Individuals with angioid streak-related macular neovascularization (AS-MNV) can achieve strong, long-term visual acuity (VA) outcomes after undergoing treatment with anti-vascular endothelial growth factor (anti-VEGF) injections, according to a report published in Ophthalmology Retina. The research shows that a higher number of injections for these patients better preserves VA, suggesting the need for more proactive treatment.
Investigators examined medical records for 84 eyes of 66 patients with AS-MNV receiving anti-VEGF therapy at centers in Brazil, India, Israel, Italy, Switzerland, or the UK from January 2009 to March 2022. The first intravitreal injection served as baseline, with longitudinal data collected during a mean follow-up time of 67.7±48.5 months. Participants’ average age was 55.7±13.8 years and 33% displayed pseudoxanthoma elasticum (PXE). At baseline, 26% of eyes had moderate to severe visual impairment (MSVI) — VA from 6/18 to 3/60 — and 4% exhibited vision from 3/60 to counting fingers.
Overall, VA improved through 6 months, and declined after 12 months, with average loss at approximately +0.04 logMAR each year. Patients with pattern dystrophy-like features experienced less rapid visual loss (P =.04), and decreased fibrosis (P =.06). Those receiving 13 or more injections were also less likely to develop MSVI (HR=0.45, 95% CI 0.19 to 0.94, P =.03). “Our findings emphasize the need to reflect upon the choice of treatment regimen, as a reactive regimen could not effectively control the disease progression,” the researchers report.
Macular fibrosis occurred at a rate of 24.1(95% CI 17.5 to 32.3) per 100-eye-years, and atrophy at 14.3 (95% CI 10.1 to 19.6) per 100-eye-years. Fibrosis risk was increased with younger age, and a larger region of MNV, as well as neovascularization situated underneath or alongside the fovea. Younger individuals may be more prone to fibrosis due to cellular reactions that prompt “fibrotic scarring.” Relationships also emerged between atrophy and baseline subretinal hyperreflective material or greater MNV size. Conversely, subretinal fluid protected against atrophy (P =.03).
Participants who presented with decreased central macular thickness showed more rapid loss of acuity (P =.001), as did those with non-naïve eyes (P =.007). Because patients with pattern dystrophy had slower visual decline, investigators speculate this group better responded to anti-VEGFs because the MNV lesions were not as “aggressive,” or they coincided with less occurrence of intraretinal fluid.
A review of other papers revealed those with higher starting VA better maintained eyesight. The current data agrees, but those with higher presenting acuity may have also shown a steeper decline, attributed by researchers to factors such as intractable MNV progression, fibrosis, or acute retinopathy. Prior research has proposed atrophy may be prompted by anti-VEGF drugs leaking through breaks in Bruch’s membrane to the choroid, but in this analysis there was no correlation between total injections and atrophy risk.
“The short-term effectiveness of intravitreal anti-VEGF agents in improving or maintaining visual acuity in eyes with AS-related MNV is undisputed. On the other hand, only a few studies provided data over 36 months or longer and a gap of knowledge exists in quantifying the long-term visual burden of these patients,” the researchers report. “A higher number of injections yielded higher chances of maintaining good visual acuity over time. In other words, higher treatment intensity was associated with better long-term outcomes, as found in neovascular age-related macular degeneration.”
Limitations of this study include possible selection bias created by worse cases typically being referred to tertiary centers, lack of a group on fixed regimen, potential grading differences of retinal specialists, and no cohort-wide PXE genetic testing. Strengths involved a large sample size, long follow-up, and frailty survival model to adjust for inter-site heterogeneity.
Cicinelli MV, Torrioli E, La Franca L, et al. Incidence and risk factors of visual impairment in patients with angioid streaks and macular neovascularization. Ophthalmol Retina. Published online on December 8, 2022. doi:10.1016/j.oret.2022.12.002