Pre-perimetric Open Angle Glaucoma with Young Age of Onset: Natural Clinical Course and Risk Factors for Progression

Abstract

To investigate the natural clinical course of more than 5 years and the risk factors of progression in patients with pre-perimetric open angle glaucoma (OAG) of "young age of onset (under age 40)" without treatment. Retrospective observational case series. Optic disc photography, red-free retinal nerve fiber layer (RNFL) photography, optical coherence tomography, and visual field (VF) examinations were performed every 6months. Glaucoma progression was defined as structural or functional deterioration. A linear mixed-effects model was used to estimate the rate of structural and functional changes. Kaplan-Meier survival analysis and log-rank testing were used to compare survival experiences, and Cox proportional hazards modeling was performed to identify risk factors for glaucoma progression. Of the 98 eyes of 98 patients (mean age, 30.6 years old), glaucoma progression was detected in 42 eyes (42.9%). The rate of average RNFL thickness thinning was-0.46 ± 0.50μm/y, and the mean deviation (MD) change was-0.03 ± 0.13 dB/y. The glaucoma progression probability at 5 years was 39% by structural criteria and 5% by functional criteria. Older age at diagnosis (P= .004), presence of temporal raphe sign (horizontal straight line on macular ganglion cell-inner plexiform layer thickness map) (P= .011), lamina pore visibility (P= .034), and greater pattern standard deviation (P= .005) were significant factors for glaucoma progression. In untreated pre-perimetric OAG patients with a "young age of onset" condition, the estimated MD slope for the disease course of more than 5 years was-0.03 dB/y, and the average RNFL thinning rate was-0.46μm/y. The predictors for progression were structural parameters of temporal raphe sign, lamina pore visibility, and functional parameter of pattern standard deviation.