Tang Peter H, Jauregui Ruben, Tsang Stephen H, Bassuk Alexander G, Mahajan Vinit B
Ophthalmic Surg Lasers Imaging Retina. 2019 Feb 1;50(2):e44-e48. doi: 10.3928/23258160-20190129-18.
RPGR-associated retinitis pigmentosa (RP) is a progressive disease with retina degeneration. Optical coherence tomography angiography (OCTA) is an imaging technique that provides novel insights. The authors report two affected male siblings who underwent OCTA imaging. The area of the foveal avascular zone (FAZ) was measured. Although the younger sibling exhibited more advanced clinical disease, his visual acuity was superior to his older sibling. OCTA imaging revealed a better preserved FAZ in the younger sibling as the reason for this. It also highlighted attenuation of choriocapillaris / choroid layers as biomarkers for disease severity. This provides new insights into retinal degeneration in RPGR-associated RP. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e44-e48.].
RPGR相关的视网膜色素变性（RP）是一种伴有视网膜变性的进行性疾病。光学相干断层扫描血管造影（OCTA）是一种能提供新见解的成像技术。作者报告了两名接受OCTA成像的患病男性同胞。测量了中心凹无血管区（FAZ）的面积。尽管弟弟表现出更晚期的临床疾病，但他的视力优于哥哥。OCTA成像显示，弟弟的FAZ保存得更好，这就是原因所在。它还突出了脉络膜毛细血管/脉络膜层的衰减作为疾病严重程度的生物标志物。这为RPGR相关RP的视网膜变性提供了新的见解。[《眼科手术、激光与视网膜成像》。2019；50：e44 - e48。]
