Pigment dispersion syndrome and pigmentary glaucoma: overview and racial disparities.

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.

Disrupting the Repeat Domain of Premelanosome Protein (PMEL) Produces Dysamyloidosis and Dystrophic Ocular Pigment Reflective of Pigmentary Glaucoma.

Pigmentary glaucoma has recently been associated with missense mutations in PMEL that are dominantly inherited and enriched in the protein's fascinating repeat domain. PMEL pathobiology is intriguing because PMEL forms functional amyloid in healthy eyes, and this PMEL amyloid acts to scaffold melanin deposition. This is an informative contradistinction to prominent neurodegenerative diseases where amyloid formation is neurotoxic and mutations cause a toxic gain of function called "amyloidosis". Preclinical animal models have failed to model this PMEL "dysamyloidosis" pathomechanism and instead cause recessively inherited ocular pigment defects via PMEL loss of function; they have not addressed the consequences of disrupting PMEL's repetitive region. Here, we use CRISPR to engineer a small in-frame mutation in the zebrafish homolog of PMEL that is predicted to subtly disrupt the protein's repetitive region. Homozygous mutant larvae displayed pigmentation phenotypes and altered eye morphogenesis similar to presumptive null larvae. Heterozygous mutants had disrupted eye morphogenesis and disrupted pigment deposition in their retinal melanosomes. The deficits in the pigment deposition of these young adult fish were not accompanied by any detectable glaucomatous changes in intraocular pressure or retinal morphology. Overall, the data provide important in vivo validation that subtle PMEL mutations can cause a dominantly inherited pigment pathology that aligns with the inheritance of pigmentary glaucoma patient pedigrees. These in vivo observations help to resolve controversy regarding the necessity of PMEL's repeat domain in pigmentation. The data foster an ongoing interest in an antithetical dysamyloidosis mechanism that, akin to the amyloidosis of devastating dementias, manifests as a slow progressive neurodegenerative disease.

Genome-Wide Association Study Identifies Two Common Loci Associated with Pigment Dispersion Syndrome/Pigmentary Glaucoma and Implicates Myopia in its Development.

PURPOSE: To identify genetic variants associated with pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG) in unrelated patients and to further understand the genetic and potentially causal relationships between PDS and associated risk factors. DESIGN: A 2-stage genome-wide association meta-analysis with replication and subsequent in silico analyses including Mendelian randomization. PARTICIPANTS: A total of 574 cases with PG or PDS and 52 627 controls of European descent. METHODS: Genome-wide association analyses were performed in 4 cohorts and meta-analyzed in 3 stages: (1) a discovery meta-analysis was performed in 3 cohorts, (2) replication was performed in the fourth cohort, and (3) all 4 cohorts were meta-analyzed to increase statistical power. Two-sample Mendelian randomization was used to determine whether refractive error and intraocular pressure exert causal effects over PDS. MAIN OUTCOME MEASURES: The association of genetic variants with PDS and whether myopia exerts causal effects over PDS. RESULTS: Significant association was present at 2 novel loci for PDS/PG. These loci and follow-up analyses implicate the genes gamma secretase activator protein (GSAP) (lead single nucleotide polymorphism [SNP]: rs9641220, P = 6.0×10-10) and glutamate metabotropic receptor 5 (GRM5)/TYR (lead SNP: rs661177, P = 3.9×10-9) as important factors in disease risk. Mendelian randomization showed significant evidence that negative refractive error (myopia) exerts a direct causal effect over PDS (P = 8.86×10-7). CONCLUSIONS: Common SNPs relating to the GSAP and GRM5/TYR genes are associated risk factors for the development of PDS and PG. Although myopia is a known risk factor, this study uses genetic data to demonstrate that myopia is, in part, a cause of PDS and PG.

Long-term complications of cosmetic iris implants.

BACKGROUND: Additive cosmetic implants (NewColorIris, Kahn Medical Devices, Panama City, Panama) are placed in the anterior chamber, in order to externally change iris color. There is a lack of robust clinical long-term prospective studies regarding the safety of these devices, as they have been related to the early-onset presentation of corneal decompensation, elevated intraocular pressure (IOP), uveitis and hyphema. However, in this case report some mild complications started to manifest unexpectedly late: 15 years after an uneventful procedure. CASE PRESENTATION: A 41-year-old Caucasian woman presented with blurred vision in both eyes over the last 6 months. Fifteen years earlier, she had undergone bilateral implantation of additive iris implants for aesthetic purposes, without any complication or ocular trauma during the follow-up. Ocular examination showed bilateral mild corneal edema, iris atrophy, and presence of pigment in the endothelium. Increased IOP (28 mmHg) was identified in the right eye. Anterior segment optical coherence tomography (AS-OCT) confirmed the decentration of the iris implant from the pupillary axis in that eye. Gonioscopy demonstrated pigment dispersion in both eyes, as well as a tendency to bilateral angle closure, that was also illustrated by AS-OCT analysis. Endothelial cell count was 1268 cells/mm2 in the right eye and 1122 cells/mm2 in the left eye. The presence of both implants was affecting corneal endothelium and anterior chamber angle in both eyes, and additionally, the decentration of the device in the case of the right eye led to secondary ocular hypertension in that eye. CONCLUSIONS: Cosmetic implants in contact to the iris can remain quiescent for years, leading to possible complications that can present even in the long-term. The degree of implant decentration, the stage of angle closure disease and the magnitude of pigment dispersion may be some important factors related to the onset time of complications in these cases.

Exome-based investigation of the genetic basis of human pigmentary glaucoma.

BACKGROUND: Glaucoma is a leading cause of visual disability and blindness. Release of iris pigment within the eye, pigment dispersion syndrome (PDS), can lead to one type of glaucoma known as pigmentary glaucoma. PDS has a genetic component, however, the genes involved with this condition are largely unknown. We sought to discover genes that cause PDS by testing cohorts of patients and controls for mutations using a tiered analysis of exome data. RESULTS: Our primary analysis evaluated melanosome-related genes that cause dispersion of iris pigment in mice (TYRP1, GPNMB, LYST, DCT, and MITF). We identified rare mutations, but they were not statistically enriched in PDS patients. Our secondary analyses examined PMEL (previously linked with PDS), MRAP, and 19 other genes. Four MRAP mutations were identified in PDS cases but not in controls (p = 0.016). Immunohistochemical analysis of human donor eyes revealed abundant MRAP protein in the iris, the source of pigment in PDS. However, analysis of MRAP in additional cohorts (415 cases and 1645 controls) did not support an association with PDS. We also did not confirm a link between PMEL and PDS in our cohorts due to lack of reported mutations and similar frequency of the variants in PDS patients as in control subjects. CONCLUSIONS: We did not detect a statistical enrichment of mutations in melanosome-related genes in human PDS patients and we found conflicting data about the likely pathogenicity of MRAP mutations. PDS may have a complex genetic basis that is not easily unraveled with exome analyses.

Prevalence of zonulopathy in primary angle closure disease.

BACKGROUND: To determine the prevalence of zonulopathy in a large cohort of eyes with primary angle closure disease (PACD) that underwent cataract surgery. METHODS: Retrospective consecutive case series of PACD eyes (including primary angle closure suspect, primary angle closure, and primary angle closure glaucoma) that underwent phacoemulsification cataract surgery or clear lens extraction between 2009 and 2020 at a single ophthalmology centre. Those with risk factors for zonulopathy such as history of trauma, pseudoexfoliation syndrome, intraocular surgery, retinitis pigmentosa or connective tissue disorders were excluded. The primary outcomes included the prevalence of zonulopathy assessed intraoperatively and secondary pigment dispersion syndrome. RESULTS: In our cohort of 806 consecutive PACD eyes, the prevalence of zonulopathy was 7.3% (59 of 806 eyes) - significantly greater than the 0.46%-2.6% range reported for the general population (p < 0.001). Intraoperative signs of zonular weakness included floppy capsular bag (29 eyes, 3.6%), zonular laxity (25 eyes, 3.1%) and zonular dehiscence (11 eyes, 1.4%). Among these eyes, capsular tension ring was used in 23 eyes (39.0%), six eyes (10.2%) experienced vitreous prolapse intraoperatively and underwent anterior vitrectomy, and two eyes (3.4%) experienced posterior capsular rupture, one of which required a scleral-fixated intraocular lens. Secondary pigment dispersion syndrome was observed in 141 eyes (17.5%). CONCLUSIONS: This study evidenced a high prevalence of zonulopathy among a large cohort of PACD eyes and suggests zonulopathy as a possible under-recognised cause of angle closure. Until more sophisticated imaging modalities become available, awareness about the prevalence of zonulopathy in angle closure disease coupled with careful preoperative examinations can help minimise or prevent the complications of zonulopathy.

[Pigmentary glaucoma: yesterday, today, tomorrow]. / Pigmentnaya glaukoma: vchera, segodnya, zavtra.

Pigment dispersion syndrome (PDS) is a condition that mostly affects young men with myopic refraction. PDS is characterized by the presence of Krukenberg spindle, peripheral iris defects, significant trabecular meshwork pigmentation, as well as convex iris configuration. Such configuration can cause friction of iris's posterior pigment layer on its ligaments, which leads to the release of pigment and its accumulation mostly in the structures of the anterior chamber. Over time PDS can progress into pigmentary glaucoma (PG), which in turn can lead to permanent loss of vision. This review analyzes available data on diagnosis and treatment of PDS and PG.

Pigment dispersion syndrome and pigmentary glaucoma: a review and update.

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.

Determination Of Association Of Pigmentary Glaucoma With Pigment Dispersion Syndrome.

BACKGROUND: Pigment Dispersion Syndrome (PDS) is an autosomal dominant disorder of white males between 20 to 40 years of age characterized by deposition of pigment on the lens, zonules of lens, trabecular meshwork and corneal endothelium (Krukenberg's spindle) in addition to radial, spoke like transillumination defects in the mid peripheral iris. This study was conducted to determine the frequency of occurrence of Pigmentary Glaucoma in patients with Pigment Dispersion Syndrome (PDS). METHODS: This longitudinal follow up study included patients presenting with Krukenberg's spindle on the endothelial side of cornea and pigmentation of angle of anterior chamber seen on slit lamp examination and gonioscopy. RESULTS: Seventy-two cases of PDS were included in the study, amongst them 63 (87.50%) were males. Mean age was 35.00±6.54 years (range 24-46 years). Forty-seven (65.28%) patients had an IOP in the range of 10-14 mmHg, 22 (30.56%) patients had an IOP in the range of 15-18 mmHg and 3 (4.17%) patients developed an IOP of greater than 19 mmHg. Fundoscopy showed myopic degeneration in 49 (68.06%) patients and optic disc cupping in 3 (4.17%) patients. Four (5.56%) patients had refractive error between +1D to +3D, 9 (12.50%) patients had refractive error between -1D to -4D, 21 (29.17%) patients had refractive error between -5 D to -8 D and 38 (52.78%) patients had refractive error between -9 D to -12 D. Our study showed that one patient having PDS developed glaucoma at 5 years of follow up and three patients developed glaucoma at 14 years of follow up. CONCLUSIONS: On the basis of this study we conclude that early onset primary open angle glaucoma associated with PDS or Juvenile glaucoma associated with PDS might have been mistaken as Pigmentary Glaucoma in Pakistani patients and a distinct entity in the form of Pigmentary Glaucoma may be non-existent.

Bilateral Late-Onset Pigment Dispersion Syndrome following Implantable Collamer Lens Surgery: A Case Report.

Introduction: We report a case of bilateral pigment dispersion syndrome after 13 years of uncomplicated implantable collamer lens (ICL) surgery. Case Presentation: A 53-year-old woman was referred from her optometrist to our glaucoma clinic due to early superonasal visual field loss in both eyes. She was asymptomatic with no changes in visual acuity and had undergone bilateral ICL implantation 13 years ago to correct her high myopia. Clinical examination revealed pigment deposition on the corneal endothelium, iris transillumination defects, and iris vaulting at the areas of contact with the ICL. Gonioscopy showed open angles with significant pigmentation of the trabecular meshwork. The diagnosis of pigment dispersion syndrome secondary to ICL implantation was made, and subsequent follow-up visits demonstrated normal intraocular pressure IOP and stable visual fields. Conclusion: Pigmentary dispersion syndrome can occur several years after ICL implantation. This case report emphasizes the need for long-term follow-up and monitoring after ICL surgery.

Secondary glaucoma: Toward interventions based on molecular underpinnings.

Glaucoma is a heterogeneous group of progressive diseases that leads to irreversible blindness. Secondary glaucoma refers to glaucoma caused by a known underlying condition. Pseudoexfoliation and pigment dispersion syndromes are common causes of secondary glaucoma. Their respective deposits may obstruct the trabecular meshwork, leading to aqueous humor outflow resistance, ocular hypertension, and optic neuropathy. There are no disease-specific interventions available for either. Pseudoexfoliation syndrome is characterized by fibrillar deposits (pseudoexfoliative material) on anterior segment structures. Over a decade of multiomics analyses taken together with the current knowledge on pseudoexfoliative glaucoma warrant a re-think of mechanistic possibilities. We propose that the presence of nucleation centers (e.g., vitamin D binding protein), crosslinking enzymes (e.g., transglutaminase 2), aberrant extracellular matrix, flawed endocytosis, and abnormal aqueous-blood barrier contribute to the formation of proteolytically resistant pseudoexfoliative material. Pigment dispersion syndrome is characterized by abnormal iridolenticular contact that disrupts iris pigment epithelium and liberates melanin granules. Iris melanogenesis is aberrant in this condition. Cytotoxic melanogenesis intermediates leak out of melanosomes and cause iris melanocyte and pigment epithelium cell death. Targeting melanogenesis can likely decrease the risk of pigmentary glaucoma. Skin and melanoma research provides insights into potential therapeutics. We propose that specific prostanoid agonists and fenofibrates may reduce melanogenesis by inhibiting cholesterol internalization and de novo synthesis. Additionally, melatonin is a potent melanogenesis suppressor, antioxidant, and hypotensive agent, rendering it a valuable agent for pigmentary glaucoma. In pseudoexfoliative glaucoma, where environmental insults drive pseudoexfoliative material formation, melatonin's antioxidant and hypotensive properties may offer adjunct therapeutic benefits. This article is categorized under: Neurological Diseases > Molecular and Cellular Physiology.

Genetic Basis of Pigment Dispersion Syndrome and Pigmentary Glaucoma: An Update and Functional Insights.

Pigment Dispersion Syndrome (PDS) and Pigmentary Glaucoma (PG) comprise a spectrum of ocular disorders characterized by iris pigment dispersion and trabecular meshwork changes, resulting in increased intraocular pressure and potential glaucomatous optic neuropathy. This review summarizes recent progress in PDS/PG genetics including rare pathogenic protein coding alterations (PMEL) and susceptibility loci identified from genome-wide association studies (GSAP and GRM5/TYR). Areas for future research are also identified, especially the development of efficient model systems. While substantial strides have been made in understanding the genetics of PDS/PG, our review identifies key gaps and outlines the future directions necessary for further advancing this important field of ocular genetics.

Lack of Association between LOXL1 Variants and Pigment Dispersion Syndrome/Pigmentary Glaucoma: A Meta-Analysis.

The phenotypic similarities between exfoliation syndrome (XFS)/exfoliation glaucoma (XFG) and pigment dispersion syndrome (PDS)/pigmentary glaucoma (PG), particularly their association with material deposition in the eye's anterior segment, have prompted investigations into genetic commonalities. This study focuses on the LOXL1 gene, conducting a comprehensive meta-analysis of three candidate gene association studies. We analyzed three single nucleotide polymorphisms (SNPs) of LOXL1: rs1048661, rs3825942, and rs2165241. Our results reveal nominal significance for the exonic SNPs rs1048661 and rs3825942 (p ≤ 0.01), but show no significant association for the intronic SNP rs2165241 (p = 0.83) with PDS/PG. There was homogeneity across study cohorts (I2 = 0), and sensitivity analyses and funnel plots confirmed a lower likelihood of bias in our findings. The lack of a statistically significant association between LOXL1 variants and PDS/PG at p < 0.05 was attributable to the insufficient statistical power of the pooled data, which ranged from 5% to 37% for the three SNPs. This study suggests no association between LOXL1 variants and PDS/PG. Further validation and exploration of XFS/XFG-associated genes in larger and more diverse cohorts would be helpful to determine the genetic correlation or distinctiveness between these conditions.

Managing Pigment Dispersion Glaucoma Postbilateral ICL Implantation in High Myopia: A Case Report on the Crucial Role of Gonioscopy in Correcting a Misdiagnosis.

Secondary open-angle glaucoma (SOAG) is a rare yet consequential complication following implantable collamer lens (ICL), also known as a phakic intraocular lens insertion, particularly in high myopia patients. This case report emphasizes the importance of recognizing SOAG and details the diagnostic complexities, reevaluation procedures, and successful long-term management of a 24-year-old bilateral high myopia (-7.00 D) patient who initially received an erroneous diagnosis of secondary angle-closure glaucoma (SACG) after ICL insertion at an external medical facility. Persistent visual issues prompted the patient to seek a second opinion, leading to a comprehensive reevaluation that eventually unveiled pigment dispersion syndrome (PDS) as the underlying cause, subsequently resulting in SOAG. This case not only highlights the diagnostic challenges but also elucidates the re-evaluation process and effective 5-year management strategies employed to restore the patient's visual health and quality of life. How to cite this article: Ramesh PV, Parthasarathi S, Azad A, et al. Managing Pigment Dispersion Glaucoma Postbilateral ICL Implantation in High Myopia: A Case Report on the Crucial Role of Gonioscopy in Correcting a Misdiagnosis. J Curr Glaucoma Pract 2024;18(1):31-36.

Long anterior zonule trait, lessons learned about an uncommon form of secondary angle closure: a case report.

A 62-year-old black woman with uncontrolled chronic narrow-angle glaucoma on 3-drug therapy underwent phaco-non-perforating deep sclerectomy of her left eye. During surgery it was revealed that she had long zonule trait. She later required goniopuncture and conjuntival needling, presenting an iris herniation in the goniopuncture that could be reduced conservatively. Long anterior zonule trait should be suspected in those patients presenting with a combination of narrow angle and pigment dispersion syndrome. The management of ocular hypertension and glaucoma associated to this trait is not protocolized. This communication discusses on the best action in this rare form of glaucoma.

Unilateral Congenital Lenticular Pigmentation.

Introduction: Release of pigments in the anterior chamber is frequently observed in pigment dispersion syndrome, an autosomal dominant disorder marked by bilateral pigment deposition on the anterior and possibly posterior lens capsule, zonules of the lens, trabecular meshwork, and corneal endothelium, in addition to radial, spoke-like transillumination defects in the mid peripheral iris [J Ayub Med Coll Abbottabad. 2017;29(3):412-414 and Optom Vis Sci. 1995;72(10):756-762]. Pigmentation of the anterior lens surface has also been associated with intraocular inflammation, pseudoexfoliation syndrome, siderosis, antipsychotic medication usage, and remnants of the tunica vasculosa lentis [Br J Ophthalmol. 1998;82(11):1344]. Case Presentation: A 23-year-old female presented to our eye clinic with chief complaint of mild blurring of vision in the right eye and inquired about refractive surgery. The patient denied any previous history of ocular inflammation, trauma, surgery, or use of topical or systemic medications. Slit-lamp examination of the right eye anterior segment was within normal limits except for the crystalline lens anterior capsular which showed confluent pigment deposits stellate in shape over the pupillary axis, whereas left eye examination was completely within normal limits. Ophthalmic examination of the posterior segment was normal in both eyes. Based on her previous ophthalmic history and slit-lamp examination of the right eye, a diagnosis of unilateral congenital lenticular pigmentation was made. Conclusion: Congenital lenticular pigmentation is a rare benign entity carrying no surgical indications with a relatively good visual response to optical correction. Recognition of this rare benign condition would add to the ophthalmologist's differential of ocular pigmentation and avoid unnecessary concern and follow-up in more potentially progressive disorders such as pigmentary glaucoma.

Bilateral Acute Depigmentation of Iris (BADI) Post COVID Infection following Systemic Moxifloxacin Therapy.

PURPOSE: To report a case of BADI post Covid infection following systemic moxifloxacin therapy. METHODS: Observational case study. CASE REPORT: A 28-year-old female presented to us with complaints of redness and pain in the right eye since 5 days. History revealed use of systemic moxifloxacin for covid infection. She was managed with topical corticosteroids and cycloplegics following a diagnosis of BADI. CONCLUSION: Bilateral acute depigmentation of iris (BADI) is a rare disease entity associated with the release of iris pigments. Various underlying aetiologies have been associated with the same. Although BADI is a benign, self-limiting disorder, it needs to be differentiated with other potential severe clinical entities. SARS-CoV2 has been associated with various ocular manifestations. However, to the best of our knowledge, BADI has never been associated with COVID infection yet. We report a case of BADI in a young healthy Asian middle - aged female 3 months after an acute COVID infection.

Aperture photometry measurements of melanin particles in Krukenberg spindles of the cornea in pigment dispersion syndrome.

BACKGROUND: Quantification of melanin pigment release in pigment dispersion syndrome as well as observations of melanin brightness changes can be valuable information in the management of this rare ocular disease. OBJECTIVES: Previous studies have focused on examining the iris pigment epithelium and aqueous humor. Therefore, the aim of this study was to examine the cornea. METHODS: A novel technique was developed for this purpose based on aperture photometry. Slit lamp digital video images of the cornea were recorded. A single frame from each video recording based on the quality was chosen for further processing and analysis. Aperture photometry was performed with AstroImageJ open source software. Aperture selection was performed automatically. Melanin particles displaying a signal-to-noise ratio above 20 were analyzed. RESULTS: A total of 16 melanin particles from the right eye of the patient participating in the study were detected and a further 9 melanin particles from the left eye. The examined area of the cornea measured 348 × 348 pixels in the image. Brightness differed by as much as 8.98 × among particles in the right eye and 2.03 × in the left eye. CONCLUSIONS: It seems feasible for this new method to be potentially used in the monitoring of patients with pigment dispersion syndrome and pigmentary glaucoma as well as in other ocular diseases.

Transient visual blurring during a sexual intercourse in a young woman with surgically corrected myopia and unrecognized pigmentary glaucoma: A rare case report.

PURPOSE: To present a rare case of unilateral visual loss episodes occurred during sexual intercourse in a young patient affected by unrecognized pigmentary glaucoma and previously undergone myopic refractive surgery. CASE DESCRIPTION: The patient presented surgically flattened corneas and markedly asymmetric pigmentary glaucoma. CONCLUSIONS: Previous refractive surgery, sexual intercourse, and athletic lifestyle might be risk factors for acute pigment dispersion and chronic progression of pigmentary glaucoma in young myopic patients. During their ophthalmic examination prior to refractive surgery, greater attention should be paid to detect early signs of pigmentary dispersion, and awareness of these dangerous situations should be raised in affected patients.

A Case of Pigment Dispersion Syndrome after Placement of Sulcus Intraocular Lens with 7-mm Optic Diameter after Posterior Capsule Rupture.

A 48-year-old woman diagnosed with primary angle closure suspect (PACS) in the right eye underwent cataract surgery, and a 7-mm optic diameter intraocular lens (IOL) was placed in the ciliary sulcus after intraoperative posterior capsule rupture. The patient developed uveitis and blurred vision the next day. The IOL was fixed between the iris and the anterior capsule. Irregularly shaped pupils due to posterior synechia and pigmentation on the IOL surface were observed. In the Scheimpflug image, the IOL on the anterior capsule was observed and the anterior chamber depth was 2.92 mm. A diagnosis of pigment dispersion syndrome and elevated intraocular pressure due to sulcus IOL placement was made. The patient underwent intrascleral IOL fixation surgery using an already inserted IOL to reposition the IOL under the anterior capsule. After 1 week, the blurred vision, anterior chamber inflammation, and IOL surface pigmentation were resolved. The right eye IOP was 15 mm Hg and the pupil became a regular circle. Scheimpflug images showed the IOL located behind the anterior capsule and an anterior chamber depth of 3.88 mm. Because the patient had a slightly shorter axial length of 22.89 mm and PACS, pigment dispersion may have occurred due to friction between the iris and the shape of the optic edge with a large optic diameter. In cases of posterior capsule rupture with short axial length and PACS, the use of a 7-mm optic diameter IOL in the sulcus should be avoided, or intrascleral IOL fixation should be selected as the surgical technique.