UNASSIGNED: We report a case of bilateral pigment dispersion syndrome after 13 years of uncomplicated implantable collamer lens (ICL) surgery.
UNASSIGNED: 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.
UNASSIGNED: 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 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.
UNASSIGNED: 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.

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.

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.

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.

UNASSIGNED: 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].
UNASSIGNED: 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.
UNASSIGNED: 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.

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.

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.

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.

UNASSIGNED: Pigment dispersion syndrome (PDS) is characterized by dispersion of pigment in the anterior chamber structures and can present with deposits on the central corneal endothelium or Krukenberg spindle, iris trans-illumination spoke-like defects, and increased pigmentation in the iridocorneal angle. It is more common in myopic patients with a predominance in young males in the third to fifth decade of life that affects about 1-2% of the population. PDS is a risk factor and can give lead to a rise in intraocular pressure (IOP) and secondary glaucoma. Pigmentary glaucoma (PG) can develop from PDS in the presence of elevated IOP coupled with glaucomatous optic neuropathy, retinal nerve fiber thinning, and/or visual field defects. PDS and PG have the same clinical features, representing different levels of severity on the same clinical spectrum.
UNASSIGNED: Early diagnosis, appropriate management, and follow-up of patients with PDS are important to prevent vision deterioration or blindness due to glaucomatous optic neuropathy.