Anti-VEGF Agents for the Treatment of Choroidal Neovascularization in Pseudoxanthoma Elasticum

Introduction: Pseudoxanthoma elasticum (PXE) is an inherited disease that affects multiple organs, including the skin, eyes, and cardiovascular system. Visual loss occurs at an early age secondary to choroidal neovascularization (CNV) resulting from angioid streaks (AS). Bevacizumab and ranibizumab, are anti-VEGF monoclonal antibodies, that have been shown to be successful treatment options for CNV secondary to neovascular age-related macular degeneration and other diseases, such as PXE. The current literature regarding the use of Anti-VEGF agents to treat CNV secondary to AS in PXE is limited. Herein, we report a small case series to show the efficacy of intravitreal bevacizumab and ranibizumab for treatment of CNV secondary to AS in PXE. Clinical Description: Two patients presented at 48 and 51 years old, respectively complaining of blurry vision. The baseline visual acuity ranged from 20/25-20/400 in each of the four affected eyes. Both patients were found to have CNV secondary to AS due to PXE. Each eye was followed for a mean of 36.8 months and required a mean of 9 bevacizumab and 0.75 ranibizumab injections. Resolution of pathologic edema was demonstrated on OCT in each eye. The mean visual acuity deviation was 0.66 below baseline. No significant adverse events were noted. Discussion: Intravitreal anti-VEGF agents are effective long term treatment options for CNV secondary to AS in PXE patients. Our small case series supports the current findings in the literature and is the only case series, to our knowledge, that has documented follow up exceeding 3 years.