Clinical Manifestations of Cuticular Drusen: Current Perspectives

Abstract

Serena Fragiotta,1 Pedro Fernández-Avellaneda,2 Mark P Breazzano,3 Gianluca Scuderi1 1NESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome “La Sapienza”, Rome, Italy; 2Department of Ophthalmology, Basurto University Hospital, Bilbao, Spain; 3Wilmer Eye Institute, Johns Hopkins Hospital, Johns Hopkins University, Baltimore, MD, UACorrespondence: Serena FragiottaNESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome “La Sapienza”, Via di Grottarossa 1035-1039, Rome, 00189, ItalyTel [phone]Fax [phone]Email [email protected]: Cuticular drusen are part of the spectrum of age-related macular degeneration (AMD) with particular clinical and multimodal imaging characteristics. This drusen subpopulation shares several high-risk single nucleotide polymorphisms with AMD. Despite this feature, they can manifest at a relatively young age, presenting with a female preponderance. Multimodal imaging is essential for characterizing such lesions, using a combination of color fundus photographs, optical coherence tomography (OCT), fluorescein angiography (FA), and fundus autofluorescence (FAF). The classic starry-sky pattern visible on FA and the typical central hypoautofluorescent lesion with hyperautofluorescent rim on FAF is considered the result of a central retinal pigment epithelium (RPE) erosion from these triangular elevations of the RPE-basal lamina. This finding may also be responsible for the typical choroidal hypertransmission appreciated through OCT. The clinical course of cuticular drusen may be relatively benign at early stages, with small drusen presenting at a young age. However, the presence of clinical phenotypes characterized by diffuse involvement and/or accompanying large drusen in patients older than 60 years may confer a significant risk for either macular neovascularization or geographic atrophy.Keywords: cuticular drusen, spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, multimodal imaging

Keywords

• cuticular drusen
• spectral-domain optical coherence tomography
• fundus autofluorescence
• fluorescein angiography
• multimodal imaging;