Pigment dispersion syndrome and pigmentary glaucoma: a review and update

• Published in: International ophthalmology 2019-07, Vol.39 (7), p.1651-1662
• Main Authors: Scuderi, Gianluca, Contestabile, Maria Teresa, Scuderi, Luca, Librando, Aloisa, Fenicia, Vito, Rahimi, Siavash
• Format: Article
• Language: English
• Subjects: Anterior Eye Segment - diagnostic imaging; Aqueous humour; Concavity; Cornea; Diagnosis; Differential diagnosis; Disease control; Dispersion; Endothelium; Endothelium, Corneal - pathology; Glaucoma; Glaucoma, Open-Angle - diagnosis; Glaucoma, Open-Angle - physiopathology; Glaucoma, Open-Angle - surgery; Humans; Hypertension; Intraocular pressure; Intraocular Pressure - physiology; Iris; Laser Therapy - methods; Management; Medicine; Medicine & Public Health; Ophthalmologic Surgical Procedures - methods; Ophthalmology; Optic neuropathy; Optical Coherence Tomography; Pathogenesis; Perspectives or Expert Opinions; Pigmentation; Prognosis; Tomography, Optical Coherence; Ultrasound
• ISSN: 0165-5701; 1573-2630
• DOI: 10.1007/s10792-018-0938-7

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.