Changes of trabecular meshwork pigmentation in patients with pigment dispersion syndrome

AbstractPigment dispersion syndrome can lead to pigmentary glaucoma (PG), a poorly understood condition of younger, myopic eyes with fluctuating, high intraocular pressure (IOP). The absence of a model similar in size and behavior to human eyes has made it difficult to investigate its pathogenesis. Here, we present a porcine ex vivo model that recreates the features of PG including intraocular hypertension, pigment accumulation in the trabecular meshwork and relative failure of phagocytosis. Inin vitromonolayer cultures as well as inex vivoeye perfusion cultures, we found that the trabecular meshwork (TM) cells that regulate outflow, form actin stress fibers and have a decreased phagocytosis. Gene expression microarray and pathway analysis indicated key roles of RhoA in regulating the TM cytoskeleton, motility, and phagocytosis thereby providing new targets for PG therapy.

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An Iconic Case of Pigmentary Glaucoma: Brief Review of the Literature

Case Reports in Ophthalmology ◽

10.1159/000508605 ◽

2020 ◽

Vol 11 (2) ◽

pp. 377-384

Author(s):

Mariachiara Di Pippo ◽

Chiara Ciancimino ◽

Luca Scuderi ◽

Andrea Perdicchi

Keyword(s):

Intraocular Pressure ◽

Trabecular Meshwork ◽

Corneal Endothelium ◽

Posterior Part ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Anterior Surface ◽

Review Of The Literature ◽

Pigment Dispersion Syndrome

Pigment dispersion syndrome and pigmentary glaucoma are two conditions characterized by pigment dispersion originating from the posterior part of the iris and its accumulation on the trabecular meshwork, corneal endothelium, and anterior surface of the lens. The pigment on the trabecular meshwork can cause chronic inflammation with a secondary reduction of its function and an increase in intraocular pressure. The case presented represents a typical example of pigmentary glaucoma in a myopic patient in which all the signs, symptoms, and complications typical of these pathologies were present. We report and describe an 8-year-long follow-up period with clinical and instrumental examinations.

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Pigment dispersion syndrome with possible visual field loss (in Norwegian)

Scandinavian Journal of Optometry and Visual Science ◽

10.5384/sjovs.vol3i1p ◽

2010 ◽

Vol 3 (1) ◽

pp. 8-14

Author(s):

Ellen Svarverud

Keyword(s):

Trabecular Meshwork ◽

Visual Fields ◽

Anterior Segment ◽

Clinical Signs ◽

Visual Field Loss ◽

Diagnostic Methods ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Pigment Dispersion Syndrome ◽

Pigment Deposition

Pigment dispersion syndrome (PDS) is a condition of the anterior segment of the eye characterised by pigment deposition on a number of ocular structures. The condition is usually bilateral but most commonly asymmetric. In PDS, pigment is released from the posterior surface of the iris due to friction between the zonules and the iris. In itself, the condition does not represent any problems for the patient, but pigment deposition in the trabecular meshwork may interfere with drainage of the anterior chamber fluid and cause pigmentary glaucoma (PG). This case report presents a young myopic female with many of the classic signs of PDS; Krukenberg’s spindle, transillumination of mid-peripheral iris, deep anterior chambers, concave iris profile and increased pigmentation in the trabecular meshwork. Various diagnostic methods were applied to reveal clinical signs and to establish the patient’s visual function. Optic nerve heads and intraocular pressure were normal, but visual fields were suspect. The patient was advised to make an appointment with an ophthalmologist. Risk factors for development of PG and management of patients with PDS will be discussed.

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What's in a Gene Pseudoexfoliation Syndrome and Pigment Dispersion Syndrome in the Same Patient

Case Reports in Ophthalmology ◽

10.1159/000443697 ◽

2016 ◽

Vol 7 (1) ◽

pp. 54-60 ◽

Cited By ~ 1

Author(s):

Olya Pokrovskaya ◽

Colm O''Brien

Keyword(s):

Significant Role ◽

Trabecular Meshwork ◽

Unusual Case ◽

Open Angle Glaucoma ◽

The Other ◽

Pseudoexfoliation Syndrome ◽

Pigment Dispersion ◽

Open Angle ◽

Pigment Dispersion Syndrome ◽

Interesting Discussion

Pseudoexfoliation syndrome (PXS) and pigment dispersion syndrome (PDS) are two of the commonest disorders to produce secondary open-angle glaucoma through trabecular meshwork blockage. Each is a defined clinical entity, and while genetics likely play a significant role in the pathogenesis of both, the specific genes involved appear to be distinct. There is surprisingly little published in the literature regarding the coexistence of PDS and PXS in the same patient. We present the intriguing case of a patient who developed PDS in one eye and PXS in the other. This unusual case acts as a platform for an interesting discussion of the genomics of PXS and PDS.

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The Role of Dynamic Ultrasound Biomicroscopy in defining Laser Treatment in Pigment Dispersion Syndrome/ Pigmentary Glaucoma. A Case Series

International Archives of BioMedical and Clinical Research ◽

10.21276/iabcr.2016.2.3.16 ◽

2016 ◽

Vol 2 (3) ◽

Author(s):

Atif Anwar ◽

◽

Triputi Nath

Keyword(s):

Laser Treatment ◽

Case Series ◽

Ultrasound Biomicroscopy ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Pigment Dispersion Syndrome ◽

Dynamic Ultrasound

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Iris configuration in accommodation in pigment dispersion syndrome

Eye ◽

10.1038/sj.eye.6700115 ◽

2002 ◽

Vol 16 (6) ◽

pp. 694-700 ◽

Cited By ~ 11

Author(s):

M O Balidis ◽

C Bunce ◽

C J Sandy ◽

R P L Wormald ◽

M H Miller

Keyword(s):

Pigment Dispersion ◽

Pigment Dispersion Syndrome

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

BMC Genomics ◽

10.1186/s12864-021-07782-0 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Carly van der Heide ◽

Wes Goar ◽

Kacie J. Meyer ◽

Wallace L. M. Alward ◽

Erin A. Boese ◽

...

Keyword(s):

Genetic Basis ◽

Immunohistochemical Analysis ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Visual Disability ◽

Primary Analysis ◽

Human Donor ◽

Similar Frequency ◽

Pigment Dispersion Syndrome ◽

Rare Mutations

Abstract 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.

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