Pigment Dispersion Syndrome and Pigmentary Glaucoma in an Emmetropic Young Male

Introduction: Pigment dispersion Syndrome (PDS) is a disorder with an onset in mid–twenties. There occurs a disruption of the iris pigment epithelium and deposition of pigment granules throughout the anterior segment. The incidence of PDS is 4-8/ 100,000. This condition is more commonly seen In Caucasians and is considered to be rare in Indians. Case: A 33-year-old male presented with the complaint of headache for three months. He had normal vision in both eyes with visual acuity of 6/6. Observation: Krukenberg’s spindle, a classic sign of pigment dispersion syndrome was evident on slit-lamp examination over the posterior corneal surface. Gonioscopy revealed a heavy and uniformly pigmented trabecular meshwork. OCT (Optical Coherence Tomography) demonstrated a characteristic iris configuration in the form of a mid-peripheral posterior bowing of the iris .Retinal nerve fibre layer analysis done on OCT revealed glaucomatous thinning in the right eye and a more advanced defect in the left eye. A visual field examination revealed the field to be outside normal limits in both the eyes pointing towards a diagnosis of pigment dispersion glaucoma. Conclusion: The purpose of presenting this case is to caution the clinicians to carefully examine young emmetropes who present with Krukenberg’s spindle as it could be associated with PDS. Patients with Krukenberg’s spindle and without elevated lOP are often treated as normal. These patients must be cautioned regarding possible future consequences of the disease and counseled regarding the hereditary nature of the syndrome.

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Use of automatic refractometer infrared images to screen pigment dispersion syndrome: a cross-sectional observational study from a preliminary hypothesis

Medical Hypothesis, Discovery & Innovation in Optometry ◽

10.51329/mehdioptometry104 ◽

2020 ◽

Vol 1 (1) ◽

pp. 25-28

Author(s):

Paolo Brusini ◽

Veronica Papa

Keyword(s):

Observational Study ◽

Pigment Epithelium ◽

Anterior Segment ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Infrared Images ◽

Cross Sectional ◽

Pigment Dispersion Syndrome ◽

Automatic Refractometer ◽

Iris Pigment Epithelium

Background: This study was performed to evaluate the use of anterior segment images, obtained with an automatic refractometer, to identify early defects of the iris pigment epithelium in patients with pigment dispersion syndrome (PDS) or pigmentary glaucoma (PG) without observable alterations at the slit lamp. Methods: In this cross-sectional observational study, carried out from January 2018 to December 2019, in Policlinico Citta di Udine Health Center, Udine, Italy, we observed anterior segment infrared images of 1700 subjects who were undergoing routine ophthalmological examination using an automatic refractometer. We selected infrared images of subjects who fulfilled the inclusion and exclusion criteria and looked for a focal defect in the iris pigment epithelium. Results: Twenty patients with focal iris pigment epithelial defect were identified and none of them showed evident signs of PDS. After the necessary explanations, they agreed to have further examinations to verify the possibility of PDS. An in-depth evaluation of ocular structures, including gonioscopy, demonstrated the presence of PDS in all subjects with iris defects. Conclusions: The use of infrared images obtained by an automatic refractometer could provide early and easy identification of PDS in crowded ophthalmology clinics or mass screening programs; yet, more well-designed studies are necessary to confirm these preliminary findings and prove this proposed screening tool.

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Weightlifters - Need to Be Cautious Especially Having Pigment Dispersion Syndrome or Pigmentary Glaucoma

Medical & Clinical Research ◽

10.33140/mcr.02.05.05 ◽

2017 ◽

Vol 2 (5) ◽

Keyword(s):

Visual Acuity ◽

Corneal Thickness ◽

Anterior Segment ◽

Retinal Nerve Fibre Layer ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Slit Lamp ◽

Pigment Dispersion Syndrome ◽

Applanation Tonometer ◽

Nerve Fibre Layer

Weightlifter developed sudden diminution of vision after exercise with visual acuity in right eye (RE) HM and in left eye (LE) CF 1foot, Intraocular pressure (IOP) with Goldmann applanation tonometer (GAT) in both eyes were 34 mmHg with antiglaucoma medications. Slit lamp examination of both eyes showed edematous cornea, concave configuration of iris, semi dilated pupil, sluggishly reacting to light and pigment on corneal endothelium & on clear lens in RE, LE lens was clear. Gonioscopy showed open angle with increased pigmentation. Fundus examination showed cup disc ratio 0.8 in RE & 0.85 in LE. Anterior segment optical coherence tomography (ASOCT) confirmed concave configuration of iris. Patient was treated with antiglaucoma medications & peripheral iridotomy done with Nd: YAG in BE which decreased concave configuration of iris. Visual acuity improved to 6/6 in RE & 6/9 in LE. BE IOP with GAT 14 mmHg, Humphery visual field (HVF) 10-2 showed advanced field defects, Central corneal thickness 598µ, OCT showed retinal nerve fibre layer thickness in RE 75µ & 64µ in LE. Planned for trabeculectomy with MMC subsequently in BE. Conclusion: Weightlifters can need to remain cautious especially having Pigment dispersion syndrome or pigmentary glaucoma & should undergo regular ophthalmic checkup

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PSEUDOEXFOLIATIVE SYNDROME AS A TRIGGER FOR PSEUDOEXFOLIATIVE GLAUCOMA

Актуальні проблеми сучасної медицини Вісник Української медичної стоматологічної академії ◽

10.31718/2077-1096.21.2.48 ◽

2021 ◽

Vol 21 (2) ◽

pp. 48-51

Author(s):

Eddine Jouini Dhia ◽

I. M. Bezkorovaуna

Keyword(s):

Optical Coherence Tomography ◽

Open Angle Glaucoma ◽

Anterior Segment ◽

Retinal Nerve Fibre Layer ◽

Pigment Dispersion ◽

Optical Coherence ◽

Drug Induced ◽

Pseudoexfoliative Glaucoma ◽

Diagnostic Measures ◽

Pigment Dispersion Syndrome

Glaucoma is one of the leading causes of blindness and disability worldwide. Pseudoexfoliative glaucoma is known to be the most aggressive and difficult-to-treat form of secondary glaucoma. According to the literature, pseudoexfoliative glaucoma occurs in 25-30% of cases among all open-angle glaucoma. The recent studies have established that a quarter of patients with pseudoexfoliative glaucoma are blind in one eye, and 7% are blind in both eyes. Risk factors for glaucoma include systemic and ophthalmic factors, one of which is the development of pseudoexfoliative syndrome. In patients with pseudoexfoliative syndrome, glaucoma occurs 20 times more often than in the general population of the same age. The purpose of this research is to study the informativeness of individual phenomena of pseudoexfoliative syndrome (PES) in order to improve early diagnosis and prevention of pseudoexfoliative glaucoma. The study included 114 patients diagnosed as having PES. Exclusion criteria were as follows: age up to 45 years, surgery on the eyeball, a history of disease (iridocyclitis, uveitis, corneal pathology, and traumatic eye disease), pigment dispersion syndrome. Along with the standard set of diagnostic measures, patients underwent biomicroscopy in the conditions of maximum drug-induced mydriasis, gonioscopic examination; the proportion of patients was examined by optical coherence tomography (SD-OCT Topson 3D oct-2000FA Plus (version 7/21/003/0)). The study revealed signs of pseudoexfoliative syndrome (deposition of pseudoexfoliative material on various structures of the anterior segment of the eye) in 100% of cases. Higher values of intraocular pressure (from 29 to 34 mm Hg) were found in the patients with elements of blocking the angle of the anterior chamber (narrowing or closing the angle, increased pigmentation and the presence of draining exfoliative conglomerates). The optical coherence tomography study revealed a thinning of the retinal nerve fibres (33.60 ± 0.43 μm), a significant thinning of retinal nerve fibre layer in patients with pseudoexfoliative syndrome and even more significant thinning in 29 patients diagnosed with pseudoexfoliative glaucoma. Thus, we can conclude knowing the microsymptoms of the disease, mandatory investigating the conditions of the maximum drug-induced mydriasis can contribute to detect pseudoexfoliative syndrome even in the initial stages and thus to prevent the development of pseudoexfoliative glaucoma.

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

Technology and Health Care ◽

10.3233/thc-202501 ◽

2021 ◽

pp. 1-8

Author(s):

Maciej Czepita

Keyword(s):

Open Source Software ◽

Signal To Noise Ratio ◽

Pigment Epithelium ◽

Video Recording ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Image Brightness ◽

Pigment Dispersion Syndrome ◽

Video Images ◽

The Right

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.

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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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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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Clinical Characteristics of Pigment Dispersion Syndrome and Pigmentary Glaucoma Patients: A Cross-sectional Study

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2020/46939.14383 ◽

2020 ◽

Author(s):

Vijay Pratap Singh Tomar ◽

Sandeep Sharma ◽

Rahul Bhardwaj ◽

Sindhuja Singh ◽

Virendra Kumar Pal ◽

...

Keyword(s):

Visual Field ◽

Optic Disc ◽

Clinical Characteristics ◽

Cross Sectional Study ◽

Pigment Dispersion ◽

Pigmentary Glaucoma ◽

Spherical Equivalent ◽

Sectional Study ◽

Cross Sectional ◽

Pigment Dispersion Syndrome

Introduction: Pigmentary Glaucoma (PG) and Pigment Dispersion Syndrome (PDS) are two different spectrums of a single disease. Since the disease is seen in younger population and is rapidly progressive blinding disease, therefore early diagnosis and treatment will reduce the burden of the disease and improve the quality of life. Aim: To evaluate clinical characteristics of PDS and PG patients in eastern part of Uttar Pradesh. Materials and Methods: This was a two years (1st January 2018 to 31st December 2019) hospital‑based retrospective cross‑sectional study of patients who attended the glaucoma clinic. Diagnosis of PDS was made when they had normal optic disc, normal visual field {with or without increased Intra Ocular Pressure (IOP)} and at least two of the following three signs were found clinically: Krukenberg spindle, homogenous moderate‑to‑heavy (≥Spaeth 2+) Trabecular Meshwork (TM) pigmentation, and any degree of zonular and/or lenticular pigment granule dusting. Patients with PDS were diagnosed with PG, if they had two or more of the following findings: initial IOP >21 mmHg, glaucomatous optic nerve damage or glaucomatous visual field loss. Various parameters such as influence of demographics, IOP, Best‑Corrected Visual Acuity (BCVA), Central Corneal Thickness (CCT), Mean Deviation (MD), Visual Field Index (VFI %), spherical equivalent and clinical finding of anterior segment of study patients were analysed. Mean, standard deviation and percentage were calculated using GraphPad Instat version 3.0. Results: Among 40 patients, nine eyes of the six patients had myopia of ‑0.5D or greater, with mean refractive error of ‑3.55±4.72 spherical equivalent. The average baseline IOP in study patients (PDS+PG), was 30.21±11.42 mmHg. Twenty four (60%) patients, either in one or both eyes had glaucoma, secondary to PDS at the initial diagnosis. Thirty three (82.5%) patients had Krukenberg spindles. Homogeneous TM pigmentation was seen in all patients. Typical spoke‑like radial Iris Transillumination Defects (ITDs) were not observed in any of the patients except in one patient, who had isolated short slit‑like trans‑illumination defects in iris crypts. Conclusion: PDS patients with normal optic disc and visual field and raised IOP, should be started prophylactic treatment and needs to be monitored more closely. Thus, the finding of PDS in Indians should alert the ophthalmologist to look for glaucoma during the initial examination.

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