Combined Hamartoma of Retina and Retinal Pigment Epithelium: A Case Report

Abstract

Background: Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is an uncommon benign tumor of the retina that often affects one eye. The typical CHRRPE consists of lesions that are somewhat elevated at the posterior pole, and the proliferative membrane frequently causes vascular distortion. Macular edema, macular holes, retinal detachments, or vitreous hemorrhages may manifest in severe cases. Patients with unusual clinical symptoms are more likely to have an incorrect diagnosis made by novice ophthalmologists.
Case summary: We present a bilateral involving CHRRPE lesion in a 26-year-old gentleman who came with complaints of defective vision in both eyes since childhood. His BCVA was Right eye 5/60 and left eye 6/60. His anterior segment examination showed no abnormality, with posterior segment examination showing both eyes greyish white elevated lesions involving the macula with thick fibrotic membrane causing the macular drag temporally in the right eye and supero-temporally in the left eye. OCT shows the thick ERM with the disorganized inner retinal layers suggestive of hamartoma of the Retina and Retinal Pigment Epithelium.
Conclusion: Although specialist retina evaluation is enough to diagnose a case of CHHRPE, associated OCT evaluation will help in differentiating the lesion from other differential diagnosis such as idiopathic Epiretinal membranes and unilateral involvement is more common but bilateral involvement can be seen as in this case.