primary congenital glaucoma

原发性先天性青光眼
  • 文章类型: Journal Article
    背景:这项回顾性研究旨在评估房角镜辅助经腔小梁切开术(GATT)在中国原发性先天性青光眼(PCG)患者中的疗效和安全性,并确定影响手术成功的因素。
    方法:招募了14例诊断为PCG的患者(24只眼),这些患者接受了房角镜辅助的经腔小梁切开术,和眼内压(IOP)的数据,抗青光眼药物,手术相关并发症,在术前和术后访视期间收集其他治疗方法。手术成功率定义为IOP≤21mmHg且比基线降低>30%。有(部分成功)或没有(完全成功)抗青光眼药物。
    结果:术前平均IOP为30.41±6.09mmHg。在最后一次访问中,平均眼压下降16.1±9.1mmHg(52%),24只眼中有19只眼不使用局部药物。与基线相比,每次术后随访的IOP均显着降低(所有时间点P<0.05)。完全和部分成功的累积比例分别为79.2%和95.8%,分别,手术后三年.之前没有抗青光眼手术的患者,没有术后IOP峰值,接受完全小梁切开术的患者手术预后改善.到各自的随访结束时,24只眼均未发生永久性视力威胁并发症。
    结论:房角镜辅助经腔小梁切开术是一种安全有效的PCG治疗方法,具有显著的眼压降低效果和较高的手术成功率。
    BACKGROUND: This retrospective study aimed to evaluate the efficacy and safety of gonioscopy-assisted transluminal trabeculotomy (GATT) in Chinese patients with primary congenital glaucoma (PCG) and identify factors influencing surgical success.
    METHODS: Fourteen patients (24 eyes) diagnosed with PCG who underwent gonioscopy-assisted transluminal trabeculotomy were recruited, and data on intraocular pressure (IOP), antiglaucoma medication, surgery-related complications, and additional treatments were collected during preoperative and postoperative visits. Surgical success was defined as IOP ≤ 21 mmHg and a reduction of > 30% from baseline, with (partial success) or without (complete success) antiglaucoma medication.
    RESULTS: Mean preoperative IOP was 30.41 ± 6.09 mmHg. At the final visit, mean IOP reduction was 16.1 ± 9.1 mmHg (52%), and 19 of 24 eyes were topical medication-free. IOP was significantly decreased at each postoperative visit compared with baseline (P < 0.05 for all time points). Cumulative proportions of complete and partial success were 79.2% and 95.8%, respectively, at three years postsurgery. Patients without prior antiglaucoma procedures, without postoperative IOP spikes, and those undergoing complete trabeculotomy exhibited improved surgical prognosis. No permanent vision-threatening complications occurred in the 24 eyes by the end of the respective follow-ups.
    CONCLUSIONS: Gonioscopy-assisted transluminal trabeculotomy emerged as a safe and effective procedure for PCG treatment, characterized by outstanding IOP reduction efficacy and high surgical success rates.
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  • 文章类型: Journal Article
    背景:儿童青光眼是全球儿童失明的主要原因。在撒哈拉以南非洲,儿童青光眼的特征尚未得到很好的表征。因此,这项研究旨在描述人口统计学,临床特征,儿童青光眼的管理,以及从基线到最终就诊的视力(VA)和眼内压(IOP)的改善。
    方法:这项回顾性研究包括2019年9月至2022年8月诊断的18岁以下青光眼患者。根据儿童青光眼研究网络分类(CGRN)进行儿童青光眼诊断和分类。
    结果:共有105名儿童(181只眼)被诊断为青光眼。儿童青光眼最常见的类型是原发性先天性青光眼(PCG)构成(42%,n=76只眼睛,95%置信区间(CI),34.7-49.5%;P=0.037),其次是青光眼嫌疑人(22.1%,n=40只眼睛,95%CI,16.3-28.9%;P<0.001)和青少年开角型青光眼(JOAG)(15.5%,n=28只眼睛,95%CI,10.5-21.6%;P<0.001)。虽然继发性青光眼最常见的类型是类固醇诱导的青光眼,其次是白内障手术后的青光眼。在72.4%的儿童中发现了双侧青光眼(n=76,儿童的95%CI,62.8-80.7%;P<0.001)。在原发性和继发性青光眼中,男孩比女孩受影响更大,比例分别为2:1和2.7:1。PCG患者的平均年龄为2.7岁。接近93.4%(71)的PCG眼通过手术治疗,其中大多数接受了联合小梁切开术和小梁切除术(CTT)。大多数继发性青光眼病例均采用药物治疗。总的来说,85.3%(111)眼成功控制IOP≤21mmHg。
    结论:PCG是儿童青光眼最常见的类型。发展中国家公认的挑战之一,PCG患者的晚期表现,在我们的研究中也观察到了。其中突出显示,需要增加获得眼部护理服务的机会,并将儿童青光眼作为主要的公共卫生问题。类固醇性青光眼是继发性青光眼最常见的类型;应采取适当措施预防这种可预防的青光眼。
    BACKGROUND: Childhood glaucoma is a major cause of childhood blindness worldwide. The profile of childhood glaucoma has not been well characterized in sub-Saharan Africa. Thus, this study was designed to describe demographics, clinical features, managements of childhood glaucoma, and improvements in visual acuity (VA) and intraocular pressure (IOP) from baseline to final visit.
    METHODS: This retrospective study included glaucoma patients below 18 years old who were diagnosed between September 2019 to August 2022. Childhood glaucoma diagnosis and classification was made as per the Childhood Glaucoma Research Network Classification (CGRN).
    RESULTS: A total of 105 children (181 eyes) were diagnosed with glaucoma. The most common type of childhood glaucoma was primary congenital glaucoma (PCG) constituting (42%, n = 76 eyes, 95% confidence interval (CI), 34.7-49.5%; P = 0.037), followed by glaucoma suspect (22.1%, n = 40 eyes, 95% CI, 16.3-28.9%; P < 0.001) and juvenile open-angle glaucoma (JOAG) (15.5%, n = 28 eyes, 95% CI, 10.5-21.6%; P < 0.001). While the most common type of secondary glaucoma was steroid-induced glaucoma, followed by glaucoma following cataract surgery. Bilateral glaucoma was found in 72.4% (n = 76 children, 95% CI, 62.8-80.7%; P < 0.001) of children. In both primary and secondary glaucoma, boys were affected more than girls, in ratio of 2:1 and 2.7:1, respectively. The mean age at presentation for patients with PCG was 2.7 years. Close to 93.4% (71) of PCG eyes were managed surgically, of which majority underwent combined trabeculotomy and trabeculectomy (CTT). Most of secondary glaucoma cases were treated medically. Overall, 85.3% (111) of eyes had successful control of IOP ≤ 21 mmHg.
    CONCLUSIONS: PCG was the most common type of childhood glaucoma. One of a well-recognized challenge in developing countries, late presentation of patients with PCG, was also observed in our study. Which highlights, the need of increasing access to eye-care service and awareness of childhood glaucoma as a major public health issue. Steroid-induced glaucoma was the most common type of secondary glaucoma; appropriate measures should be taken to prevent this preventable glaucoma.
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  • 文章类型: Journal Article
    目的:根据视敏度(VA)评估原发性先天性青光眼(PCG)患者的长期功能和结构结局,使用标准自动视野检查的视野(VF),和乳头周围视网膜神经纤维层厚度(pRNFL)。
    方法:我们回顾性回顾了1977年至2016年在丹麦诊断为PCG的所有患者的医疗记录。严重视力丧失定义为VA<6/60和/或VF>20分贝(dB)。在相关矩阵中评估预后因素。
    结果:94例患者(153只眼)的中位年龄为12岁(IQR9-16)。在PCG眼中,62%的患者VA≥6/18,但22%<6/60。视力较好的患者的VA≥6/18占90%,<6/60占5%。在59只PCG眼中测量了VF,中位数平均缺陷为5.1dB(IQR2.1-9.6),比6dB好52%,比20dB差9%。在测量pRNFL的58只PCG眼中,29%的广义pRNFL降低到年龄预期的第1百分位数以下。可怜的VA,VF差和pRNFL降低均相关(p=0.0001)。更多的手术(p<0.0001)和更长的诊断延迟(p=0.004)与较差的视力相关,而在较小程度上与较差的VFpRNFL相关。
    结论:在丹麦,大多数双侧PCG患者在视力较好的眼中保留VA≥6/18.不良的VA与不良的VF相关。较长的诊断延迟和更多的手术与较差的预后相关。
    OBJECTIVE: Evaluation of long-term functional and structural outcomes in patients with primary congenital glaucoma (PCG) based on visual acuity (VA), visual field (VF) using standard automated perimetry, and peripapillary retinal nerve fibre layer thickness (pRNFL).
    METHODS: We retrospectively reviewed medical records of all patients diagnosed with PCG in Denmark from 1977 to 2016. Severe vision loss was defined as VA <6/60 and/or VF >20 decibels (dB). Prognostic factors were evaluated in a correlation matrix.
    RESULTS: The median age of the 94 patients (153 PCG eyes) was 12 years (IQR 9-16). In PCG eyes 62% had VA ≥6/18 but 22% had <6/60. VA in the better seeing eye was ≥6/18 in 90% and <6/60 in 5%. VF was measured in 59 PCG eyes and the median mean defect was 5.1 dB (IQR 2.1-9.6) with 52% better than 6 dB and 9% worse than 20 dB. Generalized pRNFL was reduced below the age-expected 1st percentile in 29% of the 58 PCG eyes where pRNFL was measured. Poor VA, poor VF and reduced pRNFL were all correlated (p = 0.0001). More surgeries (p < 0.0001) and longer diagnostic delay (p = 0.004) were associated with poorer vision and to a lesser degree with poor VF pRNFL.
    CONCLUSIONS: In Denmark, most patients with bilateral PCG retain VA ≥6/18 in the better seeing eye. Poor VA was associated with poor VF. Longer diagnostic delay and more surgeries were associated with a poorer prognosis.
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  • 文章类型: Journal Article
    目的:评估完成至少20年随访的原发性先天性青光眼(PCG)患者的长期手术和视觉结果。
    方法:回顾性临床数据库研究。
    方法:在1991年1月至2000年12月期间接受PCG手术的121例患者的220只眼,从2021年1月至2022年1月进行随访。
    方法:回顾性回顾初次接受小梁切开-小梁切除术(CTT)而不使用丝裂霉素C作为初始手术的患者的病历。成功定义为当IOP≥6mmHg和≤21mmHg时,不使用青光眼药物,而当需要两种青光眼药物时,成功定义为完全。需要重新手术以控制IOP的眼睛被认为是故障。使用最大似然估计的混合效应模型用于基于眼睛的变量的估计,并在不同访问之间进行比较。Kaplan-Meier生存分析用于评估手术和功能成功的概率。使用夹心聚类估计的Cox比例风险回归用于评估失败和不良视觉结果的风险因素。
    方法:主要结果指标是在20年的随访中显示完全成功的患者比例。次要结果指标包括手术失败率和青光眼需要再次手术,视敏度,屈光不正,不良预后和并发症的危险因素。
    结果:Kaplan-Meier生存分析显示1年,10年,20年完成成功率90.7%,78.9%和44.5%,分别。在单变量分析中,在进行任何额外眼内手术的患者中,手术失败率较高.然而,在多变量分析中,没有一项临床参数与失败显著相关.总的来说,眼睛的比例好,正常和不良的视觉结果为33.2%,16.4%和50.4%,分别。近视占68.9%。接受原发性CTT的患者中有28只眼(14.4%)需要进行第二次手术以控制IOP。术中无明显并发症。由于痛苦的失明,六只眼睛需要摘除。
    结论:CTT在这一大型PCG患者队列中是一个有用的程序。它提供了良好的IOP控制和适度的视觉恢复,在手术后20年的随访中仍然存在。
    OBJECTIVE: To estimate the long-term surgical and visual outcomes in patients with primary congenital glaucoma (PCG) who completed at least 20 years of follow-up.
    METHODS: Retrospective study.
    METHODS: Two hundred twenty eyes of 121 patients undergoing surgery for PCG between January 1991 and December 2000 and who returned for a follow-up visit from January 2021 through January 2022.
    METHODS: Retrospective review of medical records of patients who underwent primary combined trabeculotomy-trabeculectomy (CTT) without mitomycin C as an initial procedure. Success was defined as complete when intraocular pressure (IOP) was ≥ 6 mmHg and ≤ 21 mmHg without glaucoma medication and as qualified when up to 2 glaucoma medications were required. Failure was defined as uncontrolled IOP with more than 2 glaucoma medications, need for a second IOP-lowering procedure, chronic hypotony (IOP < 6 mmHg on 2 consecutive visits), or any sight-threatening complication. A mixed-effects model using maximum likelihood estimation was used in estimation of eye-based variables and to make comparisons between different visits. Kaplan-Meier survival analysis was used to estimate the probabilities of surgical and functional successes. Cox proportional hazards regression using sandwich clustered estimation was used to evaluate risk factors for failure and poor visual outcome.
    METHODS: Primary outcome measure was the proportion of patients who demonstrated complete success over the 20-year follow-up. Secondary outcome measures included rate of surgical failure and need for reoperation for glaucoma, visual acuity, refractive errors, risk factors for poor outcome, and complications.
    RESULTS: Kaplan-Meier survival analysis revealed 1-year, 10-year, and 20-year complete success rates of 90.7%, 78.9%, and 44.5%, respectively. In univariate analysis, surgical failure was higher among patients with any additional non-glaucoma intraocular surgery. None of the clinical parameters were associated significantly with failure in multivariable analysis. Overall, the proportion of eyes with good, fair, and poor visual outcomes was 33.2%, 16.4%, and 50.4%, respectively. Myopia was seen in 68.9% eyes. Twenty-eight eyes of those who underwent primary CTT (14.4%) required second surgery for IOP control. No significant intraoperative complications occurred. Six eyes required enucleation because of painful blind eye.
    CONCLUSIONS: In this large cohort of patients with PCG, CTT is a useful procedure. It provides good IOP control and moderate visual recovery that remained over a 20-year follow-up after surgery.
    BACKGROUND: The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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  • 文章类型: Journal Article
    评估手术控制的原发性先天性青光眼(PCG)眼的VEP参数,并将其与正常年龄相似的儿童眼进行比较。
    一项横断面比较研究,对26名(19名男性)接受PCG手术的儿童的34只(19只右)眼和22名(12名男性)年龄相似的对照儿童的30只(17只右)眼进行了一项基于大学的实践。所有研究参与者都接受了标准的检查和电生理测试方案(单次闪光VEP反应,光线适应,瞳孔未扩张),报告P2隐式时间,N1-P1和N2-P2(振幅)。
    研究儿童和对照组年龄的平均值±SD分别为43.22±33.2和55.68±35.2个月(p=0.217)。在演示和测试时,研究儿童的平均±SDIOP和杯/盘比率分别为18.4±5.2和5.2±3.3mmHg和0.7±0.2和0.3±0.3,分别。在PCG手术21.5±21.3个月后进行VEP测试。患者和对照组之间的P2,N1-P1和N2-P2没有统计学上的显着差异(分别为p=0.941,0.916,0.945)。研究眼睛的大多数临床特征与任何研究的VEP参数之间没有统计学上的显着相关性。
    操作控制的PCG眼睛的VEP参数与其正常的同伴孩子相匹配。
    UNASSIGNED: To evaluate the VEP parameters in operated controlled primary congenital glaucoma (PCG) eyes and compare them to normal age similar children eyes.
    UNASSIGNED: A cross-sectional comparative study conducted on 34 (19 right) eyes of 26 (19 males) children operated for PCG and 30 (17 right) eyes of 22 (12 males) age similar control children in a university-based practice. All study participants were subjected to a standard protocol of examination and electrophysiological testing (single flash VEP response, light adapted, pupils not dilated), reporting on the P2 implicit time, N1-P1 and N2-P2 (amplitude).
    UNASSIGNED: The mean±SD of the age of the study children and controls was 43.22±33.2 and 55.68±35.2 months respectively (p=0.217). The mean±SD IOP and cup/disc ratio of the study children were 18.4±5.2 and 5.2±3.3mmHg and 0.7±0.2 and 0.3±0.3 at presentation and at testing, respectively. The VEP testing was conducted after 21.5±21.3 months of surgery for PCG. There was no statistically significant differences in P2, N1-P1 and N2-P2 between patients and controls (p=0.941, 0.916,0.945, respectively). There was no statistically significant correlation between most of the clinical characteristics of the study eyes and any of the studied VEP parameters.
    UNASSIGNED: Operated controlled PCG eyes have VEP parameters that match their normal fellow children.
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  • 文章类型: Journal Article
    OBJECTIVE: To report the flash visual evoked potential (VEP) findings in children with primary congenital glaucoma (PCG) before and after successful surgical control of the intraocular pressure (IOP).
    METHODS: The study enrolled children presenting with PCG to the Ophthalmology Department of Alexandria Main University Hospital in the period between June and December 2019. All study participants were subjected to a standard protocol of examination, to confirm the diagnosis of PCG. The child was then referred for flash VEP testing. Peaks were designated as negative and positive waves in a numerical sequence (N1, P1, N2, P2, N3 and P3). The child was then scheduled for surgery within 1 week of presentation. Postoperative VEP testing was scheduled as soon as feasible and was conducted in the same way as the preoperative VEP testing.
    RESULTS: The study was conducted on 11 (four right) eyes of eight (five males) children. The mean ± standard deviation of the age of the study children at presentation and at postoperative VEP testing was 3.5 ± 1.6 and 5.1 ± 3.0 months, respectively. The study eyes mean ± standard deviation IOP at presentation and at postoperative VEP testing was 23.0 ± 6.7 and 8.3 ± 2.4 mmHg, respectively (p < 0.0001). There were a statistically insignificant reduction in P2 implicit time (p = 0.235) and a statistically insignificant increase in each of N1-P1 (p = 0.15) and N2-P2 (p = 0.67) amplitudes postoperatively than preoperatively.
    CONCLUSIONS: IOP elevation in PCG adversely affects the optic nerve function, but, at least in the short term, has no permanent detrimental effect on the optic nerve function as evidenced by the improvement in the VEP parameters.
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  • 文章类型: Journal Article
    OBJECTIVE: To evaluate the surgical success after trabeculotomy for primary congenital glaucoma (PCG) in Denmark in the last 40 years.
    METHODS: Retrospective review of medical files on children who all underwent trabeculotomy as the first surgical intervention between January 1rst, 1977 and December 31, 2016. Information on diagnosis and surgical procedures were extracted. Primary outcome was surgical success was defined as intraocular pressure (IOP) < 21 mmHg without medications one year after surgery. Secondary outcome was the number of reoperations needed.
    RESULTS: Out of 118 children with PCG, 96 children (144 eyes) had an ab externo trabeculotomy as primary IOP lowering procedure opening Schlemm\'s channel 4 clock hours into the anterior chamber. Complete surgical success at one year was achieved in 73% (106 of 144 eyes). On the long term, IOP could be controlled by one surgical procedure in 2/3 of children, eight eyes required >5 surgical procedures to control IOP. In 12.1% of children the second glaucoma procedure was performed >5 years after the initial trabeculotomy. There was a tendency towards greater risk of needing a second procedure in patients < 3 months of age at primary trabeculotomy (hazard ratio, HR, 2.01, 95% CI 0.96-4.22) and in boys (HR 2.02, 95% CI 0.97-4.18) and a lower risk of requiring a third surgery if the second surgery was trabeculectomy with MMC.
    CONCLUSIONS: Dedicated follow-up of patients with PCG is essential as the disease may continue to progress even after years of quiescence and some children need multiple surgeries to control IOP. Additional glaucoma procedures are required in 1/3 of children and boys and younger patients seem to be at greater risk.
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  • 文章类型: Journal Article
    OBJECTIVE: To assess the long-term results of viscotrabeculotomy in infants with primary congenital glaucoma and to compare its outcome with conventional trabeculotomy.
    METHODS: A prospective randomized comparative study included infants with primary congenital glaucoma younger than 2 years. Patients were divided into two groups: viscotrabeculotomy group and conventional trabeculotomy group. Preoperative and postoperative intraocular pressures, corneal diameter, intraoperative and postoperative complications, and success rates were compared between two groups. All the patients were followed up for 5 years.
    RESULTS: The study included 154 eyes of 92 infants distributed randomly among the two groups; 78 and 76 eyes in viscotrabeculotomy and conventional trabeculotomy groups, respectively. In both groups, there was a statistically significant intraocular pressure reduction at all time points of the follow-up periods compared to the preoperative values (p < 0.0001). At 5 years, viscotrabeculotomy group showed significant reduction of the mean postoperative intraocular pressure (49.47%) than conventional trabeculotomy group (48.64%) (p < 0.0001). Intraocular pressure was statistically lower in viscotrabeculotomy starting from 12th month and till the end of the follow-up. At 5 years, the total success rate of viscotrabeculotomy group was 89.74% compared to 85.53% in conventional trabeculotomy group without significant difference (p = 0.487). The postoperative mean values of the cup/disk ratio in viscotrabeculotomy group showed statistically lower values compared to conventional trabeculotomy group (p = 0.019). Postoperative hyphema was statistically higher in conventional trabeculotomy group (p < 0.0001). All eyes that underwent a reoperation before 5 years follow-up were excluded from the statistical workup of the study after reoperation, except for calculation of success/failure.
    CONCLUSIONS: Viscotrabeculotomy and conventional trabeculotomy proved to be effective in cases of primary congenital glaucoma. Viscotrabeculotomy appeared to have prolonged stability in controlling the intraocular pressure with higher success rates and lower complications.
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  • 文章类型: Journal Article
    Primary congenital glaucoma (PCG) and anterior segment defects (ASDs) are rare ocular malformations diagnosed early in life which can cause blindness. Pathogenic variants in several genes have been linked to these conditions, but little is known about nongenetic risk factors. We investigated the association between maternal nutrition and PCG and ASDs in the National Birth Defects Prevention Study, a large population-based, multicenter case-control study of major birth defects in the United States. Mothers of cases (n = 152) and control infants without a birth defect (n = 9,178) completed an interview which included a food frequency questionnaire capturing usual dietary intake in the year before pregnancy. Maternal nutrition was assessed through individual nutrient intake, calculating a Diet Quality Index for Pregnancy (DQI-P) score for each mother, and using latent class analysis to empirically derive four dietary patterns. We calculated adjusted odds ratios (aORs) and 95% confidence intervals (CI) using logistic regression. The results for individual nutrients varied, with some having an inverse or U-shaped pattern of association with increasing intake. The DQI-P was not associated with risk of PCG and ASDs (aOR 0.91; CI 0.49-1.66, highest vs. lowest quartile). The dietary pattern analysis suggested lower odds among women with a Prudent and Mexican dietary pattern (aOR 0.82, 95% CI 0.52-1.29; aOR 0.80, 95% CI 0.36-1.78, respectively) compared to those with a Western dietary pattern. We found that higher intake of some nutrients and certain dietary patterns may be inversely associated with PCG and ASDs, though caution is urged due to imprecision of estimates.
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  • 文章类型: Clinical Trial Protocol
    Trabeculotomy and combined trabeculotomy-trabeculectomy (CTT) are major surgical options for primary congenital glaucoma (PCG). However, it is unclear which of these two surgical procedures should be recommended as the optimum first-line treatment for PCG. This trial aims to determine whether the outcomes of trabeculotomy are non-inferior to those of CTT in moderate PCG with a horizontal corneal diameter (HCD) of 12-14 mm.
    This is a 3-year, non-inferiority, prospective, randomised controlled trial. We plan to recruite 248 participants (aged ≤3 years) with PCG with an HCD of 12-14 mm from the Department of Glaucoma, Zhongshan Ophthalmic Center, Guangzhou, China. One eye per participant will be randomly (1:1) assigned to receive trabeculotomy or CTT. The primary outcome is the 3-year postoperative success rate in lowering intraocular pressure (IOP), and the secondary clinical outcomes will include IOP reduction, visual acuity, HCD, central corneal thickness, axial length, cup-disc ratio, refractive error and postoperative complications. Data will be analysed by the intention-to-treat principle.
    The study protocol has been approved by the ethics committee of Zhongshan Ophthalmic Center (2014MEKY023) and the \'5010 Plan\' evaluation committee at Sun Yat-Sen University, Guangzhou, China. The results will be disseminated in international academic meetings and published in peer-reviewed journals.
    Chinese Clinical Trial Registry, ChiCTR-IOR-14005588; Date registered: 20 November 2014.
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