Successful treatment of severe peripapillary pachychoroid syndrome with anti-vascular endothelial growth factor therapy.

PURPOSE:To describe two cases of severe peripapillary pachychoroid syndrome successfully managed with monthly intravitreal aflibercept therapy. METHODS:Medical and imaging records were retrospectively reviewed. Patients were imaged with ultra-widefield fluorescein, indocyanine green angiography, and fundus autofluorescence. Spectral-domain optical coherence tomography was performed to evaluate macular edema and choroidal thickness. Optical coherence tomography angiography excluded macular neovascularization. RESULTS:This report summarizes two cases of peripapillary pachychoroid syndrome complicated by very severe bilateral macular edema. In all four eyes, the diffuse intraretinal and subretinal fluid remarkably improved or completely resolved after monthly intravitreal aflibercept injections with commensurate improvement of visual acuity. Multimodal imaging documented the significant improvement of fluid and the reduction in choroidal thickening in response to anti-vascular endothelial growth factor therapy in each case. CONCLUSION:Severe cases of peripapillary pachychoroid syndrome associated with vision loss can be successfully treated with intravitreal aflibercept therapy.