Academic Journal

Peripapillary retinal nerve fibre layer thinning in patients with X-linked retinoschisis

التفاصيل البيبلوغرافية
العنوان: Peripapillary retinal nerve fibre layer thinning in patients with X-linked retinoschisis
المؤلفون: Kiraly, Peter, Gavrić, Ana Uršula, Reichel, Felix F, Birtel, Johannes, Mautone, Luca, Atiskova, Yevgeniya, Herrmann, Philipp, Jarc-Vidmar, Martina, Hawlina, Marko, Downes, Susan M, Fischer, M Dominik
المساهمون: National Institute for Health
المصدر: BMJ Open Ophthalmology ; volume 9, issue 1, page e001832 ; ISSN 2397-3269
بيانات النشر: BMJ
سنة النشر: 2024
الوصف: 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 (CRT), 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 the right eyes and 79% of the left eyes, with thinning across three or more sectors in 20% of the right and 17% of the left eyes. In 44% of cases, thinning occurred in the temporal sectors of both eyes, with no nasal sectoral thinning. Peripheral retinoschisis quadrants topographically matched the corresponding 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.
نوع الوثيقة: article in journal/newspaper
اللغة: English
DOI: 10.1136/bmjophth-2024-001832
الاتاحة: https://doi.org/10.1136/bmjophth-2024-001832
https://syndication.highwire.org/content/doi/10.1136/bmjophth-2024-001832
Rights: https://creativecommons.org/licenses/by/4.0/
رقم الانضمام: edsbas.7A51DC34
قاعدة البيانات: BASE
الوصف
DOI:10.1136/bmjophth-2024-001832