Exfoliation and pigmentary glaucoma – overlap syndrome

Abstract

Introduction: Concomitant signs, characteristic for both pigmentary dispersion syndrome (PDS) and exfoliation syndrome (XFS) can cause sudden IOP spike, and lead to optic nerve damage progression and associated visual field (VF) loss. This development often remains undetected. Purpose: To describe the characteristic signs of XFS and pigmentary glaucoma (PG), and to discuss the specific management of overlap syndrome. Methods: A retrospective analysis of 40 consecutive patients diagnosed with overlap syndrome for a period of 18 months and follow up period of 9 to 18 months. Accumulation of abnormal material on lens capsule and pupil, iris transillumination defects, Krukenberg’s spindle, trabecular pigmentation, increased IOP and more difficult control of IOP are main features. Treatment: topical medications, laser peripheral iridotomy (LPI), and surgical - trabeculectomy (TE) or ExPress implant. Results: Most patients had typical signs of PDS and XFS, with XFS predominating over PDS. Patients with overlap syndrome were 50-65 years of age. Glaucoma progression was registered in all overlapping forms. In 25 (62%) patients we achieved good pressure lowering effect with medication and LPI. At lack of IOP control 15 patients underwent surgery - TE (9) and ExPress implantation (6). In 3 patients with TE additional needling with 5-FU was necessary. In 2 patients with ExPress transient posterior pole edema was registered. As a result of our treatment approach, no progression of glaucoma damage was observed. Visual impairment was due to late referral with very high levels of IOP and advanced VF defects. Conclusion: Awareness of sequential appearance and overlap of those two forms of glaucoma is of decisive importance for appropriate management. The alarming signs of unexcpected loss of IOP control, rapid progression of glaucomatous optic nerve changes and VF loss indicate of need for more aggressive treatment.