Purpose

To compare choriocapillaris flow deficit (CC-FD) analysis using optical coherence tomography angiography (OCTA) in eyes of patients treated with high cumulative dosages of pentosan polysulfate sodium (PPS) but no signs of retinal toxicity versus healthy age-matched controls.

Design

Retrospective clinical cohort study.

Methods

Patients treated with PPS for interstitial cystitis with a cumulative dose of > 1000 g underwent multimodal imaging screening to exclude evidence of PPS maculopathy or other retinal findings. All study patients and age-matched healthy controls completed a 3 × 3 mm macular volume scan OCTA using the SOLIX full-range OCT. En face OCTA images at the level of the CC were exported and CC-FDs were computed and compared between groups.

Results

Fifteen patients treated with PPS and 15 age-matched controls were included. The mean PPS cumulative dose was 1974 ± 666 g over a mean of 17.6 ± 6.8 treatment years. All patients registered a visual acuity of 20/25 or better and normal fundus autofluorescence (FAF), OCT, multicolor, near-infrared reflectance (NIR), and ultra-widefield fundus color and autofluorescence images. The CC-FD was 32.7 ± 3.6% in the PPS group compared with 28.6 ± 4.3% in the control group (P = .023).

Conclusions

Patients treated with PPS long enough to accumulate dosages > 1000 g showed significant CC flow impairment before the development of macular toxicity signs with OCT, NIR, and FAF compared with age-matched normal controls. Thus, the choroid may be the earliest manifestation of ocular toxicity, predating the development of clinically evident retinal pigment epithelium (RPE) injury. The subsequent RPE disruption may be the result of choriocapillaris impairment or primary PPS toxicity. Assessment of the CC on OCTA may be a useful tool for early detection of toxicity, although further longitudinal studies are required.

中文翻译：

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戊聚糖多硫酸钠相关性黄斑病：使用 OCT 血管造影和脉络膜毛细血管血流不足分析进行早期检测

目的

比较使用光学相干断层扫描血管造影术 (OCTA) 对接受高累积剂量戊聚糖多硫酸钠 (PPS) 治疗但没有视网膜毒性迹象的患者与健康年龄匹配对照的脉络膜毛细血管血流不足 (CC-FD) 分析。

设计

回顾性临床队列研究。

方法

接受累积剂量 > 1000 g 的 PPS 治疗间质性膀胱炎的患者接受了多模态成像筛查，以排除 PPS 黄斑病变或其他视网膜发现的证据。所有研究患者和年龄匹配的健康对照使用 SOLIX 全范围 OCT 完成 3 × 3 mm 黄斑体积扫描 OCTA。导出 CC 级别的面部 OCTA 图像，计算 CC-FD 并在组间进行比较。

结果

包括 15 名接受 PPS 治疗的患者和 15 名年龄匹配的对照组。在平均 17.6 ± 6.8 年的治疗期间，平均 PPS 累积剂量为 1974 ± 666 g。所有患者的视力均达到 20/25 或更高，并且眼底自发荧光 (FAF)、OCT、多色、近红外反射 (NIR) 以及超广角眼底彩色和自发荧光图像均正常。PPS 组的 CC-FD 为 32.7 ± 3.6%，而对照组为 28.6 ± 4.3% ( P  = .023)。

结论

与年龄匹配的正常对照相比，使用 PPS 治疗时间足够长以累积剂量 > 1000 g 的患者在 OCT、NIR 和 FAF 黄斑毒性征兆发展之前显示出显着的 CC 血流障碍。因此，脉络膜可能是眼部毒性的最早表现，早于临床上明显的视网膜色素上皮 (RPE) 损伤的发展。随后的 RPE 中断可能是脉络膜毛细血管损伤或原发性 PPS 毒性的结果。尽管需要进一步的纵向研究，但对 OCTA 的 CC 评估可能是早期检测毒性的有用工具。