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--- 在先天性X连接视网膜瘤中进行Pars plana玻璃切除术:一个范围审查

Sakshi Shiromani¹, Kevin Ferenchak¹, Joshua Barnett¹

¹Department of Ophthalmology, Emory University School of Medicine, 1365B Clifton Road, NE, Suite 2400, Atlanta, GA, 30322, USA.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie

|September 6, 2025

概括

帕斯平面玻璃切除术 (PPV) 可以改善X连接视网膜裂变 (XLRS) 的视觉敏度和分裂腔体积. 对现有研究的审查为基因治疗结果提供了洞察力,并指出视网膜脱落修复的挑战.

科学领域:

眼科眼科
视网膜退行性疾病
外科创新

背景情况:

遗传性X连接视网膜分裂 (XLRS) 是一种遗传性疾病,导致视力丧失,治疗选择有限.
使用RS1进行子视网膜基因疗法对XLRS具有前景,但通过帕斯普拉纳玻璃切除术 (PPV) 进行基因疗法需要了解PPV的影响.

研究的目的:

进行现有文献的范围审查,以评估X链接视网膜分裂 (XLRS) 患者的Pars plana视网膜切除术 (PPV) 的结果.
评估PPV的安全性和有效性,以治疗骨分裂和其他XLRS相关的并发症,如视网膜脱落 (RD).

主要方法:

一个涉及XLRSPPV的25项研究的范围审查,按手术适应症分类:骨分裂 (类别1) 或其他类似RD的后果 (类别2).
研究分析了视觉和解剖结果,包括骨分裂状态 (OCT) 和视网膜重新连接率,随访时间至少为3个月.

主要成果:

在第1类 (骨分裂) 中,92%的眼睛表现出改善,平均视力敏度从约20/250提高到约20/80 (p=0.0002).
在类别2 (其他后果) 中,PPV对RD的最终重新连接率达到90. 2%,并且在56%的眼睛中改善了VA;在其他并发症的眼睛中改善了VA的76. 5%.
并发症包括白内障 (19%),黄斑折叠 (2.2%) 和新血管绿内障 (2.2%).

结论:

帕斯平面玻璃切除术 (PPV) 可能对X链接视网膜瘤 (XLRS) 的视腔体积和视觉敏度有好处,安全性可控.
单次手术成功率较低的XLRS相关的视网膜脱落强调了这些眼睛的复杂手术挑战.
这些发现需要仔细考虑PPV的作用,并解释涉及XLRS新疗法的研究结果,例如基因疗法.

关键词:

基因治疗遗传性视网膜疾病帕斯普拉纳玻璃切除术有关X的视网膜分裂

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