Monthly Ranibizumab for Choroidal Neovascularizations Secondary to Angioid Streaks in Pseudoxanthoma Elasticum: A One-Year Prospective Study

Department of Ophthalmology, University of Bonn, Germany.
American Journal of Ophthalmology (Impact Factor: 3.87). 06/2011; 152(4):695-703. DOI: 10.1016/j.ajo.2011.03.022
Source: PubMed


To evaluate the efficacy and safety of monthly intravitreal ranibizumab for the treatment of choroidal neovascularizations (CNV) secondary to angioid streaks (AS) in pseudoxanthoma elasticum (PXE).
Twelve-month prospective, open-label, uncontrolled, nonrandomized interventional clinical trial.
In 7 patients, 1 eye with an active CNV was injected with 0.5 mg ranibizumab monthly over 1 year. Distance and reading visual acuity, reading speed, angiographic findings, and central retinal thickness (CRT) on optical coherence tomography were assessed at each visit. Central retinal light increment sensitivity (LIS) was assessed by microperimetry at baseline, at 6 months, and 3 to 4 months after the last injection.
Best-corrected visual acuity increased significantly from baseline to month 12 (20/63 or 61 ETDRS letters to 20/32 or 73 ETDRS letters; P = .012). The effect was maintained 3 months later (61 ETDRS letters to 72 ETDRS letters; P = .055). Reading acuity and speed could be maintained throughout the study. Central LIS improved (6.6 dB, SD ± 5.9 at baseline to 7.4 dB, SD ± 6.2 at last follow-up; P < .001). Leakage from active CNVs subsided. Mean change in CRT from baseline to month 12 and 15 was -86 μm (P = .074) and -65 μm (P = .182), respectively. No serious adverse events occurred.
Efficacy outcomes indicate a beneficial therapeutic effect of intravitreal ranibizumab on central visual function including retinal LIS. Both the functional and morphologic response based on angiographic and OCT findings to ranibizumab treatment implicate an important pathophysiological role of vascular endothelial growth factor in CNVs secondary to AS in PXE. Intravitreal ranibizumab appears to be a safe and efficacious treatment in these patients.

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    • "Reported treatment for CNV, as a complication of AS that is unrelated to ocular injury, include photodynamic therapy9–11 and intravitreal injection of the anti-VEGF antibodies bevacizumab12–16 and ranibizumab.17,18 Photodynamic therapy has a short-term effect and may help prevent visual impairment9,10 but has also been reported to cause enlargement of scarring and loss of vision over the long term.11 "
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    ABSTRACT: To report on the five-year follow-up of ranibizumab therapy for choroidal neovascularization (CNV) secondary to angioid streaks (AS) in pseudoxanthoma elasticum (PXE). A 51-year-old patient with PXE presenting with macular CNV secondary to AS was treated with a series of intravitreal ranibizumab (0.5 mg) injections and followed for five years with fundoscopy, optical coherence tomography (OCT), and fluorescein angiography (FA). Fluorescein leakage resolved and OCT evidence of subretinal and intraretinal fluid disappeared one year after presentation following an initial 12 injections. There was mild recurrent neovascular activity on two occasions resulting in two injections in the four years subsequent to resolution. Though peripapillary scar formation occurred, the fovea was preserved with 20/20 visual acuity in the affected eye at five years. This case provides further evidence for the long-term effectiveness of ranibizumab in the treatment of CNV secondary to AS in PXE. Though multiple initial injections were required to control the disease, once stabilization of the CNV was achieved, recurrent neovascular activity was minimal.
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