From the Newcastle Eye Centre (Papastavrou, O'Brien, Ray-Chaudhuri, Browning), Royal Victoria Infirmary, the Institute of Genetic Medicine (Browning), International Centre for Life and the Institute of Health and Society (Vieira), Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, and the Department of Ophthalmology (Borooah, Dhillon), Princess Alexandra Eye Pavilion, Edinburgh, Scotland, United Kingdom.
From the Newcastle Eye Centre (Papastavrou, O'Brien, Ray-Chaudhuri, Browning), Royal Victoria Infirmary, the Institute of Genetic Medicine (Browning), International Centre for Life and the Institute of Health and Society (Vieira), Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, and the Department of Ophthalmology (Borooah, Dhillon), Princess Alexandra Eye Pavilion, Edinburgh, Scotland, United Kingdom.
J Cataract Refract Surg. 2017 Aug;43(8):1036-1043. doi: 10.1016/j.jcrs.2017.05.041.
To review the outcomes in a series of patients with long anterior lens zonular fibers associated with late-onset retinal degeneration who had phacoemulsification cataract surgery.
Newcastle Eye Centre, Newcastle upon Tyne, United Kingdom.
Retrospective case series.
Inclusion criteria were patients with genetically confirmed late-onset retinal degeneration requiring cataract surgery. Perioperative data relating to surgery were collected. In addition, the corrected distance visual acuity (CDVA) and retinal imaging data were recorded. Selected lens capsules were examined using immunohistochemistry or scanning electron microscopy (SEM).
Eleven eyes of 7 patients were included. The long anterior lens zonular fibers made capsulorhexis challenging; however, it was completed safely in all cases. There were no intraoperative or postoperative issues with lens stability. The CDVA improved postoperatively in those cases with intact foveal photoreceptors and retinal pigment epithelium. Over the longer term, the CDVA slowly declined because of progressive atrophy of the macula. Most patients noticed a subjective improvement in vision, even those with advanced disease at baseline. Immunohistochemistry showed that the C1QTNF5 protein was expressed within the lens capsule epithelial cells, although SEM of the long anterior lens zonular fibers showed them to be smaller in diameter than normal anterior lens zonular fibers and to be composed of a helix of fibers.
In this small series of patients with late-onset retinal degeneration, cataract surgery was successfully performed without long-term complications involving intraocular lens stability. The objective improvement in CDVA seemed to be limited to patients with good foveal photoreceptor architecture.
回顾一系列伴有迟发性视网膜变性的长前晶状体悬韧带纤维患者的手术结果，这些患者均行超声乳化白内障吸除术。
英国泰恩河畔纽卡斯尔的纽卡斯尔眼科中心。
回顾性病例系列。
纳入标准为经基因证实患有需要白内障手术的迟发性视网膜变性的患者。收集与手术相关的围手术期数据。此外，还记录了矫正视力（CDVA）和视网膜成像数据。使用免疫组织化学或扫描电子显微镜（SEM）检查选定的晶状体囊。
7 名患者的 11 只眼被纳入研究。长的前晶状体悬韧带纤维使撕囊变得具有挑战性；但是，在所有情况下均安全完成。晶状体稳定性在手术过程中或手术后均未出现问题。在具有完整的中心凹光感受器和视网膜色素上皮的病例中，术后 CDVA 得到改善。从长远来看，由于黄斑逐渐萎缩，CDVA 缓慢下降。大多数患者注意到视力的主观改善，即使是那些在基线时有晚期疾病的患者。免疫组织化学显示 C1QTNF5 蛋白在晶状体囊上皮细胞中表达，尽管 SEM 显示长的前晶状体悬韧带纤维的直径比正常的前晶状体悬韧带纤维小，并且由纤维的螺旋组成。
在这一系列患有迟发性视网膜变性的小患者中，白内障手术成功进行，没有涉及眼内晶状体稳定性的长期并发症。CDVA 的客观改善似乎仅限于具有良好中心凹光感受器结构的患者。
