Pigment dispersion syndrome and pigmentary glaucoma: a review and update

Scuderi, Gianluca; Contestabile, Maria Teresa; Scuderi, Luca; Librando, Aloisa; Fenicia, Vito; Rahimi, Siavash

doi:10.1007/s10792-018-0938-7

Cited by 47 publications

(43 citation statements)

References 94 publications

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“…The ocular pathology detected here is reminiscent of pigment dispersion syndrome (PDS), a disease in humans, and in mouse models, where pigmented cells detach from the iris and accumulate at the iridocorneal angle, leading to glaucoma in 35 to 50% of patients [45][46][47][48][49] . We therefore propose that loss of GCase activity results in elevated GluSph lipids in the eye which may then lead to detachment of pigmented cells from the iris, accumulation of the cells in the iridocorneal angle.…”

Section: Discussionmentioning

confidence: 99%

Ocular phenotypes in a mouse model of impaired glucocerebrosidase activity

Weber¹,

Min²,

Truong³

et al. 2021

Sci Rep

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Mutations in the GBA1 gene encoding glucocerebrosidase (GCase) are linked to Gaucher (GD) and Parkinson’s Disease (PD). Since some GD and PD patients develop ocular phenotypes, we determined whether ocular phenotypes might result from impaired GCase activity and the corresponding accumulation of glucosylceramide (GluCer) and glucosylsphingosine (GluSph) in the Gba1D409V/D409V knock-in (Gba KI/KI; “KI”) mouse. Gba KI mice developed age-dependent pupil dilation deficits to an anti-muscarinic agent; histologically, the iris covered the anterior part of the lens with adhesions between the iris and the anterior surface of the lens (posterior synechia). This may prevent pupil dilation in general, beyond an un-responsiveness of the iris to anti-muscarinics. Gba KI mice displayed atrophy and pigment dispersion of the iris, and occlusion of the iridocorneal angle by pigment-laden cells, reminiscent of secondary open angle glaucoma. Gba KI mice showed progressive thinning of the retina consistent with retinal degeneration. GluSph levels were increased in the anterior and posterior segments of the eye, suggesting that accumulation of lipids in the eye may contribute to degeneration in this compartment. We conclude that the Gba KI model provides robust and reproducible eye phenotypes which may be used to test for efficacy and establish biomarkers for GBA1-related therapies.

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Section: Discussionmentioning

confidence: 99%

Ocular phenotypes in a mouse model of impaired glucocerebrosidase activity

Weber¹,

Min²,

Truong³

et al. 2021

Sci Rep

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“…It should be noted that the Sampaolesi line is not pathognomonic for PES and is also present in pigmentary dispersion syndrome (PDS). However, the prevalence of PDS is much lower than PES, representing only 1–1.5% of open-angle glaucoma cases in Caucasian countries, and therefore the Sampaolesi line is mostly met as part of PES [17].…”

Section: Discussionmentioning

confidence: 99%

Diagnosis of Pseudoexfoliation Syndrome in Pseudophakic Patients

et al. 2020

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Objective: The aim of this study was to evaluate the sensitivity and specificity of pseudoexfoliation syndrome diagnosis in pseudophakic patients and potential means of improving it. Methods: This prospective, nonrandomized study comprised 41 consecutive patients (41 eyes) scheduled for cataract surgery at a tertiary medical center during 2016 and 2017. Preoperatively, all patients underwent a detailed slit-lamp examination, including gonioscopic assessment of the iridocorneal angle. The examination was performed by a glaucoma specialist who completed an assessment form documenting the presence/absence of clinical signs of pseudoexfoliation syndrome. It was repeated 1–2 weeks postoperatively by a second, masked, glaucoma specialist. Results: Sixteen patients (39.0%) were diagnosed with pseudoexfoliation syndrome preoperatively. The diagnosis was confirmed postoperatively in 11/16 patients (68.8% sensitivity) and in an additional patient not diagnosed preoperatively (96% specificity). The ability to diagnose pseudoexfoliation syndrome postoperatively was significantly worse than preoperatively (Z = 12.161, p < 0.0001). Pupillary border deposits (75% of cases) and the Sampaolesi line (83.3%) were the cornerstones of the postoperative diagnosis; anterior capsular deposits were evident in only 41.6% of cases diagnosed postoperatively (31.3% of the originally diagnosed cases). Conclusions: Underdiagnosis of pseudoexfoliation syndrome is common in pseudophakic patients and may have significant implications for future management. Careful attention to pupillary border anatomy and meticulous gonioscopic assessment of the iridocorneal angle are essential for accurate diagnosis. Preoperative documentation of pseudoexfoliation syndrome could help prevent this diagnostic pitfall.

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“…Iris transillumination also occurs in pigment dispersion syndrome [26]. A concave iris results in iridolenticular contact and continued pigment dispersion in the anterior chamber, with deposition of pigment in the trabecular meshwork and corneal endothelium (Krukenbergʼs spindle).…”

Section: Differential Diagnosismentioning

confidence: 99%

“…BADI and BAIT are now considered to be part of the same spectrum as indicated by the author who first described both syndromes. (I. Tugal-Tutkun, personal communication) Iris transillumination also occurs in pigment dispersion syndrome [26]. A concave iris results in iridolenticular contact and continued pigment dispersion in the anterior chamber, with deposition of pigment in the trabecular meshwork and corneal endothelium (Krukenbergʼs spindle).…”

Section: Differential Diagnosismentioning

confidence: 99%

Two Rare Causes of Anterior Pseudo-Uveitis Affecting the Iris: Sequelae of Bilateral Acute Iris Transillumination (BAIT) Syndrome and Iridocorneal Endothelial (ICE) Syndrome

Papasavvas¹,

Lehoang

Herbort³

2021

Klin Monbl Augenheilkd

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Axenfeld-Rieger syndrome BADI bilateral acute depigmentation of the iris BAIT bilateral acute iris transillumination ICE iridocorneal endothelial IOP intraocular pressure OD oculus dexter, right eye OS oculus sinister, left eye OU oculus uterque, both eyes PVRL primary vitreoretinal lymphoma RNFL retinal nerve-fibre layer SD-OCT spectral domain optical coherence tomography UBM ultrasound biomicroscopy

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Pigment dispersion syndrome and pigmentary glaucoma: a review and update

Cited by 47 publications

References 94 publications

Ocular phenotypes in a mouse model of impaired glucocerebrosidase activity

Ocular phenotypes in a mouse model of impaired glucocerebrosidase activity

Diagnosis of Pseudoexfoliation Syndrome in Pseudophakic Patients

Two Rare Causes of Anterior Pseudo-Uveitis Affecting the Iris: Sequelae of Bilateral Acute Iris Transillumination (BAIT) Syndrome and Iridocorneal Endothelial (ICE) Syndrome

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