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ABSTRACT Pigment dispersion syndrome is associated with clinical features such as Krukenberg's spindles, trabecular pigmentation, Scheie's stripe and Zentmayer's ring. Another less common feature of this syndrome is retrolental pigment deposits due to anterior hyaloid detachment or a defect in the Wieger's ligament. We present two cases of pigment deposits on the posterior lens capsule. In both cases, there is bilateral dispersion of pigment throughout the anterior segment. The retrolental deposits are unilateral in the first case and bilateral in the second. Both patients report a history of ocular trauma. This is a possible important clinical sign of pigment dispersion syndrome, rarely described.

RESUMO A síndrome de dispersão pigmentar associa-se a sinais clínicos característicos como fuso de Krukenberg, hiperpigmentação da malha trabecular, linha de Scheie e anel de Zentmeyer. Um sinal menos comum dessa síndrome é o depósito de pigmento posterior ao cristalino, que ocorre por um descolamento da hialoide anterior ou um defeito no ligamento de Wieger. Apresentamos dois casos de depósitos de pigmento posterior à cápsula posterior do cristalino. Em ambos os casos, existia dispersão bilateral de pigmento por todo o segmento anterior. No primeiro caso, os depósitos eram unilaterais e, no segundo, estavam presentes em ambos os olhos. Este pode corresponder a um sinal potencialmente importante da síndrome de dispersão pigmentar, raramente descrito.

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PURPOSE: To investigate whether pseudoexfoliation syndrome affects arterial stiffness by using cardio-ankle vascular index measurement. METHODS: This cross-sectional case-control study included 55 patients with pseudoexfoliation syndrome and 106 age- and gender-matched healthy control subjects. All subjects underwent a complete ophthalmic exa mination of both eyes and cardio-ankle vascular index measu rements. Echocardiographic and body mass index measurements were performed in all patients, and the results were recorded. A binary regression model was used to determine the relationship between cardio-ankle vascular index and pseudoexfoliation. RESULTS: There were no significant differences between the pseudoexfoliation and control groups in baseline clinical and demographic characteristics, echocardiographic measurements of left ventricular ejection fraction, and body mass index. The mean cardio-ankle vascular index value was significantly higher in the pseudoexfoliation group than in the controls (9.47 ± 1.23 vs. 8.33 ± 1.50, p<0.001). Intraocular pressure was significantly higher in the pseudoexfoliation group than in the controls (18.31 ± 1.78 vs. 15.24 ± 2.42 mm Hg, p<0.05). Although the logistic regression analysis showed that mean cardio-ankle vascular index and IOP values were positively associated with pseudoexfoliation syndrome (Odds ratios (OR) = 1.973, 95% CI, 1.051-3.706, p=0.035; OR=3.322, 95% CI = 2.000-5.520, p<0.001, respectively), the Pearson correlation analysis revealed a borderline significant positive correlation between age and mean cardio-ankle vascular index and a significant positive correlation between dyslipidemia and intraocular pressure and mean cardio-ankle vascular index (r=0.265, p=0.050; r=0.337, p=0.012; r=0.433, p=0.001, respectively). CONCLUSION: Our findings demonstrated that cardio-ankle vascular index values increased in patients with pseudoexfoliation syndrome.

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PURPOSE: To investigate inter-eye retinal vessel density and thickness asymmetry in unilateral pseudoexfoliation syndrome and understand its use for the early detection of glaucoma. METHODS: Thirty patients with unilateral pseudoexfoliation syndrome were enrolled in our study. Optical coherence tomography angiography macular scans were used measure the retinal vessel density, and optical coherence tomography scans were used to assess the thickness parameters of the peripapillary retinal nerve fiber layer and the macular ganglion cell complex. Inter-eye asymmetry was determined by taking the absolute value of the difference in the vessel density and thickness parameters between the pseudoexfoliation syndrome eye and fellow eye. RESULTS: The mean patient age was 64.20 ± 7.05 y in the study group. Inter-eye asymmetry in the peripapillary retinal nerve fiber layer thickness and macular ganglion cell complex measurements were significant in the study group (p=0.03 and p=0.001, respectively). The vessel density of the macular superficial inner region was significantly lower in eyes with pseudoexfoliation syndrome than in fellow eyes (p=0.035). However, there was no inter-eye asymmetry in the central and full region macular superficial vessel density of eyes with pseudoexfoliation syndrome and fellow eyes (p>0.05). CONCLUSION: Retinal vessel density can be evaluated using optical coherence tomography angiography measurements. There was inter-eye asymmetry in the inner region macular superficial vessel density, peripapillary retinal nerve fiber layer, and macular ganglion cell complex thickness of the unilateral pseudoexfoliation syndrome eyes and fellow eyes. Further studies on a larger number of subjects might provide more clarity regarding the relationship between the inter-eye asymmetry of the retinal vessel density and thickness parameters with early detection of glaucomatous damage.

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The Mayan population of Guatemala is understudied within eye and vision research. Studying an observational homogenous, geographically isolated population of individuals seeking eye care may identify unique clinical, demographic, environmental and genetic risk factors for blinding eye disease that can inform targeted and effective screening strategies to achieve better and improved health care distribution. This study served to: (a) identify the ocular health needs within this population; and (b) identify any possible modifiable risk factors contributing to disease pathophysiology within this population. We conducted a cross-sectional study with 126 participants. Each participant completed a comprehensive eye examination, provided a blood sample for genetic analysis, and received a structured core baseline interview for a standardized epidemiological questionnaire at the Salama Lions Club Eye Hospital in Salama, Guatemala. Interpreters were available for translation to the patients' native dialect, to assist participants during their visit. We performed a genome-wide association study for ocular disease association on the blood samples using Illumina's HumanOmni2.5-8 chip to examine single nucleotide polymorphism SNPs in this population. After implementing quality control measures, we performed adjusted logistic regression analysis to determine which genetic and epidemiological factors were associated with eye disease. We found that the most prevalent eye conditions were cataracts (54.8%) followed by pseudoexfoliation syndrome (PXF) (24.6%). The population with both conditions was 22.2%. In our epidemiological analysis, we found that eye disease was significantly associated with advanced age. Cataracts were significantly more common among those living in the 10 districts with the least resources. Furthermore, having cataracts was associated with a greater likelihood of PXF after adjusting for both age and sex. In our genetic analysis, the SNP most nominally significantly associated with PXF lay within the gene KSR2 (p < 1 × 10-5). Several SNPs were associated with cataracts at genome-wide significance after adjusting for covariates (p < 5 × 10-8). About seventy five percent of the 33 cataract-associated SNPs lie within 13 genes, with the majority of genes having only one significant SNP (5 × 10-8). Using bioinformatic tools including PhenGenI, the Ensembl genome browser and literature review, these SNPs and genes have not previously been associated with PXF or cataracts, separately or in combination. This study can aid in understanding the prevalence of eye conditions in this population to better help inform public health planning and the delivery of quality, accessible, and relevant health and preventative care within Salama, Guatemala.

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ABSTRACT Pseudoexfoliation syndrome is more frequent in people aged >50 yeears, and its prevalence increases with age. Few reports have described cases in younger patients, all with a history of ocular surgery, especially iris resection. Herein, we describe the case of a 27-year old man with bilateral advanced glaucoma and pseudoexfoliation material in OS. He had undergone cataract surgeries OU and a penetrating keratoplasty OD during childhood. Currently, he presented with an intraocular pressure of 40 mmHg OU. The OS showed a white flaky material in the pupillary rim and anterior capsule and a Sampaolesi line as a gonioscopic finding. Trabeculectomy was performed OU, and intraocular pressure control was achieved. Unlike other previously reported cases, this patient did not present any apparent iris manipulation in the affected eye. However, he did undergo an iridectomy in the contralateral eye. This is also the first case to be accompanied by bilateral glaucoma at the time of detection of the pseudoexfoliation material.(AU)

RESUMO A síndrome de pseudoesfoliação é mais frequente em pessoas com mais de 50 anos e sua prevalência aumenta com a idade. Poucos relatos descrevem casos em pacientes mais jovens, todos com história de cirurgia ocular, especialmente ressecção da íris. Descrevemos o caso de um homem de 27 anos com glaucoma bilateral avançado e material de pseudoesfoliação no OE. O paciente foi submetido a cirurgias de catarata em AO e a uma ceratoplastia penetrante no OD durante a infância. Atualmente, ele apresentou PIOs de 40 mmHg em AO. O OE apresentou material escamoso branco na borda pupilar e cápsula anterior, e linha Sampaolesi como achado gonioscópico. A trabeculectomia foi realizada em AO e obteve-se o controle da pressão intraocular. Diferentemente de outros casos relatados, o paciente não apresentou qualquer manipulação aparente da íris no olho afetado. No entanto, ele foi submetido a uma iridectomia no olho contralateral. Além disso, este é o primeiro caso a ser acompanhado de glaucoma bilateral no momento da detecção do material de pseudoesfoliação.(AU)

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PURPOSE: The aim of this study was to evaluate the appropriate timing of capsular tension ring implantation in cases of zonular weakness due to pseudoexfoliation syndrome. METHODS: This prospective, comparative study was performed at the Ophthalmology Department of Inonu University, Malatya, Turkey. There were 43 patients included in the study. Group 1 (16 patients) had early capsular tension ring implantation, and group 2 (27 patients) had late capsular tension ring implantation. Patients with pseudoexfoliation syndrome who underwent phacoemulsification surgery, posterior chamber intraocular lens implantation, and capsular tension ring implantation were included in the study. Intraoperative complications and difficulties with either capsular tension ring implantation or cortex removal were evaluated in each eye. RESULTS: There was no significant difference between the groups in the difficulty of capsular tension ring implantation (p=0.124). The difficulty of cortex removal differed significantly between the groups (p=0.003). Intraoperative complications were observed in 3 patients in group 1 and 11 patients in group 2; the difference between the groups was not significant (p=0.18). Posterior capsule fluctuations were observed in 8 patients (29.5%) in group 2, which resulted in posterior capsule rupture in 2 patients. CONCLUSIONS: Cortex removal is more difficult with early capsular tension ring implantation, and posterior capsule fluctuations may cause problems with late capsular tension ring implantation. The surgeon must consider the risk-to-benefit ratio of early versus late insertion for the optimal timing of capsular tension ring implantation.

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Pseudoexfoliation syndrome is more frequent in people aged >50 yeears, and its prevalence increases with age. Few reports have described cases in younger patients, all with a history of ocular surgery, especially iris resection. Herein, we describe the case of a 27-year old man with bilateral advanced glaucoma and pseudoexfoliation material in OS. He had undergone cataract surgeries OU and a penetrating keratoplasty OD during childhood. Currently, he presented with an intraocular pressure of 40 mmHg OU. The OS showed a white flaky material in the pupillary rim and anterior capsule and a Sampaolesi line as a gonioscopic finding. Trabeculectomy was performed OU, and intraocular pressure control was achieved. Unlike other previously reported cases, this patient did not present any apparent iris manipulation in the affected eye. However, he did undergo an iridectomy in the contralateral eye. This is also the first case to be accompanied by bilateral glaucoma at the time of detection of the pseudoexfoliation material.

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PRECIS: We conducted a literature review of younger patients with exfoliation syndrome (XFS) in an attempt to identify case similarities and better understand disease etiology. PURPOSE: XFS that predisposes to secondary glaucoma is a strongly age-related condition. We performed a literature review of XFS and exfoliation glaucoma (XFG) in patients aged younger than 40 years to examine potential common characteristics and gain clues to its etiology. METHODS: We conducted a broad literature search with appropriate keywords and manually extracted key demographic and ocular features on younger XFS and XFG cases. Articles that did not provide past ocular history on early-onset XFS/XFG were excluded. RESULTS: We identified 12 cases of XFS and XFG in patients from 13 to 40 years old (8 females; 11 White; 5 from Iran). All had past ocular history remarkable for intraocular surgery for other glaucoma conditions (7 cases), other ocular diseases (3 cases), or ocular trauma (2 cases). CONCLUSIONS: All reported early-onset XFS and XFG cases arise in the setting of events that produced a significant disruption of the blood-aqueous barrier. Understanding the metabolic alterations of aqueous humor from such cases could provide clues regarding how exfoliation material forms.

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