Benign foveal retinal pigment epithelium hypopigmentation without functional loss : pediatric case series

We report a case series of pediatric patients with incidentally discovered atypical maculopathy, namely, benign foveal depigmentation of the retinal pigment epithelium (BFD). Longitudinal data, both functional and morphological, were analyzed.

Functional tests (perimetry, full-field, multifocal and pattern ERG) were performed using the Mon PackOne® unit (Métrovision, Pérenchies, France), and multimodal retinal imaging was carried out using the Heidelberg Retinal Tomograph 2 and Heidelberg Spectralis SD-OCT (Heidelberg Inc., Germany).

Four patients were examined, two of whom were followed longitudinally. Pattern ERG, multifocal ERG, and central static perimetry were all within normal limits. The reflective structure of the retinal layers in the foveolar zone appeared normal on SD-OCT, except for a cone of choroidal hyperreflectivity corresponding to the lesion site. The lesion was more evident on near-infrared autofluorescence imaging, presenting as a well-defined, round, hypoautofluorescent foveal area. No progression was observed in the two patients for whom follow-up data were available.

BFD is a rare and sporadic condition that may affect both adults and children. It has no functional visual impact. However, long-term follow-up is advisable to confirm the benign and non-progressive nature of these depigmented macular lesions.