Simple Congenital Hamartoma of Retinal Pigment Epithelium

A 45-year female presented in the Outpatient Department of a tertiary care hospital of Lahore with dimness of vision in the right eye. Her history revealed that the patient had noticed decreased vision since her childhood. Fundus examination revealed a jet-black slightly raised lesion about the size of ¼-disc diameter, located in the centre of macula. Optical coherence tomography (OCT) showed a highly hyper-reflective lesion at the fovea with sharp steep shadowing of the underlying structures due to backscattering. We excluded congenital hypertrophy of the retinal pigment epithelium (CHRPE) from our differential diagnosis, as solitary CHRPE is a flat well-demarcated lesion at the level of retinal pigment epithelium (RPE). Adenoma of RPE was eliminated as it has a large feeding and draining blood vessels with intra- and sub-retinal exudation. Choroid and surrounding retina was normal in our patient, which was helpful in differentiating it from choroidal nevus/choroidal melanoma. Our final diagnosis was simple congenital hamartoma of RPE. Key Words: Hamartoma, Retinal pigment epithelium, Macula.