Anti-VEGF treatment for choroidal neovascularization complicating pattern dystrophy-like deposit associated with pseudoxanthoma elasticum
To evaluate the efficacy of intravitreal anti-VEGF injections in choroidal neovascularization (CNV) related to pattern dystrophy-like deposit in pseudoxanthoma elasticum (PXE).
One-year prospective, interventional study. Nine eyes were recruited in the ophthalmology departments of San Raffaele University and University of Barcelona. Each patient underwent best corrected visual acuity (BCVA) measurement on ETDRS chart, slit-lamp biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). The protocol included a first anti-VEGF injection, followed by monthly evaluations with re-treatments based on new funduscopic hemorrhages, fluid on OCT or leakage on FA and/or ICGA. Primary outcome measures were the mean BCVA changes. Secondary outcomes included central macular thickness (CMT) variations and the number of injections needed.
At month 12, mean BCVA significantly improved from 20/45 to 20/35 Snellen equivalent, with 3 eyes gaining at least 3 ETDRS lines. Mean CMT decreased from 297 ± 22 to 262 ± 13 μm, after 5.5 ± 4.0 injections. No leakage was observed at the end of follow-up.
Intravitreal anti-VEGF injections represent an effective treatment for CNV related to pattern dystrophy-like deposit in PXE, with an improvement of BCVA and CMT. Mean injection number is in line with other studies performed in CNV secondary to angioid streaks.
KeywordsChoroidal neovascularization CNV Pattern dystrophy Pseudoxanthoma elasticum PXE Anti-VEGF Multimodal imaging
Compliance with ethical standards
Conflict of interest
Battaglia Parodi M has the following financial disclosures: Bausch & Lomb Inc. (C). Bandello F has the following financial disclosures: Alcon (C), Allergan Plc.(C), Famila-Thea (C), Bayer Schering Pharma AG (C), Bausch & Lomb (C), Hoffmann-La-Roche (C), Novartis (C), Sanofi-Aventis (C), and Carl Zeiss AG (C). FR, AM, AA, and MVC have no financial disclosures. Llorenç V has the following financial disclosures: Abbvie (G, C), Allergan(C), UCB(C). Adan A has the following financial disclosures: Abbvie (G, C), Allergan (G, C), UCB(C).
The research was approved by the local Institutional Review Board and adhered to the tenets of the Declaration of Helsinki.
Informed consent was obtained from all individual participants included in the study.
- 22.Vaclavik V, Tran HV, Gaillard MC, Schorderet DF, Munier FL (2012) Pattern dystrophy with high intrafamilial variability associated with Y141C mutation in the peripherin/RDS gene and successful treatment of subfoveal CNV related to multifocal pattern type with anti-VEGF (ranibizumab) intravitreal injections. Retina 32:1942–1949CrossRefGoogle Scholar