Forte Paolo, Paques Michel, Cattaneo Jennifer, Dupas Bénédicte, Castro-Farias Daniela, Girmens Jean-François, Siab Mehdi, Biagini Francesco, Nicolò Massimo, Eandi Chiara Maria, Sacconi Riccardo, Querques Giuseppe
Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, Department of Ophthalmology, University of Lausanne, Switzerland; IRCCS Ospedale Policlinico San Martino, Eye Unit, Genoa, Italy; DINOGMI, University of Genoa, Italy.
Hôpital des Quinze-Vingts, Centre d'Investigation Clinique 1423, INSERM, Paris, France; FRCRNet/FCRIN Network, Paris, France.
Surv Ophthalmol. 2025 May-Jun;70(3):369-379. doi: 10.1016/j.survophthal.2025.01.007. Epub 2025 Jan 15.
Focal capillary ectasia in the macular region can manifest in distinct clinical scenarios, which can be categorized into 2 main entities: perifoveal vascular anomalous complex (PVAC) and telangiectatic capillaries (TelCaps). PVAC represents a primary, idiopathic condition, whereas TelCaps occur secondary to underlying vascular disorders, including diabetic macular edema and retinal vein occlusion. We provide a comprehensive analysis of these 2 entities, encompassing their clinical presentations, multimodal imaging findings, histological evidence, and differential diagnosis from other retinal microvascular abnormalities, such as Type 1 macular telangiectasia, adult-onset Coats disease, Type 3 macular neovascularization in age-related macular degeneration, and retinal arterial macroaneurysms. Although PVAC and TelCaps are distinct entities, they may share common pathogenic mechanisms, including progressive endothelial dysfunction, pericyte loss, and intraluminal deposition of blood components. Selective laser photocoagulation has emerged as a promising therapeutic approach for both conditions. The proposed standardization of nomenclature for accurate reporting and meaningful cross-study comparisons is expected to facilitate future advancements in this field, ultimately leading to improved patient outcomes.
黄斑区的局灶性毛细血管扩张可在不同的临床情况下表现出来，可分为两个主要类型：黄斑周围血管异常复合体（PVAC）和毛细血管扩张（TelCaps）。PVAC是一种原发性特发性疾病，而TelCaps继发于潜在的血管疾病，包括糖尿病性黄斑水肿和视网膜静脉阻塞。我们对这两个类型进行了全面分析，包括它们的临床表现、多模态成像结果、组织学证据，以及与其他视网膜微血管异常的鉴别诊断，如1型黄斑毛细血管扩张、成人型Coats病、年龄相关性黄斑变性中的3型黄斑新生血管形成和视网膜动脉大动脉瘤。虽然PVAC和TelCaps是不同的类型，但它们可能有共同的致病机制，包括进行性内皮功能障碍、周细胞丢失和血液成分的管腔内沉积。选择性激光光凝已成为这两种情况的一种有前景的治疗方法。为准确报告和进行有意义的跨研究比较而提议的命名标准化，有望促进该领域未来的进展，最终改善患者的治疗效果。
