Clinical Ophthalmology (Sep 2022)
Late-Onset Retinal Degeneration: Clinical Perspectives
Abstract
Leonardo Lando,1,2 Shyamanga Borooah1 1Shiley Eye Institute, University of California San Diego, La Jolla, CA, USA; 2Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, ON, CanadaCorrespondence: Shyamanga Borooah, Shiley Eye Institute, University of California San Diego, 9415 Campus Point Drive, La Jolla, CA, 92093, USA, Email [email protected]: Late-onset retinal degeneration (L-ORD) is a type of retinal dystrophy marked by nyctalopia and subretinal pigment epithelium deposits, which eventually promote retinal atrophy with final visual compromise. L-ORD may also present with changes in the anterior segment, notably long anterior zonules and iris atrophy, distinguishing it from other inherited eye conditions. Although it can clinically simulate age-related macular degeneration, L-ORD has a different course of progression and prognosis, requiring adequate diagnosis for patient counseling. This review summarizes the main clinical, genetic, pathophysiological, diagnostic, and therapeutic aspects of L-ORD to help ophthalmologists identify and manage this rare ocular disease.Keywords: retinal dystrophy, retinal degeneration, macular dystrophy, late-onset retinal degeneration, C1QTNF5, CTRP5
