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Improved Intermediate Visual Function with New Monofocal Intraocular Lens in Combined Cataract and Vitrectomy Surgery for Retinal Disease. 视网膜疾病白内障和玻璃体切除术联合应用新型单焦点人工晶状体改善中间视觉功能。
Q3 Medicine Pub Date : 2023-10-01 Epub Date: 2023-08-25 DOI: 10.3341/kjo.2023.0056
Min Ji Park, Ho Seok Lee, In Boem Chang, In Hwan Hong

Purpose: To evaluate the usefulness of a newly generated monofocal intraocular lens (IOL) in patients with various retinal diseases who underwent combined cataract and pars plana vitrectomy surgery.

Methods: This prospective observational study included 33 patients with various retinal diseases. Monocular best-corrected distance visual acuity (BCDVA), uncorrected distance visual acuity (UCDVA), uncorrected intermediate visual acuity (UCIVA), uncorrected near visual acuity (UCNVA), and contrast sensitivity were measured and compared with 40 age-matched patients in the standard monofocal IOL.

Results: The Eyhance IOL group demonstrated significantly better UCIVA at 6 months follow-up compared to the standard monofocal IOL group. No significant differences were observed between the two groups in contrast sensitivity, BCDVA, UCDVA, or UCNVA. The regression analysis showed a significant association between preoperative corrected distance visual acuity and improved UCIVA in the Eyhance IOL group.

Conclusions: The Eyhance ICB00 IOL proved to be a valuable option for patients with retinal diseases undergoing combined cataract surgery and vitrectomy. It effectively improved intermediate vision without compromising contrast sensitivity or distance visual acuity.

目的:评价新型单焦点人工晶状体(IOL)在接受白内障和平坦部玻璃体切除术的各种视网膜疾病患者中的实用性。方法:这项前瞻性观察研究包括33名患有各种视网膜疾病的患者。单核最佳矫正远视力(BCDVA)、裸眼远视力(UCDVA)、裸中视力(UCIVA)、近视力(UCNVA),结果:与标准单焦点IOL组相比,Ehance IOL组在6个月的随访中表现出明显更好的UCIVA。在对比敏感度、BCDVA、UCDVA或UCNVA方面,两组之间没有观察到显著差异。回归分析显示,Ehance IOL组术前矫正远距视力与UCIVA改善之间存在显著相关性。结论:Ehance ICB00人工晶状体是白内障联合玻璃体切除术治疗视网膜疾病的一种有价值的选择。它在不影响对比敏感度或远视敏锐度的情况下有效地改善了中间视力。
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引用次数: 0
Clinical Outcomes of Descemet Membrane Endothelial Keratoplasty Using a Preloaded Imported Graft. 使用预加载的进口移植物进行后弹力膜内皮角膜移植术的临床结果。
Q3 Medicine Pub Date : 2023-10-01 Epub Date: 2023-08-10 DOI: 10.3341/kjo.2023.0053
Minha Kim, Kang Hyun Kim, Hyung Keun Lee

Purpose: Although the popularity of Descemet membrane endothelial keratoplasty (DMEK) is increased, there is still few clinical studies in Korea. In this study, we aimed to report the initial clinical outcomes of DMEK in patients followed up for more than 6 months.

Methods: A total of 96 eyes that underwent DMEK by a single surgeon for Fuchs endothelial corneal dystrophy, pseudophakic bullous keratopathy, or other indications were evaluated for best-corrected visual acuity (BCVA), endothelial cell density (ECD), central corneal thickness (CCT), postoperative complications, and graft survival.

Results: The postoperative BCVA significantly increased compared to the preoperative BCVA by 59.4% (1.00 ± 0.77 logarithm of the minimum angle of resolution vs. 0.67 ± 0.76 logarithm of the minimum angle of resolution, p < 0.001). The average preoperative ECD was 754 ± 382 cells/mm2, increasing to 1,333 ± 562 cells/mm2 at 3 months (76.8%, p < 0.001), 1,334 ± 632 cells/mm2 at 6 months (76.9%, p < 0.001), 1,121 ± 474 cells/mm2 at 12 months (48.7%, p = 0.024), and 972 ± 458 cells/mm2 at 24 months postoperatively (28.9%, p = 0.445). Compared to 3 months, the ECD declined by 15.9% at 12 months (p = 0.009) and 27.1% at 24 months postoperatively (p = 0.158). The average CCT was 675 ± 113 μm preoperatively, decreasing to 581 ± 102, 574 ± 101, and 594 ± 94 μm at 6, 12, and 24 months after DMEK, respectively (p < 0.001 between all follow-up time points). Allograft rejection was detected in three (3.1%) and 14 eyes (14.6%) underwent retransplantation at an average of 10.1 ± 8.4 months after DMEK.

Conclusions: DMEK is promising for maintaining corneal clarity, low postoperative complication rates, and stable graft longevity.

目的:尽管后弹力膜内皮角膜移植术(DMEK)越来越受欢迎,但在韩国仍很少有临床研究。在这项研究中,我们旨在报告随访超过6个月的DMEK患者的初步临床结果。方法:对96只因Fuchs内皮性角膜营养不良、人工晶状体大泡性角膜病变或其他适应症而由一名外科医生进行DMEK的眼睛进行最佳矫正视力(BCVA)、内皮细胞密度(ECD)、中央角膜厚度(CCT)、术后并发症和移植物存活率的评估。结果:术后BCVA比术前显著增加59.4%(最小分辨角的对数为1.00±0.77,最小分辨角为0.67±0.76,p<0.001)。术前平均ECD为754±382个细胞/mm2,3个月时增加到1333±562个细胞/mm 2(76.8%,p<001),术后6个月为1334±632个细胞/mm2(76.9%,p<0.001),12个月为1121±474个细胞/mm2(48.7%,p=0.024),24个月为972±458个细胞/mm2(28.9%,p=0.445)。与3个月相比,ECD在12个月下降15.9%(p=0.009),在24个月下降27.1%(p<0.158)。术前平均CCT为675±113μm,降至581±102,DMEK后6、12和24个月分别为574±101和594±94μm(所有随访时间点之间的p<0.001)。DMEK术后平均10.1±8.4个月,有3眼(3.1%)和14眼(14.6%)再次移植发现同种异体移植物排斥反应。结论:DMEK在保持角膜清晰度、降低术后并发症发生率和稳定的移植物寿命方面是有希望的。
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引用次数: 0
Proliferative Diabetic Retinopathy Requiring Vitrectomy Over Clinic Management During COVID-19: Impact of Delayed Care. COVID-19期间需要玻璃体切除术的增殖性糖尿病视网膜病变:延迟护理的影响
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2022.0147
Bennett E Ahearn, Huy Nguyen, Jeong-Hyeon Sohn

Purpose: This study aimed to investigate the impact of delayed retinal clinical care during the COVID-19 pandemic on the severity of proliferative diabetic retinopathy (PDR) and drivers of follow-up delay. We compared disease severity and follow-up rates of PDR patients requiring vitrectomy to those managed nonsurgically, and identified factors associated with need for vitrectomy.

Methods: The study included 739 patients diagnosed with PDR between January 1, 2018, and March 4, 2021, categorized into two groups based on outcome: vitrectomy nonvitrectomy. Statistical methods such as t-tests and chi-square tests were used to analyze differences in disease severity and follow-up rates before and after COVID-19 shutdown. A multivariate regression evaluated factors leading to vitrectomy by comparing initial ETDRS (Early Treatment of Diabetic Retinopathy Study) DR staging, disease stability, DR progression, proliferative complications, appointment intervals, follow-up variance, rescheduling rate, and no-show rate.

Results: Of the 739 patients, 202 required vitrectomy, 537 were managed nonsurgically. The vitrectomy group had more severe or unstable disease before shutdown. The interval between patient visits preshutdown was 29.76 ± 45.11 days in the vitrectomy group and 40.23 ± 56.73 days in the nonvitrectomy group (p < 0.001). Both groups experienced a significant increase in appointment intervals after shutdown, with the vitrectomy group having a greater increase. Both groups had significantly increased rescheduling rate and minimally increased no-show rate. Patient-directed rescheduling was the main driver of appointment delays. Disease factors, such as tractional retinal detachment and higher ETDRS DR staging, increased the odds of vitrectomy, while appointment burden and follow-up variability had a minimal impact.

Conclusions: Patients with more severe PDR and greater delay in appointments due to the pandemic were more likely to require vitrectomy for proliferative complications. Patient-directed rescheduling was identified as the main driver of care delays, as opposed to clinic-directed rescheduling. This study highlights the importance of maintaining regular follow-up appointments for PDR patients during pandemics.

目的:本研究旨在探讨COVID-19大流行期间延迟视网膜临床护理对增殖性糖尿病视网膜病变(PDR)严重程度的影响及延迟随访的驱动因素。我们比较了需要玻璃体切除术和非手术治疗的PDR患者的疾病严重程度和随访率,并确定了与玻璃体切除术需求相关的因素。方法:该研究纳入了2018年1月1日至2021年3月4日期间诊断为PDR的739例患者,根据结果分为两组:玻璃体切除术和非玻璃体切除术。采用t检验、卡方检验等统计方法分析停药前后疾病严重程度及随访率的差异。通过比较初始ETDRS(糖尿病视网膜病变早期治疗研究)DR分期、疾病稳定性、DR进展、增殖性并发症、预约时间间隔、随访方差、重新安排率和缺席率,多因素回归评估导致玻璃体切除术的因素。结果:739例患者中,202例需要玻璃体切除术,537例非手术治疗。玻璃体切割组在关闭前病情严重或不稳定。玻璃体切除术组和非玻璃体切除术组术前就诊间隔分别为29.76±45.11天和40.23±56.73天(p <0.001)。两组在关闭后的预约时间间隔都显著增加,玻璃体切割组的增加幅度更大。两组患者的重新预约率均显著增加,缺勤率则有轻微增加。以患者为导向的重新安排是预约延误的主要原因。疾病因素,如牵拉性视网膜脱离和较高的ETDRS DR分期,增加了玻璃体切除术的几率,而预约负担和随访变异性的影响最小。结论:更严重的PDR患者和由于大流行而延误预约的患者更有可能需要玻璃体切除术来治疗增生性并发症。患者导向的重新安排被确定为护理延误的主要驱动因素,而不是临床导向的重新安排。这项研究强调了在大流行期间对PDR患者保持定期随访预约的重要性。
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引用次数: 0
Incidence and Risk Factors of Recurrent Anterior Uveitis in Initial Acute-Onset Vogt-Koyanagi-Harada Disease. 急性Vogt-Koyanagi-Harada病复发性前葡萄膜炎的发生率及危险因素
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0017
Yeon Ju Lim, Iksoo Byon, Hyun Woong Kim, Sung Who Park, Han Jo Kwon, EunAh Kim

Purpose: We report the estimated incidence of, and risk factors for, recurrent anterior uveitis in patients with initial acute-onset Vogt-Koyanagi-Harada (VKH) disease using survival analyses.

Methods: Patients who were diagnosed with initial acute-onset VKH disease during 2003-2022 at two university hospitals were included. Recurrent anterior uveitis was defined as the first occurrence of the granulomatous anterior uveitis with anterior chamber cells and flare of 2+ or more by the Standardization of Uveitis Nomenclature (SUN) Working Group grading scheme, after the disappearance of conspicuous uveitis and serous retinal detachment for at least 3 months, regardless of systemic or local treatment. The univariate log-rank test and multivariate Cox regression analyses were performed, including patients' demographic characteristics, underlying diseases, presence of prodromal symptoms, duration of visual symptoms, visual acuity, slit-lamp and fundus findings, and height of serous retinal detachment. The treatment method and response to treatment were also included.

Results: The estimated incidence was 39.3% at 10 years. Fifteen of 55 patients (27.3%) had recurrent anterior uveitis during the mean follow-up of 4.5 years. The presence of focal posterior synechiae at the diagnosis increased the risk of recurrent anterior uveitis 6.97-fold compared to the absence of synechiae (95% confidence interval [CI], 2.20-22.11; p < 0.001). Use of systemic high-dose steroid therapy more than 7 days after the development of visual symptoms resulted in a hazard ratio of 4.55 (95% CI, 1.27-16.40; p = 0.020).

Conclusions: This study reports the estimated incidence and risk factors of recurrent anterior uveitis in VKH disease from survival analyses. However, because of the retrospective nature of this study, it is hard to confirm the consistency of the medical records regarding risk factors, thus, the presence of focal posterior synechiae can be inconclusive as a risk factor. Further studies are warranted.

目的:我们通过生存分析报告初始急性发作Vogt-Koyanagi-Harada (VKH)病患者复发性前葡萄膜炎的估计发生率和危险因素。方法:选取2003-2022年在两所大学医院诊断为初发急性VKH病的患者。复发性葡萄膜炎定义为:根据葡萄膜炎命名标准化工作组(SUN)分级方案,在显著性葡萄膜炎和浆液性视网膜脱离消失至少3个月后,首次出现伴有前房细胞和2+以上光斑的肉芽肿性葡萄膜前炎,无论是否进行全身或局部治疗。进行单因素log-rank检验和多因素Cox回归分析,包括患者的人口学特征、基础疾病、前症症状的存在、视觉症状的持续时间、视力、裂隙灯和眼底检查以及浆液性视网膜脱离的高度。包括治疗方法和治疗反应。结果:10年估计发病率为39.3%。55例患者中有15例(27.3%)在平均4.5年的随访期间复发前葡萄膜炎。诊断时有局灶性后粘连比无粘连增加了前葡萄膜炎复发的风险6.97倍(95%可信区间[CI], 2.20-22.11;p & lt;0.001)。在出现视觉症状后超过7天使用全体性大剂量类固醇治疗导致的风险比为4.55 (95% CI, 1.27-16.40;P = 0.020)。结论:本研究从生存分析中报告了VKH疾病复发性前葡萄膜炎的估计发生率和危险因素。然而,由于本研究是回顾性的,很难确认关于危险因素的医疗记录的一致性,因此,局灶性后粘连的存在可能是一个不确定的危险因素。进一步的研究是必要的。
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引用次数: 0
Mini-Review: Clinical Features and Management of Granular Corneal Dystrophy Type 2. 小型综述:2型颗粒性角膜营养不良的临床特征和治疗。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0032
Myung Soo Chang, Ikhyun Jun, Eung Kweon Kim

Granular corneal dystrophy type 2 (GCD2) is an autosomal dominant corneal stromal dystrophy that is caused by p.Arg124His mutation of transforming growth factor β induced (TGFBI) gene. It is characterized by well demarcated granular shaped opacities in central anterior stroma and as the disease progresses, extrusion of the deposits results in ocular pain due to corneal epithelial erosion. Also, diffuse corneal haze which appears late, causes decrease in visual acuity. The prevalence of GCD2 is high in East Asia including Korea. Homozygous patients show a severe phenotype from an early age, and the heterozygote phenotype varies among patients, depending on several types of compound heterozygous TGFBI mutations. In the initial stage, conservative treatments such as artificial tears, antibiotic eye drops, and bandage contact lenses are used to treat corneal erosion. Different surgical methods are used depending on the depth and extent of the stromal deposits. Phototherapeutic keratectomy removes anterior opacities and is advantageous in terms of its applicability and repeatability. For deeper lesions, deep anterior lamellar keratoplasty can be used as the endothelial layer is not always affected. Recurrence following these treatments are reported within a wide range of rates in different studies due to varying definition of recurrence and follow-up period. In patients who have undergone corneal laser vision-correction surgeries such as photorefractive keratectomy, LASEK, or LASIK including SMILE surgery, corneal opacity exacerbates rapidly with severe deterioration of visual acuity. Further investigations on new treatments of GCD2 are necessary.

2型颗粒状角膜营养不良(GCD2)是一种常染色体显性角膜基质营养不良,由转化生长因子β诱导(TGFBI)基因p.a g124his突变引起。其特征是中央前间质中有界限清晰的颗粒状混浊物,随着疾病进展,沉积物挤压导致角膜上皮侵蚀导致眼部疼痛。弥漫性角膜薄雾出现较晚,导致视力下降。GCD2在包括韩国在内的东亚地区的发病率较高。纯合子患者从小就表现出严重的表型,而杂合子表型在患者之间存在差异,这取决于几种类型的复合杂合子TGFBI突变。在初期,使用人工泪液、抗生素眼药水、绷带隐形眼镜等保守疗法来治疗角膜糜烂。根据基质沉积物的深度和范围,采用不同的手术方法。光疗性角膜切除术可去除前混浊,在适用性和可重复性方面具有优势。对于更深的病变,可以使用深前板层角膜移植术,因为内皮层并不总是受到影响。由于不同的复发定义和随访时间,这些治疗后的复发率在不同的研究中有很大的差异。在接受角膜激光视力矫正手术(如光屈光性角膜切除术、LASEK或LASIK包括SMILE手术)的患者中,角膜混浊迅速加剧,视力严重恶化。有必要进一步研究新的GCD2治疗方法。
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引用次数: 0
Experimental Assessment of the Performance of Dual Pneumatic Vitreous Cutters According to Gauge and Cut Rate. 基于量规和切割速率的双气动玻璃体切割机性能实验评估。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0045
Sooyeon Lee, Kyung Seek Choi

Purpose: To compare and analyze the performance of dual pneumatic ultra-high-speed vitreous cutters based on their cut rates, vacuum levels, and diameters in terms of flow rate and cutting time.

Methods: The Constellation Vision System was used to remove egg white for 30 seconds, after which we calculated the flow rate by measuring the change in weight. We then measured the time required to remove 4 mL of egg white. We tested the UltraVit (UV) 7,500 cuts per minute (cpm) probe and the Advanced UltraVit (AUV) 10,000 cpm probe in biased open duty cycle mode, using 23-, 25-, and 27-gauge probes, respectively.

Results: In biased open duty cycle, the flow rate tended to decrease as the cut rates increased for all three gauges. At the same cut rates, the flow rate increased as the vacuum level increased (p < 0.05), and as the diameter increased (p < 0.05). Among cutters with the same diameter, the AUV cutter showed higher flow rates than the UV cutter, with increases of 0.267 mL/min (18.5%) at 27 gauge, 0.627 mL/min (20.8%) at 25 gauge, and 1.000 mL/min (20.7%) at 23 gauge (all p < 0.05). The times required to remove 4 mL of egg white took longer with the UV cutter than the AUV cutter for all three gauges (all p < 0.05).

Conclusions: The use of a smaller gauge vitreous cutter may reduce the flow rate and increase the time required for vitrectomy, but this can be partially compensated for by increasing the vacuum level as well as using a vitreous cutter with a higher maximum cut rate, improved port size, and improved duty cycle.

目的:对比分析双气动超高速玻璃体切割机在切割速度、真空度、直径方面的流量和切割时间。方法:使用星座视觉系统去除蛋白30秒,然后通过测量重量变化计算流速。然后我们测量了除去4ml蛋清所需的时间。在偏置开占空比模式下,我们分别使用23号、25号和27号测量探头,测试了紫外线(UV) 7,500切割/分钟(cpm)探头和先进紫外线(AUV) 10,000 cpm探头。结果:在偏开占空比中,流速随着三种压力表切割率的增加而下降。在相同切割速率下,流量随真空度的增加而增加(p <0.05),随着直径的增大(p <0.05)。在相同直径的切割器中,AUV切割器的流量比UV切割器高,在27规格下增加了0.267 mL/min(18.5%),在25规格下增加了0.627 mL/min(20.8%),在23规格下增加了1.000 mL/min (20.7%) (p <0.05)。对于所有三种量规(均为p <0.05)。结论:使用较小尺寸的玻璃体切割器可能会降低流量并增加玻璃体切割所需的时间,但这可以通过增加真空度以及使用具有更高最大切割率,改善端口尺寸和改善占空比的玻璃体切割器来部分补偿。
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引用次数: 0
Familial Cataracts: Profile of Patients and Their Families at a Child Eye Care Tertiary Facility in a Developing Country. 家族性白内障:一个发展中国家儿童眼科保健三级机构的患者及其家庭概况。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0028
Mary Ogbenyi Ugalahi, Ezinne Obioma Onebunne, Bolutife Ayokunnu Olusanya, Aderonke Mojisola Baiyeroju

Purpose: The aim of this study is to describe the clinical profile, pedigree charting, and management of children with familial cataracts at a child eye health tertiary facility in southwest Nigeria.

Methods: The clinical records of children ≤16 years diagnosed with familial cataracts at the Pediatric Ophthalmology Clinic, University College Hospital Ibadan (Ibadan, Nigeria) from January 1, 2015, to December 31, 2019, were retrospectively reviewed. Information on demographic data, family history, visual acuity, mean refractive error (spherical equivalent), and surgical management was retrieved.

Results: The study included 38 participants with familial cataract. The mean age at presentation was 6.30 ± 3.68 years, with a range of 7 months to 13 years. Twenty-five patients (65.8%) were male. All patients had bilateral involvement. The mean duration from onset of symptoms to presentation at the hospital was 3.71 ± 3.20 years, with a range of 3 months to 13 years. In 16 of the 17 pedigree charts obtained, at least one individual was affected in each generation. The most common cataract morphology was cerulean cataract, observed in 21 eyes (27.6%). The most common ocular comorbidity was nystagmus which was observed in seven patients (18.4%). Sixty-seven eyes of 35 children underwent surgery within the period of the study. The proportion of eyes that had best-corrected visual acuity ≥6 / 18 before surgery was 9.1%; this proportion had increased to 52.7% at the last postoperative visit.

Conclusions: Autosomal dominant inheritance appears to be the major pattern among our patients with familial cataract. The most common morphological type found in this cohort was cerulean cataract. Genetic testing and counseling services are vital for the management of families with childhood cataract.

目的:本研究的目的是描述临床概况,谱系图和管理的儿童家族性白内障在儿童眼科保健三级机构在尼日利亚西南部。方法:回顾性分析2015年1月1日至2019年12月31日在尼日利亚伊巴丹市伊巴丹大学附属医院儿童眼科门诊诊断为家族性白内障的16岁以下儿童的临床资料。检索了人口统计资料、家族史、视力、平均屈光不正(球面等效)和手术处理等信息。结果:本研究纳入38例家族性白内障患者。平均发病年龄6.30±3.68岁,年龄范围7个月~ 13岁。男性25例(65.8%)。所有患者均有双侧受累。从出现症状到到医院就诊的平均时间为3.71±3.20年,范围为3个月至13年。在获得的17个谱系图中,有16个每代至少有一个个体受到影响。最常见的白内障形态为蓝色白内障,共21眼(27.6%)。最常见的眼部合并症是眼球震颤,7例(18.4%)。在研究期间,35名儿童的67只眼睛接受了手术。术前最佳矫正视力≥6 / 18眼的比例为9.1%;这一比例在最后一次术后随访时增加到52.7%。结论:常染色体显性遗传是家族性白内障患者的主要遗传模式。该队列中最常见的形态类型为蓝色白内障。基因检测和咨询服务对于儿童期白内障家庭的管理至关重要。
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引用次数: 0
Neuroprotective Strategies for Nonarteritic Anterior Ischemic Optic Neuropathy: A Systematic Review. 非动脉性前缺血性视神经病变的神经保护策略:系统综述。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2022.0166
Brigitta Marcia Budihardja, Erika Anggraini, Rianti Wulandari Pratiwi, Anya Dewi Nastiti, Syntia Nusanti

Purpose: Nonarteritic anterior ischemic optic neuropathy (NAION) is the second most common form of optic neuropathy. Most patients show no improvement over time. Until now, there is still no definitive therapy for NAION. The available literatures on the possible treatment of NAION are quite diverse and controversial. Neuroprotection strategies have been suggested as one of the potential treatments for NAION. This review aims to critically evaluate the literature on neuroprotective strategy for NAION.

Methods: This report was written in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines. We performed a systematic literature search in Pubmed, Science Direct, Proquest, and Cochrane databases. Only neuroprotective agents that directly work in protecting neurons were included. The outcome of interest in this review is retinal ganglion cell density and apoptosis for animal studies and retinal nerve fiber layer thickness for human studies.

Results: The systematic search identified 591 studies of which 24 met the eligibility criteria, including 21 animal studies and three human studies. Only a few of the studies evaluated the same treatments, showing how diverse neuroprotector treatments are currently being evaluated as NAION treatment. From 21 animal studies, 14 studies showed significantly higher retinal ganglion cell density (1.49- to 2.81-fold) with neuroprotective treatment compared to control group. Two of three human studies in this review had also found a beneficial effect of preserving retinal nerve fiber layer thickness in NAION patients.

Conclusions: This review suggests the potential of neuroprotection as a viable option in the quest for an effective treatment strategy for NAION. Further studies, particularly clinical studies, are necessary to establish its efficacy in NAION patients.

目的:非动脉性前部缺血性视神经病变(NAION)是第二常见的视神经病变。随着时间的推移,大多数患者没有任何改善。到目前为止,对于NAION仍然没有明确的治疗方法。现有的文献对NAION可能的治疗方法是相当多样化和有争议的。神经保护策略已被认为是NAION的潜在治疗方法之一。这篇综述旨在批判性地评价关于NAION神经保护策略的文献。方法:本报告按照PRISMA(系统评价和荟萃分析首选报告项目)指南撰写。我们在Pubmed、Science Direct、Proquest和Cochrane数据库中进行了系统的文献检索。只包括直接保护神经元的神经保护剂。本综述主要关注动物实验中视网膜神经节细胞密度和凋亡的变化,以及人实验中视网膜神经纤维层厚度的变化。结果:系统检索确定了591项研究,其中24项符合入选标准,包括21项动物研究和3项人体研究。只有少数研究评估了相同的治疗方法,这表明目前有多种神经保护剂治疗被评估为NAION治疗。在21项动物研究中,14项研究显示,与对照组相比,神经保护治疗组的视网膜神经节细胞密度显著提高(1.49至2.81倍)。在本综述中,三分之二的人体研究也发现了保留NAION患者视网膜神经纤维层厚度的有益效果。结论:本综述提示神经保护的潜力是寻求NAION有效治疗策略的可行选择。需要进一步的研究,特别是临床研究来确定其对NAION患者的疗效。
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引用次数: 1
Atypical Spindle Cell/Pleomorphic Lipomatous Tumor of the Orbit: A Case Report. 眼眶非典型梭形细胞/多形性脂肪瘤1例。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0015
Sung Hyun Ahn, Kyoung Min Kim, Nam Chun Cho, Min Ahn
Dear Editor, Atypical spindle cell/pleomorphic lipomatous tumor (ASC/PLT) is a benign adipocytic neoplasm newly classified in the fifth edition of the WHO Classification of Tumors: Soft Tissue and Bone, published in 2020 [1]. Here, we report a case of ASC/PLT, which was diagnosed histopathologically, involving the orbit which is uncommon. A written informed consent for the publication of research details and clinical images was obtained from the patient. A 57-year-old man presented with painless and progressive proptosis of the left eye that had developed 6 months earlier. The patient also complained of intermittent diplopia. He had no ocular disease or medical history. On initial evaluation, he had a best-corrected visual acuity of 25 / 20 in the right eye and 20 / 20 in the left eye, full color vision in both eyes, and no relative afferent pupillary defects. Mild lid swelling and conjunctival chemosis were present in the left eye, with no tenderness or palpable mass (Fig. 1A). Hertel exophthalmometry showed a 16-mm left eye protrusion compared to 14 mm on the right. There were –2-mm limitations to supraduction and abduction in the left eye. Fundoscopic examination results were unremarkable. Thyroid function test results were within the normal range. Computed tomography (CT) of the orbits revealed thickening of the inferior rectus (IR), medial rectus (MR), and lateral rectus (LR) muscles of the left eye and enhancement of soft tissue in the orbit, compatible with nonspecific orbital inflammation, also known as orbital inflammatory pseudotumor and idiopathic orbital inflammation (Fig. 1B). The patient was managed with oral prednisolone (60 mg/ day) for 3 weeks, followed by tapering for 4 weeks, but it was not effective. Orbital MRI showed severe enlargement of the IR and mild thickening of the MR and LR with hyperintensity on T2-weighted imaging, revealing extension of the infiltrating lesion into the preseptal space of the lower eyelid, which was suspected to be a lymphoma (Fig. 1C). Three biopsies were performed in other facilities over 6 months on the demand of the patient, and a lipomatous tumor was only diagnosed on the third result. The patient returned to our hospital and was followed up for over 2 years. During this period, the patient maintained relatively good vision but complained of slightly worsening diplopia and limitation of supraduction of the left eye. Repeat CT demonstrated progression of the disease, and the decision was made to proceed with orbitotomy for the fourth biopsy to make an accurate diagnosis (Fig. 1D). All biopsies were performed from the mass infiltrating orbit (Fig. 1C). The pathology of the specimen showed that mature adipose cells were present between skeletal muscle fibers. The tumor was primarily composed of spindle cells, with some cells showing atypia. The immunohistochemical stains of MDM2 and CDK4 showed negative. A diagnosis of an Received: February 8, 2023 Final revision: June 26, 2023 Accepted: July 2,
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引用次数: 0
Clinical and Genetic Findings in Korean Patients with Choroideremia. 韩国脉络膜血症患者的临床和遗传学研究。
Q3 Medicine Pub Date : 2023-08-01 DOI: 10.3341/kjo.2023.0020
Woo Gyeong Jo, Christopher Seungkyu Lee, Jinu Han

Purpose: We share and analyze the clinical presentations and genotypes of Korean male patients and female carriers who visited our clinic.

Methods: Six male patients and three female carriers with comprehensive ophthalmic examinations and next-generation sequencing were included. Detailed clinical features were identified using visual field (VF) test and multimodal imaging including color fundus photography, fundus autofluorescence (FAF), and optical coherence tomography (OCT).

Results: Six male patients were diagnosed with choroideremia at the median age of 25 years. Before genetic testing, three patients (50.0%) were clinically diagnosed with choroideremia, while the other three patients (50.0%) with retinitis pigmentosa. Patients showed different types of hemizygous CHM variants, including two nonsense variants, c.715C>T:p.(Arg239*) and c.799C>T:p.(Arg267*); two frameshift variants, c.1584_1587del:p.(Val529Hisfs*7) and c.403_404del:p.(Asp135Phefs*9); one splicing variant c.1511-28_1511-2del; and one exon 2-8 duplication. The latter three variants were novel. Two female carriers had heterozygous exon 2-8 duplication and the other one female carrier had heterozygous nonsense variant c.715C>T:p. (Arg239*). Fundus showed diffuse yellow-whitish scleral reflex and granular pigmented lesions. FAF showed multiple patchy hypofluorescence lesions, sparing macula. OCT showed thinning of outer nuclear layer, ellipsoid zone, retinal pigment epithelium layer, choroid thickness, interlaminar bridges, outer retinal tubulations, and microcysts in the inner nuclear layer. VF showed ring scotoma pattern with small amount of remaining central field. Asymptomatic female carriers showed variable fundus findings and mild changes in OCT.

Conclusions: A detailed description of the genotypes with three novel mutations and phenotypes of six choroideremia patients and three CHM mutation female carriers are presented.

目的:我们分享和分析来我们诊所就诊的韩国男性患者和女性携带者的临床表现和基因型。方法:男性6例,女性3例,经眼科综合检查和下一代测序。通过视野(VF)测试和多模态成像(包括彩色眼底摄影、眼底自身荧光(FAF)和光学相干断层扫描(OCT))确定详细的临床特征。结果:6例男性患者被诊断为脉络膜血症,中位年龄25岁。基因检测前,临床诊断为脉络膜血症3例(50.0%),色素性视网膜炎3例(50.0%)。患者出现不同类型的半合子CHM变异,包括2个无义变异,c.715C>T:p.(Arg239*)和c.799C>T:p.(Arg267*);两个移码变体,c.1584_1587del:p.(Val529Hisfs*7)和c.403_404del:p.(Asp135Phefs*9);一个剪接变体c.1511-28_1511-2del;还有一个2-8外显子重复。后三种变体是新颖的。2名女性携带者存在杂合性外显子2-8重复,另1名女性携带者存在杂合性无义变异c.715C>T:p。(Arg239 *)。眼底可见弥漫性黄白色巩膜反射及颗粒状色素病变。FAF显示多发斑片状低荧光病变,保留黄斑。OCT示外核层变薄,椭球带变薄,视网膜色素上皮层变薄,脉络膜增厚,层间桥变薄,视网膜外管变细,内核层微囊变细。VF呈环形暗斑,中心野少量残留。结论:本文详细描述了6例脉络膜血症患者和3例CHM突变女性携带者的3种新突变和表型基因型。
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引用次数: 0
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Korean Journal of Ophthalmology : KJO
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