Case of the Month | September 2022

This patient has a large congenital hypertrophy of the retinal pigment epithelium lesion (CHRPE). They are flat lesions that characteristically have variable internal pigmentation surrounded with rim of hyperpigmentation.. They tend to reside in the retinal periphery, though rarely they present in the macula or juxtapapillary area. In childhood, they typically present with hyperpigmentation, and non-pigmented lacunae develop over time. The lesions are quite stationary but can have modest, concentric growth over time. Uncommonly, choroidal neovascular membranes can emanate from CHRPE lesions, and transformation into an adenocarcinoma of the RPE rarely occurs. Histopathologically, there is atrophy of the overlying outer retinal tissues. Clinically, patients have scotomas that correspond to the CHRPE lesions.