Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, Los Angeles, California; and.
Greater Los Angeles Veteran's Healthcare Center, Los Angeles, California.
Retin Cases Brief Rep. 2023 Nov 1;17(6):664-667. doi: 10.1097/ICB.0000000000001276.
BACKGROUND/PURPOSE: The purpose of this study was to describe the characteristic pattern of progression of pentosan polysulfate (PPS) maculopathy with multimodal retinal imaging in two patients, including one with over 9 years of follow-up.
Two patients with PPS maculopathy were sequentially evaluated with near-infrared reflectance (NIR) and optical coherence tomography.
Near-infrared reflectance showed characteristic centrifugal progression of the parafoveal hyperreflective lesions toward the vascular arcades with the development of hyporeflective areas in both cases. Optical coherence tomography demonstrated focal retinal pigment epithelium (RPE) thickening that corresponded to the hyperreflective lesions on NIR. On subsequent optical coherence tomography scans, the hyperreflective areas resolved with the development of ellipsoid zone attenuation, RPE disruption, and atrophy, which colocalized with hyporeflectivity on NIR.
This report describes the progression of pentosan polysulfate maculopathy over almost 10 years of PPS treatment and highlights the importance of NIR as a tool for the diagnosis and monitoring of PPS maculopathy. Pentosan polysulfate lesions present as areas of focal RPE thickening with ensuing development of ellipsoid zone loss and RPE drop-out. The pathophysiology of PPS toxicity is unknown and may either result from primary RPE or choroidal toxicity.
背景/目的：本研究的目的是描述 2 例患者多模态视网膜成像中聚戊烯多糖（PPS）性黄斑病变的特征性进展模式，其中 1 例患者的随访时间超过 9 年。
对 2 例 PPS 黄斑病变患者进行近红外反射（NIR）和光相干断层扫描（OCT）的序贯评估。
近红外反射显示，病变呈向血管弓呈离心性进展的特征性改变，在 2 例患者中均出现了低反射区域。OCT 显示局灶性视网膜色素上皮（RPE）增厚，与 NIR 上的高反射病变相对应。在随后的 OCT 扫描中，高反射区域消退，出现椭圆体带衰减、RPE 破裂和萎缩，与 NIR 上的低反射区域相吻合。
本报告描述了近 10 年 PPS 治疗期间聚戊烯多糖黄斑病变的进展情况，并强调了 NIR 作为诊断和监测 PPS 黄斑病变的工具的重要性。聚戊烯多糖病变表现为局灶性 RPE 增厚，随后出现椭圆体带丢失和 RPE 脱落。PPS 毒性的发病机制尚不清楚，可能是由于 RPE 或脉络膜毒性引起的。
