Table_1_Case report: Sympathetic ophthalmia after vitrectomies in a patient with Von Hippel–Lindau syndrome.XLS

BackgroundSympathetic ophthalmia (SO) is a rare but sight-threatening uveitis, and most observations have been made after typical manifestations occur. This report focuses on the choroidal changes detected by multimodal imaging at the presymptomatic stage of SO, which is implicated in the early recognition of SO.
Case presentationA 21-year-old woman suffered from decreased vision in the right eye and was diagnosed with retinal capillary hemangioblastomas associated with Von Hippel–Lindau syndrome. The patient underwent two 23-G pars plana vitrectomies (PPVs), soon after which typical signs of SO manifested. SO resolved quickly after the oral administration of prednisone and remained stable during the follow-up of more than 1 year. The retrospective analysis revealed preexisting bilaterally increased choroidal thickness, dots of flow void on the choroid, and choriocapillaris en-face slabs in optical coherence tomography angiography (OCTA) after the first PPV, which were all reversed by corticosteroid treatment.
ConclusionThe case report highlights the involvement of the choroid and choriocapillaris at the presymptomatic stage of SO after the first inciting event. Abnormally thickened choroid and flow void dots suggested that SO had started and an ensuing surgery would run the risk of exacerbating SO. OCTA scanning of both eyes should be ordered routinely for patients with a history of trauma or intraocular surgeries, especially before the next surgical intervention. The report also suggests that non-human leukocyte antigen gene variation may also regulate the progression of SO, which requires further laboratory investigations.

背景 交感眼炎（Sympathetic ophthalmia, SO）是一种罕见但可致盲的葡萄膜炎，既往多数研究均聚焦于典型症状出现后的临床观察。本报告旨在探讨交感眼炎症状前期阶段的多模态影像学脉络膜改变，为该病的早期识别提供参考依据。
病例报告 一名21岁女性因右眼视力下降就诊，确诊为伴发冯·希佩尔-林道综合征（Von Hippel–Lindau syndrome）的视网膜毛细血管瘤。患者先后接受两次23G经睫状体平坦部玻璃体切割术（pars plana vitrectomies, PPVs），术后不久即出现交感眼炎的典型体征。经口服泼尼松治疗后，交感眼炎迅速缓解，并在超过1年的随访期间病情保持稳定。回顾性影像学分析显示，首次玻璃体切割术后，患者双眼脉络膜厚度已出现异常增厚，光学相干断层扫描血管成像（optical coherence tomography angiography, OCTA）的脉络膜层面可见血流信号缺失点，脉络膜毛细血管层断层图像亦存在异常表现，上述异常均在糖皮质激素治疗后恢复正常。
结论 本病例报告证实，在首次诱发事件发生后的交感眼炎症状前期阶段，脉络膜及脉络膜毛细血管即已受累。异常增厚的脉络膜与血流缺失点提示交感眼炎已启动，后续手术可能加重病情进展。对于有外伤史或眼内手术史的患者，应常规行双眼光学相干断层扫描血管成像检查，尤其在计划实施再次手术干预前。本研究同时提示，非人类白细胞抗原基因变异或可调控交感眼炎的疾病进程，该结论有待进一步实验室研究验证。