关键词: Stargardt disease X-linked retinoschisis best disease choroideremia familiar exudative vitreoretinopathy pars plana vitrectomy retinitis pigmentosa

来  源:   DOI:10.3390/life13061241   PDF(Pubmed)

Abstract:
Inherited retinal diseases (IRDs) are a group of clinically and genetically heterogeneous disorders that may be complicated by several vitreoretinal conditions requiring a surgical approach. Pars plana vitrectomy (PPV) stands as a valuable treatment option in these cases, but its application in eyes with such severely impaired chorioretinal architectures remains controversial. Furthermore, the spreading of gene therapy and the increasing use of retinal prostheses will end up in a marked increase in demand for PPV surgery for IRD patients. The retinal degeneration that typically affects patients with hereditary retinal disorders may influence the execution of the surgery and the expected results. Considering the importance of PPV application in IRD-related complications, it is fundamental to try to understand from the literature what is adequate and safe in posterior eye segment surgery. Use of dyes, light toxicity, and risk of wounding scar development have always been themes that discourage the execution of vitreoretinal surgery in already impaired eyes. Therefore, this review aims to comprehensively summarize all PPV applications in different IRDs, highlighting the favorable results as well as the potential precautions to consider when performing vitreoretinal surgery in these eyes.
摘要:
遗传性视网膜疾病(IRD)是一组临床和遗传异质性疾病,可能因需要手术方法的几种玻璃体视网膜疾病而复杂化。在这些情况下,平坦部玻璃体切除术(PPV)是一种有价值的治疗选择,但是它在脉络膜视网膜结构严重受损的眼睛中的应用仍然存在争议。此外,基因治疗的推广和视网膜假体的日益使用将导致IRD患者对PPV手术的需求显著增加.通常影响遗传性视网膜疾病患者的视网膜变性可能会影响手术的执行和预期的结果。考虑到PPV在IRD相关并发症中应用的重要性,尝试从文献中了解在后眼节手术中什么是足够和安全的是至关重要的。使用染料,光毒性,伤疤发展的风险一直是阻碍在已经受损的眼睛中执行玻璃体视网膜手术的主题。因此,这篇综述旨在全面总结所有PPV在不同IRD中的应用,强调在这些眼睛中进行玻璃体视网膜手术时的有利结果以及需要考虑的潜在预防措施。
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