APMPPE and Placoid Variant Diseases Masquerading as Age-Related Macular Degeneration in the Elderly: A Case Series.

Abstract

To report eight cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or persistent placoid maculopathy (PPM) initially masquerading as age-related macular degeneration (AMD) in elderly individuals. APMPPE or PPM eyes in patients above age 55 years with macular RPE disruption including drusenoid lesions on macular examination and/or with multimodal imaging were included. At least one method of multimodal imaging including fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT) and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization (MNV). Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular AMD. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and ICGA, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity (ASHH) was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. MNV requiring anti-VEGF injection therapy complicated 3 of 8 cases. Both APMPPE and PPM may develop in elderly individuals and may masquerade as AMD on presentation. Multimodal imaging including FA, ICGA, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis, and to detect MNV which frequently complicates APMPPE and PPM. In these patients, serial anti-VEGF intravitreal injections are essential in treating MNV and in preventing significant vision loss.