Higgins Bethany E, Rodriguez-Martinez Ana Catalina, Montesano Giovanni, Tailor-Hamblin Vijay K, Malka Samantha, Henderson Robert H, Moosajee Mariya
UCL Institute of Ophthalmology, London EC1V 9EL, UK.
Moorfields Eye Hospital NHS Foundation Trust, London EC1V 2PD, UK.
Biomedicines. 2025 Feb 21;13(3):555. doi: 10.3390/biomedicines13030555.
A cross-sectional study was conducted at Moorfields Eye Hospital, UK, involving patients with -associated retinopathies: macular dystrophy (MD), cone-rod dystrophy (CORD), and early-onset severe retinal dystrophy/Leber congenital amaurosis (EOSRD/LCA). The study aimed to evaluate -associated retinopathies using microperimetry (macular integrity assessment (S-MAIA) fast protocol) and spectral domain optical coherence tomography (SD-OCT). Data quality and participant attrition were assessed in 18 patients (10 MD, 5 EOSRD/LCA, 3 CORD), aged 10-52 years, with a median best corrected visual acuity (BCVA) of 0.41 logMAR. Microperimetry and SD-OCT data were obtained from 14 and 18 patients, respectively, but eccentric fixation hindered structure-function analysis. All participants showed overall abnormal sensitivity on the S-MAIA fast protocol. Parafoveal volume was significantly increased, while foveal thickness and volume were reduced compared to normative data ( < 0.01). This study highlights the challenges of participant attrition and the need for alternative functional metrics to complement traditional evaluations. It also reinforces previous findings of abnormal retinal architecture in -associated retinopathies, providing further insights into S-MAIA and SD-OCT assessments for this patient population.
一项横断面研究在英国摩尔菲尔德眼科医院开展，研究对象为患有相关视网膜病变的患者：黄斑营养不良（MD）、视锥视杆营养不良（CORD）以及早发性严重视网膜营养不良/莱伯先天性黑蒙（EOSRD/LCA）。该研究旨在使用微视野计（黄斑完整性评估（S-MAIA）快速方案）和频域光学相干断层扫描（SD-OCT）来评估相关视网膜病变。对18例患者（10例MD、5例EOSRD/LCA、3例CORD）进行了数据质量和参与者损耗评估，这些患者年龄在10至52岁之间，最佳矫正视力（BCVA）中位数为0.41 logMAR。分别从14例和18例患者中获取了微视野计和SD-OCT数据，但偏心注视妨碍了结构-功能分析。所有参与者在S-MAIA快速方案上均显示出整体异常的敏感度。与正常数据相比，旁中心凹体积显著增加，而中心凹厚度和体积减小（<0.01）。本研究凸显了参与者损耗带来的挑战以及需要替代功能指标来补充传统评估。它还强化了先前关于相关视网膜病变中视网膜结构异常的研究结果，为该患者群体的S-MAIA和SD-OCT评估提供了进一步的见解。
