Early-onset Exfoliation Syndrome: A Literature Synthesis

Precis: We conducted a literature review of younger patients with exfoliation syndrome (XFS) in an attempt to identify case similarities and better understand disease etiology. Purpose: XFS that predisposes to secondary glaucoma is a strongly age-related condition. We performed a literature review of XFS and exfoliation glaucoma (XFG) in patients aged younger than 40 years to examine potential common characteristics and gain clues to its etiology. Methods: We conducted a broad literature search with appropriate keywords and manually extracted key demographic and ocular features on younger XFS and XFG cases. Articles that did not provide past ocular history on early-onset XFS/XFG were excluded. Results: We identified 12 cases of XFS and XFG in patients from 13 to 40 years old (8 females; 11 White; 5 from Iran). All had past ocular history remarkable for intraocular surgery for other glaucoma conditions (7 cases), other ocular diseases (3 cases), or ocular trauma (2 cases). Conclusions: All reported early-onset XFS and XFG cases arise in the setting of events that produced a significant disruption of the blood-aqueous barrier. Understanding the metabolic alterations of aqueous humor from such cases could provide clues regarding how exfoliation material forms.