综述

探索表皮玻璃疣:从多模式影像特点到临床诊疗的全方位解析

Exploration of cuticular drusen: comprehensive analysis from multi-modal imaging characteristics to clinical diagnosis and treatment

:208-214
 
随着多模式影像技术的应用,年龄相关性黄斑变性(age related macular degeneration, AMD)的一种特殊亚型:表皮玻璃疣(cuticular drusen)被发现,其与AMD尤其是非渗出性AMD又称干性(dry)AMD进展密切相关。但目前聚焦于AMD合并表皮玻璃疣的多模式影像特点等的相关文献较少,因此研究和阐明表皮玻璃疣的多模式影像特点、与其他AMD亚型玻璃疣的鉴别诊断、病理生理机制、治疗方法等具有重要的临床意义。运用多模式影像可以看到表皮玻璃疣呈双眼对称的无数散在的大小均匀的黄色视网膜下结节,典型的“星空状”荧光素钠眼底血管造影(fundus fluorescein angiography, FFA)表现, 以及光学相干断 层成像(optical coherence tomography, OCT)中视网膜色素上皮(retinal pigment epithelium, RPE)和Bruch膜之 间的锯齿状隆起。AMD合并表皮玻璃疣患者的分布特点个体差异很大,容易与AMD其他亚型的玻璃疣混淆,需要与硬性玻璃疣、软性玻璃疣、大胶质玻璃疣、Sorsby眼底营养不良(Sorsby's fundus dystrophy , SFD) 等相鉴别。研究表明表皮玻璃疣具有遗传性的类似动脉粥样硬化的发病机制,与RPE分泌的大型脂蛋白颗粒的堆积密切相关。未来可能会有针对补体系统的药物用于延缓表皮玻璃疣相关病变的进展。文章就表皮玻璃疣的临床表现、多模式影像的典型特点、鉴别诊断、遗传学基础、病理生理学发病机制、 并发症以及临床诊疗策略进行综述。

With the application of multimodal imaging techniques, a specific subtype of age-related macular degeneration (AMD),
known as cuticular drusen, has been identified. This subtype is closely associated with the progression of AMD, particularly non-exudative AMD, also referred to as dry AMD. However, there is a scarcity of literature focusing on the multimodal imaging characteristics of AMD combined with cuticular drusen. Therefore, studying and elucidating the multimodal imaging features of cuticular drusen, its differential diagnosis from other AMD subtypes of drusen, pathophysiological mechanisms, and treatment methods holds significant clinical importance. Multimodal imaging reveals cuticular drusenas numerous, symmetrical, evenly sized, yellow subretinal nodules in both eyes, exhibiting a typical "stars-in-the-sky" appearance on fluorescein angiography (FFA) and zigzag elevations between the retinal pigment epithelium (RPE) and Bruch's membrane on optical coherence tomography (OCT). The distribution characteristics of AMD patients with cuticular drusen vary greatly among individuals and can easily be confused with drusen from other AMD subtypes, so the differentiation from hard drusen, soft drusen, large colloidal drusen, and Sorsby's fundus dystrophy (SFD) is necessary. Studies suggest that cuticular drusen have a genetic, atherosclerosis- like pathogenesis which closely related to the accumulation of large lipoprotein particles secreted by the RPE. Future therapies targeting the complement system maybe employed to delay the progression of cuticulardrusen-related lesions. This article reviews the clinical manifestations, typical multimodal imaging features, differential diagnosis, genetic basis, pathophysiological mechanisms, complications, and clinical management strategies of cuticulardrusen.

其他期刊
  • 眼科学报

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
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  • Eye Science

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
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