Aims: This study describes vascular abnormalities in X-linked retinoschisis (XLRS) using fundus fluorescein angiography (FFA) and ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) to better understand the disease's vascular features and impact. Methods: A retrospective cross-sectional study was conducted on 26 XLRS patients (46 eyes). A comprehensive ophthalmic examination was performed, including FFA and UWF SS-OCTA. FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types. Results: The mean age of patients was 11.3±6.5 years. Macular schisis appeared in 97.8% of eyes, peripheral schisis in 89.1%, and peripheral bullous schisis (PBS) in 67.39%. Major vascular changes identified by FFA included dendritic capillary dilation/leakage (91.3%), internal residual vessel leakage (78.3%), and capillary dropout/ischemia (71.7%). Minor changes included zonal retinal pigment epithelium (RPE) proliferation (6.5%), bridging vessels (4.4%), and capillary sheathing (4.4%). peripapillary choroidal neovascularization (PPCNV) was noted in 10.9% and situs inversus of optic disc in 13.0% of eyes. Additionally, situs inversusof optic disc and zonal RPE proliferation were novel findings. Major FFA changes correlated with broader PBS (P = 0.045) (P ＜ 0.001) (P = 0.003). Clock hours of PBS were significant predictors for internal residual vessel leakage (OR = 0.30, P = 0.03). No significant correlation was found between gene mutation type and FFA abnormalities(P = 1.000)(P = 0.539). Conclusions: This study highlighted the significant prevalence (95.7%) of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation, enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.

Aims: This study describes vascular abnormalities in X-linked retinoschisis (XLRS) using fundus fluorescein angiography (FFA) and ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) to better understand the disease's vascular features and impact. Methods: A retrospective cross-sectional study was conducted on 26 XLRS patients (46 eyes). A comprehensive ophthalmic examination was performed, including FFA and UWF SS-OCTA. FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types. Results: The mean age of patients was 11.3±6.5 years. Macular schisis appeared in 97.8% of eyes, peripheral schisis in 89.1%, and peripheral bullous schisis (PBS) in 67.39%. Major vascular changes identified by FFA included dendritic capillary dilation/leakage (91.3%), internal residual vessel leakage (78.3%), and capillary dropout/ischemia (71.7%). Minor changes included zonal retinal pigment epithelium (RPE) proliferation (6.5%), bridging vessels (4.4%), and capillary sheathing (4.4%). peripapillary choroidal neovascularization (PPCNV) was noted in 10.9% and situs inversus of optic disc in 13.0% of eyes. Additionally, situs inversusof optic disc and zonal RPE proliferation were novel findings. Major FFA changes correlated with broader PBS (P = 0.045) (P ＜ 0.001) (P = 0.003). Clock hours of PBS were significant predictors for internal residual vessel leakage (OR = 0.30, P = 0.03). No significant correlation was found between gene mutation type and FFA abnormalities(P = 0.539). Conclusions: This study highlighted the significant prevalence (95.7%) of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation, enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.

目 的: 比 较 不 同 能 量 氪 离 子 黄 绿 光 联 合 Nd ∶YAG 激 光 行 周 边 虹 膜 切 开 术 的 临 床 疗 效 和 对 眼压、血- 房 水 屏 障 的 影 响。

方 法: 使 用 激 光 蛋 白 细 胞 仪 及 压 平 式 眼 压 计 对 31 例(62 只 眼) 接 受 高 能 量 组 700 mW, 低 能量 组 400 mW的 氪 离 子 联 合 Nd: YAG 激 光 行 周 边 虹 膜 切 除 术 前、术 后 的 房 水 蛋 白 浓 度、前 房细 胞 数 和 眼 压 进 行 比 较。术 后 随 访 1 个 月。

结 果: 激 光 周 边 虹 膜 切 开 术 两 组 周 切 口 通 畅 无 闭 合, 无 角 膜 损 伤 及 晶 体 混 浊。 术 前 和 术 后1 h、3 d、7 d 及 1 个月的平均 Goldmann 眼压值在高能量组分别为 ( 15.68±2.41) 、(27.13±3.48) 、(20.97±5.27) 、(16.35±1.14) 、(15.06±2.02) , 在 低 能 量 组 分 别 为( 15.35±1.78) 、(22.77±3.26) 、(16.26±2.41) 、(15.68±2.06) 、( 15.06±1.36) 。术 前 和 术 后 3 d、7 d 及 1 个 月 的 平 均 房 水 闪 光 值在 高 能 量 组 分 别 为( 4.65±1.50) 、(10.41±2.47) 、(7.31±2.31) 、(6.15±2.16) , 在 低 能 量 组 分 别 为(4.45±1.19) 、(6.47±1.11) 、(4.81±0.55) 、(4.98±1.48) pc/ms; 前 房 细 胞 数 平 均 值 在 高 能 量 组 分别 为( 0.47±0.42) 、( 36.22±9.16) 、(18.54±3.60) 、( 6.29±0.98) , 在 低 能 量 组 分 别 为(0.58±0.52) 、(24.73±6.09) 、(10.61±1.70) 、(2.96±1.35) 。高 能 量 组 术 后 1 h 及 第 3 天 眼 压 的 升 高 幅 度 较 低能 量 组 高, 术 后 各 点 的 房 水 闪 光 值 和 前 房 细 胞 数 升 高 幅 度 均 较 低 能 量 组 明 显, 术 后 1 个 月 两组 前 房 细 胞 数 及 高 能 量 组 房 水 闪 光 值 仍 未 能 降 至 术 前 水 平, 差 异 有 显 著 性 意 义( P < 0.05) 。

结 论: 低 能 量 与 高 能 量 氪 黄 绿 激 光 联 合 Nd ∶YAG 激 光 周 边 虹 膜 切 开 术 两 组 周 切 口 通 畅, 临 床效 果 相 同; 低 能 量 组 术 后 一 过 性 眼 压 升 高 恢 复 至 正 常 较 快, 前 房 蛋 白 较 快 恢 复 正 常。提 示 在 进行 激 光 周 边 虹 膜 切 开 术 时 应 尽 可 能 用 低 能 量。两 组 术 后 1 个 月 前 房 仍 有 细 胞, 应 继 续 随 访。

Purpose: To compare the influence of krypton laser with different power densities combined with Nd: YAG laser peripheral iridotomy (LPI) on the intraocular pressure, blood-aqueous barrier and inflammatory of anterior chamber as well as the therapeutic effect.

Methods: Using a laser flare cell meter and Goldmann tonometer, the level of aqueous protein, the number of cells in the anterior chamber and intraocular pressure of 31 patients (62 eyes) who underwent krypton laser with different power densities combined with Nd: YAG laser peripheral iridotomy were examined and recorded preoperatively and postoperatively.

Results: The mean preoperative and 1-hour, 3-day, 7-day, 1-month postoperative intraocular pressure ( IOP) of the high power-density group were (15.68±2.41) , (27.13 ±3.48) , (20.97 ±5.27) , (16.35 ±1.14) and (15.06 ±2.02) mmHg, while those of the low were (15.35±1.78) , (22.77±3.26) , (16.26±2.41) , (15.68±2.06) and (15.06±1.36) mmHg. The mean preoperative and 3-day, 7-day, 1-month postoperative flare intensity of the high power-density group were (4.65±1.50) , (10.41±2.47) , (7.31±2.31) and (6.15±2.16) pc /ms, while those of the low were (4.45±1.19) , (6.47±1.11) , (4.81±0.55) and (4.98±1.48) pc/ms. The number of aqueous cells of the high was (0.47±0.42) , (36.22±9.16) , ( 18.54±3.60) and (6.29±0.98) , while that of the low was (0.58±0.52) , (24.73±6.09) , (10.61±1.70) and (2.96±1.35) . The mean 1-hour and 3-day postoperative IOP of the high was higher than that of the low. Both the mean flare intensity and the mean number of aqueous cells of the high power-density group were higher than those of the low. The differences were of statistical significance (P <0.05) . The mean flare intensity of the high power-density group in the 1-month postoperative follow-up was still higher than the baseline. The mean number of aqueous cells of both the high and the low power-density groups in the 1- month postoperative follow-up was still higher than the baseline. During 1-month follow-up, no obvious visual damage, diffuse corneal endothelial burns or corneal decompensation, lens injury and closure of the peripheral iris incision were observed.

Conclusion: When krypton laser combined with Nd: YAG laser peripheral iridotomy is under consideration, relatively low power-density krypton laser is recommended because it can achieve the similar therapeutic effects as high power-density krypton laser but leads to less complications and a briefer recovery. More follow-ups are needed after LPI, because the number of aqueous cells in 1-month follow-up was still abnormal. 

眼眶部副神经节瘤极为罕见，多属非功能性肿瘤。本病例为1位中年女性，以右眼眼睑抬举无力为主诉就诊，眼眶计算机断层扫描(computed tomography，CT)和磁共振成像(magnetic resonance imaging，MRI)检查发现右眼眼眶内肿物，手术完整切除，结合HE染色和免疫组织化学检查，病理组织学诊断为眼眶副神经节瘤，随访3年，肿瘤无复发。

Orbital paraganglioma is a rare disease and mostly belongs to non-functional tumors. In this report, we described a middle-aged female admitted to our hospital with the chief complaint of weak lifting of her right eyelid. Orbital computed tomography (CT) and magnetic resonance imaging (MRI) examination detected an intra-orbital mass in the right eye. Complete excision was conducted, and post-operative histopathological and immunohistochemical examination revealed the mass to be orbital paraganglioma. Within the 3-year follow-up,the patient showed no recurrence.

眼眶部副神经节瘤极为罕见，多属非功能性肿瘤。本病例为1位中年女性，以右眼眼睑抬举无力为主诉就诊，眼眶计算机断层扫描(computed tomography，CT)和磁共振成像(magnetic resonance imaging，MRI)检查发现右眼眼眶内肿物，手术完整切除，结合HE染色和免疫组织化学检查，病理组织学诊断为眼眶副神经节瘤，随访3年，肿瘤无复发。

Orbital paraganglioma is a rare disease and mostly belongs to non-functional tumors. In this report, we described a middle-aged female admitted to our hospital with the chief complaint of weak lifting of her right eyelid. Orbital computed tomography (CT) and magnetic resonance imaging (MRI) examination detected an intra-orbital mass in the right eye. Complete excision was conducted, and post-operative histopathological and immunohistochemical examination revealed the mass to be orbital paraganglioma. Within the 3-year follow-up, the patient showed no recurrence.