Conclusions:In our small series, ICG angiography distinguished two phenotypes of CACD, correlated with FA features.