Presumed Natural History of Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHRRPE).

Abstract

PURPOSE\nTo correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age.\n\n\nDESIGN\nRetrospective study.\n\n\nSUBJECTS\nThere were 50 eyes of 49 patients (age range 1-74 years) with CHRRPE studied at nine tertiary vitreoretinal institutions.\n\n\nMETHODS\nWe analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the optical coherence tomography (OCT) findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption and retinal pigment epithelium/Bruch s membrane complex involvement of CHRRPE.\n\n\nMAIN OUTCOMES\nClinical and imaging findings of CHRRPE at different ages.\n\n\nRESULTS\nAnalysis of 50 CHRRPE revealed younger patients were more likely to have partial thickness involvement of the retina (p = 0.009) with predominantly inner retinal layer involvement (p = 0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to have an increase in central macular thickness independently of tumor location.\n\n\nCONCLUSION\nBased on these findings, we believe that CHRRPE typically begins in the inner retina and continues towards the outer retina over time, with increase in central macular thickness, despite the location of the tumor.