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Pigment dispersion syndrome and pigmentary glaucoma: a review and update

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A Correction to this article was published on 06 June 2019

This article has been updated

Abstract

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.

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Change history

  • 06 June 2019

    In the original publication, introduction section under Abstract was published incorrectly. The correct version is given below.

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Authors and Affiliations

  1. Ophthalmology Unit, St. Andrea Hospital, Faculty of Medicine and Psychology, NESMOS Department, University of Rome “Sapienza”, Rome, Italy

    Gianluca Scuderi, Maria Teresa Contestabile & Vito Fenicia

  2. Ophthalmology Unit, Azienda Ospedaliera Universitaria Policlinico Umberto I, University of Rome “Sapienza”, Viale del Policlinico 155, Rome, 00161, Italy

    Luca Scuderi & Aloisa Librando

  3. Pathology Centre-Histopathology, Queen Alexandra Hospital, School of Pharmacy and Biomedical Science, University of Portsmouth, Portsmouth, UK

    Siavash Rahimi

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  1. Gianluca Scuderi
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Correspondence to Luca Scuderi.

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The original version of this article was revised: Introduction section under Abstract was published incorrectly. The correct version is updated in the article.

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Scuderi, G., Contestabile, M.T., Scuderi, L. et al. Pigment dispersion syndrome and pigmentary glaucoma: a review and update. Int Ophthalmol 39, 1651–1662 (2019). https://doi.org/10.1007/s10792-018-0938-7

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