Jacob Julie, Krivosic Valérie, Paques Michel, Tadayoni Ramin, Gaudric Alain
*Assistance Publique-Hôpitaux de Paris AP-HP, Hôpital Lariboisière, Department of Ophthalmology, Université Paris Diderot, Sorbonne Paris Cité, Paris, France; †Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium; and ‡Clinical Investigation Center 1423, Centre Hospitalier National des Quinze-Vingts, Institut National de la Santé et de la Recherche Médicale & Université Pierre et Marie Curie, Paris, France.
Retina. 2016 Mar;36(3):545-51. doi: 10.1097/IAE.0000000000000737.
To determine whether cone photoreceptors are impaired early in macular telangiectasia type 2 (MacTel 2) progression.
Eight patients with MacTel 2 lacking intraretinal cavitation underwent multimodal retinal imaging including adaptive optics. Cone packing metrics were determined in 5 sampling windows at different eccentricities from the fovea. Results were compared with a previously established normative database.
In MacTel 2 eyes, cone density was significantly lower than normal at all eccentricities (P < 0.0001). Mean cone spacing and mean percentage of hexagonally organized cone photoreceptors were respectively significantly larger and lower than normal at all eccentricities (P = 0.0488 and P < 0.0001). In MacTel 2 patients, adaptive optics showed an irregular patchy disturbance of the cone mosaic corresponding to some fragmentation of the interdigitation zone on optical coherence tomography. The ellipsoid zone remained intact in the studied area.
Adaptive optics showed that the macular cone density was lower than normal even outside the telangiectasia in MacTel 2 lacking intraretinal cavitation, although the ellipsoid zone remained intact on optical coherence tomography. These findings do not indicate that the cone density loss is causative of the disease as it might be secondary to Müller cell or rod loss in this area. However, cone density assessment could become a useful parameter to monitor disease progression.
确定在2型黄斑毛细血管扩张症（MacTel 2）进展的早期，视锥光感受器是否受损。
对8例无视网膜内空洞的MacTel 2患者进行了包括自适应光学在内的多模态视网膜成像。在距中央凹不同偏心度的5个采样窗口中测定视锥细胞排列指标。将结果与先前建立的正常数据库进行比较。
在MacTel 2患者眼中，所有偏心度下的视锥细胞密度均显著低于正常水平（P < 0.0001）。所有偏心度下，平均视锥细胞间距和六边形排列的视锥光感受器的平均百分比分别显著大于和低于正常水平（P = 0.0488和P < 0.0001）。在MacTel 2患者中，自适应光学显示视锥细胞镶嵌存在不规则的斑片状干扰，对应于光学相干断层扫描上指状交叉区的一些碎片化。在所研究区域，椭圆体带保持完整。
自适应光学显示，在无视网膜内空洞的MacTel 2中，即使在毛细血管扩张区域之外，黄斑视锥细胞密度也低于正常水平，尽管光学相干断层扫描显示椭圆体带保持完整。这些发现并不表明视锥细胞密度降低是该疾病的病因，因为它可能是该区域Müller细胞或视杆细胞丢失的继发结果。然而，视锥细胞密度评估可能成为监测疾病进展的一个有用参数。
