X 连锁视网膜裂孔症患者视网膜周围神经纤维层变薄。

IF 2 Q2 OPHTHALMOLOGY
Peter Kiraly, Ana Uršula Gavrić, Felix F Reichel, Johannes Birtel, Luca Mautone, Yevgeniya Atiskova, Philipp Herrmann, Martina Jarc-Vidmar, Marko Hawlina, Susan M Downes, M Dominik Fischer
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引用次数: 0

摘要

目的:评估X连锁视网膜裂孔症(XLRS)患者视网膜毛周神经纤维层(pRNFL)的厚度,因为pRNFL变薄可能会限制基因治疗试验中功能的改善:这项回顾性多中心研究包括 25 名确诊为 XLRS 患者的 49 只眼睛。基线和最后一次随访(如有)时通过多模态成像收集的数据包括年龄、最佳记录视力(BRVA)、中心视网膜厚度、黄斑体积(MV)、周围视网膜裂孔的存在和位置,以及全视网膜(G)、颞上视网膜(TS)、颞上视网膜(NS)、颞下视网膜(TI)、颞下视网膜(NI)、鼻侧视网膜(N)和颞侧视网膜(T)的 pRNFL 厚度。结果:72% 的右眼和 79% 的左眼至少有一个视区的 pRNFL 变薄(低于百分位数第五位),其中 20% 的右眼和 17% 的左眼有三个或更多视区变薄。在 44% 的病例中,双眼的颞区均出现视网膜变薄,鼻侧视网膜没有变薄。周边视网膜裂孔象限的数量与变薄的 pRNFL 扇区相匹配。结论:约四分之三的 XLRS 患者会出现 pRNFL 变薄,主要是扇形变薄,与黄斑或周边视网膜裂孔有关,而五分之一的患者会出现弥漫性变薄。时间性的 pRNFL 变薄可能只发生在黄斑视网膜内囊状腔塌陷之后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Peripapillary retinal nerve fibre layer thinning in patients with X-linked retinoschisis.

Aims: To assess peripapillary retinal nerve fibre layer (pRNFL) thickness in patients with X-linked retinoschisis (XLRS), as pRNFL thinning may limit functional improvements in gene therapy trials.

Methods: This retrospective multicentre study included 49 eyes from 25 patients diagnosed with XLRS. Data collected with multimodal imaging at baseline and last follow-up (when available) included age, best-recorded visual acuity (BRVA), central retinal thickness, macular volume (MV), presence and location of peripheral retinoschisis and pRNFL thickness in the global (G), superotemporal (TS), superonasal (NS), inferotemporal (TI), inferonasal (NI), nasal (N) and temporal (T) sectors. Retinal sensitivity, assessed by microperimetry, was also recorded for seven patients at baseline.

Results: pRNFL was thinner (below the fifth percentile) in at least one sector in 72% of right eyes and 79% of left eyes, with thinning across three or more sectors in 20% of right and 17% of left eyes. In 44% of cases, thinning occurred in the temporal sectors of both eyes, with no nasal sectoral thinning. Number of peripheral retinoschisis quadrants matched thinned pRNFL sectors. A strong positive correlation was found between MV and temporal pRNFL thickness (r=0.71, p<0.01), while weak negative correlation trends were noted with age (p=0.05) and BRVA (logMAR; p=0.12) related to temporal thickness of pRNFL sectors.

Conclusion: pRNFL thinning, predominantly sectoral and linked to macular or peripheral retinoschisis, occurs in about three-quarters of patients with XLRS, while diffuse thinning occurs in one-fifth. Temporal pRNFL thinning might occur only after the collapse of intraretinal cystoid cavities in the macula.

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来源期刊
BMJ Open Ophthalmology
BMJ Open Ophthalmology OPHTHALMOLOGY-
CiteScore
3.40
自引率
4.20%
发文量
104
审稿时长
20 weeks
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