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Pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG) are two stages within the same ophthalmic disease spectrum, which are known to be affected by race. The prevalence of PDS is underestimated, largely due to its minor clinical symptoms. Although the prevalence of PG is low, the visual impairment associated with PG is extremely severe. The prevalence of PDS-PG is four or more times higher in Caucasians than in Blacks or Asians, and the "classic" PDS in Caucasians has long been used as a benchmark diagnostic criterion. Following extensive research focused on African Americans and Asians, the standard for diagnosing PDS-PG was refined. At the same time, the pathogenesis of PDS is not the same in different races. Hence, the effectiveness of preventive treatment and the need for treatment may not be equivalent in different races. The rate of conversion of PDS to PG is nearly 1/3 in Caucasians and higher in blacks and Asians, requiring more aggressive treatment and monitoring. We systematically searched a PubMed database from inception to March 2022 to provide an overview of research progress in various aspects of PDS-PG. Specifically, this paper considers the effects of race on disease prevalence, clinical manifestation, diagnostic criteria, disease mechanism, hereditary traits, treatment, and prevention to provide an accurate and comprehensive guide for the diagnosis and treatment of PDS-PG in various races. [ABSTRACT FROM AUTHOR]

Introduction: Pigment dispersion syndrome (PDS) is a condition where anomalous iridozonular contact leads to pigment dispersion throughout the anterior segment and the released pigment is abnormally deposited on various ocular structures. Clinical presentation: The clinical presentation of PDS is defined by the presence of pigmented cells on the corneal endothelium, an increase of pigmentation of the trabecular meshwork, and mid-periphery transillumination defects of the iris. This syndrome, more common in myopes, is usually bilateral and can be associated with ocular hypertension or glaucoma. Secondary open-angle pigmentary glaucoma (PG) can develop due to reduction of the outflow of aqueous humour and consequent increase in intraocular pressure leading to glaucomatous optic neuropathy. Diagnosis of PG is commonly between 40 and 50 years of age, occurring more frequently in men. The advent of ultrasound biomicroscopy and anterior segment optical coherence tomography has contributed to enhancing our knowledge on the condition. Typical alterations of the anterior segment are the posterior insertion of the iris and iris concavity. Treatment of PG should be initiated early to hinder disease progression, glaucomatous damage, and vision loss. Management is based on medical therapy, laser iridotomy, selective laser trabeculoplasty, and filtration procedures. Conclusions: The differential diagnosis of PDS with other disorders can be challenging and awareness of the condition together with meticulous ophthalmologic examination allows early diagnosis followed by appropriate management strategies. The present review is a comprehensive report on the clinical characteristics, pathogenesis, current management, and status quo of PDS and PG. [ABSTRACT FROM AUTHOR]

Purpose: Dysfunction of the trabecular meshwork (TM) in pigmentary glaucoma contributes to increased aqueous humor outflow resistance and intraocular pressure. In this study, we investigated the effect of pigment dispersion on trabecular meshwork cells. Methods: Porcine TM cells from ab interno trabeculectomy specimens were exposed to pigment dispersion, then, analyzed for changes in morphology, immunostaining, and ultrastructure. Their abilities to phagocytose migrate, and contraction was quantified. An expression microarray, using 23,937 probes, and a pathway analysis were performed. Results: Stress fiber formation was increased in the pigment dispersion group (P) (60.1 ± 0.3%, n = 10) compared to control (C) (38.4 ± 2.5%, n = 11, p < 0.001). Phagocytosis declined (number of cells with microspheres in P = 37.0 ± 1.1% and in C = 68.7 ± 1.3%, n = 3, p < 0.001) and migration was reduced after 6 h (cells within the visual field over 6 h in P = 28.0.1 ± 2.3 (n = 12) and in C = 40.6 ± 3.3 (n = 13), p < 0.01). Pigment induced contraction at 24 h onwards (p < 0.01). Microarray analysis revealed that Rho signaling was central to these responses. Conclusion: Exposure of TM cells to pigment dispersion resulted in reduced phagocytosis and migration, as well as increased stress fiber formation and cell contraction. The Rho signaling pathway played a central and early role, suggesting that its inhibitors could be used as a specific intervention in treatment of pigmentary glaucoma. [ABSTRACT FROM AUTHOR]

Purpose: This study investigated the hypotensive effect of RKI-1447, a Rho kinase inhibitor, in a porcine ex vivo pigmentary glaucoma model.Methods: Twenty-eight porcine anterior chambers were perfused with medium supplemented with 1.67 × 107 pigment particles/ml for 48 h before treatment with RKI-1447 (n = 16) or vehicle control (n = 12). Intraocular pressure (IOP) was recorded and outflow facility was calculated. Primary trabecular meshwork cells were exposed to RKI-1447 or vehicle control; effects on the cytoskeleton, motility, and phagocytosis were evaluated.Result: Compared to baseline, the perfusion of pigment caused a significant increase in IOP in the RKI-1447 group (P = 0.003) at 48 h. Subsequent treatment with RKI-1447 significantly reduced IOP from 20.14 ± 2.59 to 13.38 ± 0.91 mmHg (P = 0.02). Pigment perfusion reduced the outflow facility from 0.27 ± 0.03 at baseline to 0.18 ± 0.02 at 48 h (P < 0.001). This was partially reversed with RKI-1447. RKI-1447 caused no apparent histological changes in the micro- or macroscopic TM appearance. RKI-1447-treated primary TM cells showed significant disruption of the actin cytoskeleton both in the presence and absence of pigment (P < 0.001) but no effect on TM migration was observed. Pigment-treated TM cells exhibited a reduction in TM phagocytosis, which RKI-1447 reversed.Conclusion: RKI-1447 significantly reduces IOP by disrupting TM stress fibers and increasing TM phagocytosis. These features may make it useful for the treatment of secondary glaucomas with an increased phagocytic load. [ABSTRACT FROM AUTHOR]

Purpose: To describe our experience with exchanging sulcus-fixated single-piece intraocular lens (IOL) with 3-piece IOLs for management of pigmentary glaucoma.Method: In this retrospective study, records of patients who underwent sulcus-fixated single-piece IOL exchanged with 3-piece IOLs were retrieved, and demographic and baseline data of patients, type of IOL, pre- and post-IOL exchange BCVA, IOP, number of anti-glaucoma medications, and optic nerve head examination were documented. Baseline and final examinations were analyzed and compared.Results: Mean age of the patients was 59 ± 10 years, and 5 (41.6%) were female. Mean interval between primary cataract extraction operation and IOL exchange was 17 ± 5 months. Nine patients received in sulcus implantation of Alcon SA60AT, and three patients had SN60WF model at the end of primary surgery. BCVA changed insignificantly from 0.06 ± 0.06 logMAR to 0.06 ± 0.06 after IOL exchange. (P = 0.22) IOP was controlled in 8 cases (66.6%), but four cases (33.3%) needed glaucoma surgery to further control glaucoma condition. IOP decreased significantly from preoperative 17 ± 3 to 14 ± 1 mmHg postoperatively. Patients with advanced age and higher baseline IOP were more likely to undergo glaucoma surgery after IOL exchange. (P = 0.07 and 0.00, respectively).Conclusion: single-piece IOL exchange with 3-piece IOL dramatically decreases pigment release and reduces IOP. Those with advanced age and higher IOP are less likely to respond to IOL exchange and may need glaucoma surgery to control high intraocular pressure. [ABSTRACT FROM AUTHOR]

To compare visual field defect patterns between pigmentary glaucoma and primary open-angle glaucoma. Retrospective, comparative study. Patients with diagnosis of primary open-angle glaucoma (POAG) and pigmentary glaucoma (PG) in mild to moderate stages were enrolled in this study. Each of the 52 point locations in total and pattern deviation plot (excluding 2 points adjacent to blind spot) of 24-2 Humphrey visual field as well as six predetermined sectors were compared using SPSS software version 20. Comparisons between 2 groups were performed with the Student t test for continuous variables and the Chi-square test for categorical variables. Thirty-eight eyes of 24 patients with a mean age of 66.26 ± 11 years (range 48-81 years) in the POAG group and 36 eyes of 22 patients with a mean age of 50.52 ± 11 years (range 36-69 years) in the PG group were studied. ( P = 0.00). More deviation was detected in points 1, 3, 4, and 32 in total deviation ( P = 0.03, P = 0.015, P = 0.018, P = 0.023) and in points 3, 4, and 32 in pattern deviation ( P = 0.015, P = 0.049, P = 0.030) in the POAG group, which are the temporal parts of the field. It seems that the temporal area of the visual field in primary open-angle glaucoma is more susceptible to damage in comparison with pigmentary glaucoma. [ABSTRACT FROM AUTHOR]

Background: The effectiveness and complication profile of the iStent inject implantation among different open angle glaucoma subgroups were analyzed. Methods: In this retrospective cohort outcome study, 35 consecutive patients suffering from glaucoma (primary open angle glaucoma (POAG) N = 17, pseudoexfoliation glaucoma (PEX) N = 15, and pigmentary glaucoma (PG) N = 3) were treated with the iStent inject. The intraocular pressure (IOP) and the number of antiglaucoma medications before and after surgery were evaluated. Results: In POAG, the mean IOP at 6 months measured 14.19 ± 1.38 mmHg with an average decrease of 33 % from preoperative IOP ( p < 0.001), and 15.33 ± 1.07 mmHg with an average decrease of 35 % in PEX ( p < 0.001), respectively. The number of antiglaucoma medications significantly decreased from 2.19 ± 0.91 to 0.88 ± 0.62 in POAG ( p < 0.001) and from 2.33 ± 1.23 to 1.04 ± 0.30 in PEX ( p < 0.001) after 6 months. In PG, IOP before surgery was 28.31 ± 3.21 mmHg and the number of antiglaucoma medications was 3.66 ± 0.57. One day after surgery, IOP decreased significantly to 12.33 mmHg ± 4.93 ( p < 0.001). Within four weeks after surgery, IOP was raised above 30 mmHg in every patient. To exclude a steroid response, topical steroids were stopped, but IOP did not decrease. To exclude blockage, Nd:YAG - laser treatment of the visible opening of the iStents was performed. Since the IOP stayed high and escalation of antiglaucoma medication was insufficient to control IOP, trabeculectomy was performed. Conclusions: In conclusion, implantation of the iStent inject has the ability to lower the postoperative IOP significantly in POAG and PEX after a short follow-up of 6 months with a favorable risk profile. However, limitation of this surgical procedure in phakic PG may exist and need to be investigated in further studies. [ABSTRACT FROM AUTHOR]

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Background: In this retrospective comparative cohort outcome study, the influence of Selective Laser Trabeculoplasty (SLT) on combined clear cornea phacoemulsification and ab interno trabeculectomy (Trabectome) outcomes in Primary Open Angle Glaucoma (POAG), Pseudoexfoliation Glaucoma (PEX), and Pigmentary Glaucoma (PG) was examined. Methods: Combined clear cornea phacoemulsification and Trabectome were performed in 27 consecutive patients with POAG, in 27 patients with PEX, and in 20 patients with PG. Each group was divided into two subgroups including patients without SLT treatment prior to surgery and patients who had insufficient response to 360° SLT treatment three months prior to surgery. Results: In the SLT group, mean IOP at six months measured 13.33 ± 2.08 mmHg with an average decrease of 30 % from preoperative IOP in the POAG group, 12.10 ± 1.40 mmHg with an average decrease of 46 % in the PEX group, and 11.83 ± 2.21 mmHg with an average decrease of 38 % in the PG group. In eyes without previous SLT, mean IOP sixt 6 months measured 11.00 ± 1.73 mmHg with an average decrease of 38 % from preoperative IOP in the POAG group, 15.50 ± 1.41 mmHg with an average decrease of 35 % in the PEX group, and 15.67 ± 2.91 mmHg with an average decrease of 36 % in the PG group, respectively. Conclusions: Prior SLT treatment seems not to negatively influence combined clear cornea phacoemulsification and Trabectome outcomes in glaucoma patients. However, SLT treatment may even have an additive effect on following combined Trabectome outcomes in patients with PEX and PG. [ABSTRACT FROM AUTHOR]

In this study, the role of melanopsin-expressing retinal ganglion cells (mRGCs) in the glaucoma-induced depressive behavioral response pattern was investigated. The CFP-D2 transgenic glaucoma animal model from five age groups was used in this study. Immunohistochemical labeling, quantitative analysis of mRGC morphology, open field test (OFT), and statistical analysis were used. In comparison with C57 BL/6 mice, the age-matched CFP-D2 mice had significantly elevated intraocular pressure (IOP). We observed parallel morphological changes in the retina, including a reduction in the density of cyan fluorescent protein-(CFP) expressing cells (cells mm at 2 months of age, 1309±26; 14 months, 878±30, P<0.001), mRGCs (2 months, 48±3; 14 months, 19±4, P<0.001), Brn3b-expressing RGCs (2 months, 1283±80; 14 months, 950±31, P <0.001), Brn-3b expressing mRGCs (5 months, 50.17%±5.5%; 14 months, 12.61%±3.8%, P<0.001), and reduction in the dendritic field size of mRGCs (mm at 2 months, 0.077±0.015; 14 months, 0.065±0.015, P<0.05). CFP-D2 mice had hyperactive locomotor activity patterns based on OFT findings of the total distance traveled, number of entries into the center, and time spent in the center of the testing apparatus. The glaucoma induced hyperactive response pattern could be associated with dysfunctional mRGCs, most likely Brn-3b-positive mRGCs in CFP-D2 mice. [ABSTRACT FROM AUTHOR]

Purpose: To report two Japanese cases of pigmentary glaucoma (PG) treated with laser peripheral iridotomy (LPI) that were followed for 15 and 16 years, respectively. Methods: The medical records of two patients with PG who were successfully treated with LPI were reviewed. Changes in the intraocular pressure (IOP) were followed. Results: Case 1 was that of a 35-year-old man with LPI who underwent argon laser trabeculoplasty twice. He required ocular hypotensive drugs to maintain the IOP at normal levels. Case 2 involved a 36-year-old man with LPI who required ocular hypotensive drugs to maintain his IOP at the low-teen level. The IOP of both patients was unstable during the first 6-8 years following the LPI, but showed a decrease at each annual follow-up examination up to the age of 50 years. Conclusions: Although only two cases were followed, we conclude that the long-term effects of LPI may play a role, at least partly, in stabilizing the IOP at the low-teen level. Both patients were relatively young at the time of the LPI, and age may have been a factor in the stabilization process. Our findings confirm similar findings in Western countries. [ABSTRACT FROM AUTHOR]

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Purpose: To investigate the racial heritage of a group of African-Americans who exhibit the pigment dispersion syndrome (PDS). Subjects and Methods: Ten unrelated African-American PDS patients (age range 13–59) from a primary eye care population in Chicago, Illinois, USA, were interviewed to determine their racial heritage. Since Caucasian and Native American heritage were commonly reported, 101 unaffected African-American control subjects (age range 18–55) were also interviewed to determine the reported frequency of these specific racial heritage characteristics. Results: Some degree of Caucasian heritage was reported by 100% of the PDS subjects and by 46.5% of controls. Native American heritage was reported by 90% of the PDS subjects and by 71.3% of controls. Based on these data, the PDS subjects were significantly more likely to report Caucasian heritage (Fisher's exact test, P=0.001) but they were not significantly more likely to report Native American heritage ( P=0.282). In addition, the PDS subjects were significantly more likely to report Caucasian heritage from both parents ( P=0.024) and more likely to report a combination of both Caucasian and Native American heritage ( P=0.0006) than corresponding controls. In general, the PDS subjects had `light' or `medium' complexions. Conclusion: Results from this analysis are consistent with the hypothesis that non-African heritage, particularly Caucasian, is likely to be prevalent in the background of African-Americans who exhibit PDS. It is suggested that non-African heritage may not only be important for the transference of PDS causing genes, but it may also influence factors such as degree of iris pigmentation and rigidity which could influence iris contour and the subsequent expression of PDS in those who are genetically predisposed. Further investigation is needed to study the factors that influence the expression and severity of PDS among African-Americans, a population which traditionally has been considered to be rarely affected by this condition. [ABSTRACT FROM AUTHOR]

Copyright of Spektrum der Augenheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Ophthalmologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)