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: 4.02). 06/2011; 152(4):695-703. DOI: 10.1016/j.ajo.2011.03.022
Source: PubMed

ABSTRACT 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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    ABSTRACT: We describe the long-term effectiveness and tolerability of intravitreal vascular endothelial growth factor inhibitor ranibizumab in a patient with pseudoxanthoma elasticum with bilateral macular choroidal neovascularization secondary to angioid streaks. A 54-year-old Caucasian man with history of heart disease presented with visual loss in his right eye. An examination revealed choroidal neovascularization and reduced visual acuity, while no abnormalities were seen in his left eye. He was diagnosed with angioid streaks associated with pseudoxanthoma elasticum. Off-label treatment with intravitreal bevacizumab once a month initiated in December 2007 was discontinued after 3 months due to lack of efficacy. In September 2008, the patient reported reduced visual acuity in his left eye and an examination revealed changes. Left eye treatment was initiated in October 2008 with a loading dose (three consecutive monthly intravitreal injections of ranibizumab 0.5mg/50muL) followed by 0.5mg/50muL followed by treatment as needed until May 2014. After 21 ranibizumab injections, an examination revealed angioid streaks and choroidal neovascularization in both eyes. His right eye showed retinal layer deterioration with outer limiting membrane and photoreceptor inner/outer segment junction involvement. His left eye had a smaller foveal scar, with other areas preserved. Visual acuity was stable in his treated left eye, but had deteriorated in his right eye. Ranibizumab treatment was well tolerated with no adverse events reported. In the present case, an as-needed regimen of ranibizumab after an initial loading dose, achieved maintenance of visual function and was well tolerated over a period of almost 6 years in a patient with pseudoxanthoma elasticum and high cardiovascular risk. As anti-vascular endothelial growth factor agents are associated with increased risk of systemic effects, particularly arterial thromboembolic events, following intravenous administration, the absence of serious thromboembolic or cardiovascular adverse events throughout the 6-year treatment period is particularly encouraging considering our patient's high cardiovascular risk status.
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    ABSTRACT: The objective of this pilot study is to determine the prevalence of pseudoxanthoma elasticum (PXE)-like connective changes in an oral biopsy service and compare it with the estimated prevalence of PXE as well as to the prevalence of the mutated PXE gene ABCC6. This prevalence study utilized 500 oral mucosal biopsy specimens received from the biopsy service of the Oral Pathology Consultants at the Ohio State University. Each specimen was microscopically evaluated using hematoxylin and eosin, Verhoeff-van Gieson and von Kossa stains. A prevalence of 9.8% was identified for PXE-like changes in the connective tissue of oral biopsy specimens submitted to this service. The overall prevalence of PXE-like connective tissue changes found in routine oral mucosal biopsy specimens (9.8%) was much higher than either the suspected prevalence of PXE (0.001%-0.004%) or the estimated prevalence of the mutated gene ABCC6 (0.625%-1.25%). Copyright © 2015 Elsevier Inc. All rights reserved.
    Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 12/2014; DOI:10.1016/j.oooo.2014.12.003 · 1.46 Impact Factor
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