Background: Choroidal neovascularization (CNV) in adults is most commonly associated with neovascular age-related macular degeneration (AMD) and pathologic myopia. Though less common, CNV can also develop from other conditions such as uveitis, central serous chorioretinopathy, angioid streaks, intraocular tumors, hereditary chorioretinal dystrophies, or can be idiopathic in origin. If left untreated, CNV may cause visual loss because of exudation of intraretinal or subretinal fluid, retinal or subretinal hemorrhage, or fibrosis involving the macula. It is well known that one of the main drivers of angiogenesis in CNV development is vascular endothelial growth factor (VEGF) and therefore inhibitors of VEGF might be an effective treatment for CNV. Methods: The goal of this review is to provide an overview and summary in the use of pharmacotherapy especially anti-VEGF therapy, in the treatment of CNV due to uncommon causes. Results: Results from uncontrolled case series and controlled clinical trials have reported good efficacy and safety in using anti-VEGF agents including bevacizumab, ranibizumab, aflibercept and ziv-aflibercept in the treatment of CNV due to uncommon causes. Anti-VEGF has also been used in combination with verteporfin PDT and anti-inflammatory agents for treating CNV of various causes. Conclusion: Pharmacotherapy with anti-VEGF agents is an effective treatment option for CNV due to uncommon etiologies.
Polypoidal choroidal vasculopathy (PCV) is a retinal disorder commonly found in Asians presenting as neovascular age-related macular degeneration and is characterized by serous macular detachment, serous or hemorrhagic pigment epithelial detachment, subretinal hemorrhage, and occasionally visible orange-red subretinal nodular lesions. PCV is diagnosed using indocyanine green angiography (ICGA), and the lesions appear as polypoidal aneurysmal vascular lesions with or without abnormal branching vascular network. Although ICGA remains the gold standard for the diagnosis of PCV, various imaging modalities have also facilitated the diagnosis and monitoring of PCV. Recent advances in imaging technology including the use of high resolution spectral domain optical coherence tomography (OCT) and OCT angiography have provided new insights on the pathogenesis of PCV, suggesting a link between PCV and pachychoroid spectrum of macular disorders. With the evolving understanding on the pathogenesis and clinical characteristics of PCV, different therapeutic options have been proposed. These include intravitreal anti-vascular endothelial growth factor (anti-VEGF) monotherapy, combination therapy with anti-VEGF and verteporfin photodynamic therapy, and thermal laser photocoagulation. In recent years, major multi-center randomized clinical trials such as EVEREST, EVEREST II, and PLANET studies have been conducted to compare the efficacy and safety of various treatment options for PCV. This review aims to summarize the results of recent literature, clinical trials and studies to provide an update on the management options of PCV. An overall management strategy for PCV will also be proposed.
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