Abstract
Purpose: :
To investigate the long-term effectiveness of intravitreal bevacizumab for treating active choroidal neovascularization (CNV) secondary to pseudoxanthoma elasticum (PXE) in a retrospective case series.
Methods: :
A total of 23 patients (25 eyes) with active CNV secondary to PXE received intravitreal bevacizumab (1.5 mg) and were reviewed in monthly intervals. Further treatments were administered depending on signs of disease activity. Baseline and follow-up examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, fundus autofluorescence (FAF) and digital fundus photography. Areas of atrophy of the retinal pigment epithelium (RPE) and fibrosis were quantified using semi-automated image analysis of FAF images.
Results: :
Mean age of the overall sample was 53 ± 11.7 years and mean visual acuity at baseline 20/80 (LogMAR 0.55, SD 0.48). At last follow up, after an average of 5 ± 5.1 injections over 20 months, visual acuity was 20/50 (LogMAR 0.36, SD 0.37; p = 0.028). Central OCT thickness was reduced from 255.38 microm (SD 50.68 microm) to 212.56 microm (SD 33.92 microm; p = 0.006). The size of the atrophy and fibrosis measured on FAF images increased in both the treated and the fellow eye (p<0.05). Mean visual acuity of patients with early disease stages improved significantly over the treatment course to 20/32 (paired t-test p = 0.030), compared to 20/63 in the subgroup of patients with advanced fundus alterations (paired t-test p = 0.264; comparing both groups p = 0.01).
Conclusions: :
Intravitreal bevacizumab demonstrates long-term effectiveness in preserving function in advanced disease stages and improving function in early disease stages. Best results are achieved when treatment starts the earliest possible. An adverse effect of bevacizumab on RPE vitality in the long run cannot be ruled out.
Keywords: choroid: neovascularization • retinal degenerations: hereditary • vascular endothelial growth factor