Acute Posterior Multifocal Placoid Pigment Epitheliopathy on Optical Coherence Tomography Angiography

Abstract

Purpose

To investigate choroidal involvement in acute posterior multifocal placoid pigment epitheliopathy (APMPPE).

Methods

A retrospective observational case series using multimodal imaging including optical coherence tomography angiography (OCTA).

Results

Five patients with APMPPE were included. In most acute lesions, OCTA revealed outer retinal and retinal pigment epithelium (RPE) hyper-reflective lesions with attenuated OCT signal in the underlying choroid, but careful examination allowed us to identify a single lesion with decreased choriocapillaris flow outside the signal attenuation. OCTA obtained after healing of lesions revealed areas of hypo-intense circular flow voids clustered in groups surrounded by either iso- or hyper-intense signal background. Point-by-point evaluation revealed these flow voids did not correspond to areas of RPE thickening or focal pigmentary changes. Larger hypo-intense lesions were observed and did correlate with pigmentary changes.

Conclusions

Our case series demonstrates choriocapillaris flow abnormalities in acute APMPPE extending beyond the OCT lesions, and distinct residual vascular abnormalities in healed APMPPE lesions on OCTA. Our findings support a primary ischemic insult to the photoreceptors and RPE, but choriocapillaris flow abnormalities could be secondary to (OCT invisible) retinal and RPE involvement. The lack of understanding of the etiology along with the inability to visualize most of the choroid in acute lesions preclude definite conclusions about the true pathogenesis of APMPPE.