Fragiotta Serena, Fernández-Avellaneda Pedro, Breazzano Mark P, Scuderi Gianluca
NESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome "La Sapienza", Rome, Italy.
Department of Ophthalmology, Basurto University Hospital, Bilbao, Spain.
Clin Ophthalmol. 2021 Sep 21;15:3877-3887. doi: 10.2147/OPTH.S272345. eCollection 2021.
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.
角质样玻璃膜疣是年龄相关性黄斑变性(AMD)谱系的一部分,具有特定的临床和多模态影像学特征。这种玻璃膜疣亚群与AMD共享几种高风险单核苷酸多态性。尽管有此特征,但它们可在相对年轻时出现,女性更为常见。多模态成像对于表征此类病变至关重要,可结合彩色眼底照片、光学相干断层扫描(OCT)、荧光素血管造影(FA)和眼底自发荧光(FAF)。FA上可见的经典星空图案以及FAF上典型的中央低自发荧光病变伴高自发荧光边缘被认为是视网膜色素上皮(RPE)从这些RPE - 基底膜的三角形隆起处侵蚀的结果。这一发现也可能是通过OCT观察到的典型脉络膜高透过率的原因。角质样玻璃膜疣的临床过程在早期可能相对良性,小玻璃膜疣在年轻时出现。然而,60岁以上患者中以弥漫性受累和/或伴有大玻璃膜疣为特征的临床表型的存在可能会使黄斑新生血管形成或地图样萎缩的风险显著增加。