Acute Exudative Polymorphous Paraneoplastic Vitelliform Maculopathy Managed With Intravitreal Aflibercept

The authors report on two patients with bilateral acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) treated with intravitreal aflibercept (Eylea; Regeneron, Tarry-town, NY [marketed locally in Turkey by Bayer]). Underlying malignancy had been treated in each case, including breast carcinoma in one case and colon carcinoma in the other case. A macular vitelliform lesion was noted in the right eye and atrophic retinal pigment epithelial (RPE) changes were noted in the left eye of each case. Enhanced depth imaging optical coherence tomography (EDI-OCT) of the vitelliform lesion showed sensorineural retinal detachment, highly reflective subretinal material, ellipsoid loss in the right eye, and photoreceptor loss in both eyes of each patient. In both cases, the right eye with a vitelliform macular lesion was treated with intravitreal aflibercept (2.0 mg/0.05 mL) at monthly intervals for the first three injections and at bimonthly intervals for the following injections. Case 1 received a total of six injections and visual acuity (VA) increased from 20/70 to 20/50 at 10 months' follow-up. EDI-OCT showed slight gradual resolution of subretinal vitelliform material. Case 2 received three injections and VA increased from 20/100 to 20/40 at 4 months' follow-up with a decrease in the subretinal vitelliform deposit and intraretinal edema on EDI-OCT. Intravitreal aflibercept may control progression of APPVME in newly diagnosed cases by decreasing vascular leakage and stabilizing RPE function.