Management of Combined Hamartoma of the Retina and Retinal Pigment Epithelium in Children

Abstract

Combined hamartoma of the retina and the retinal pigment epithelium (CHRRPE) is a congenital benign proliferation of the retinal pigment epithelium, neurosensory retina, retinal vasculature, and adjacent vitreous. CHRRPE usually presents as a unilateral slightly elevated and variably pigmented mass with vascular tortuosity and epiretinal membrane (ERM) formation in a pediatric patient. Lesions may be classified based on fundus location, structural retinal components and optical coherence tomography findings using the CHRRPE classification system. This system may be a valuable tool for quantifying severity and guiding management. In general, macular lesions are more likely to cause poor visual acuity and progressive visual loss. Close follow-up is recommended to monitor disease progression including exudation, subretinal neovascularization and ERM progression. Pars plana vitrectomy with membrane peeling may be considered in select cases.