Bilateral Idiopathic Multifocal Pigment Epithelial Detachments: A Case Series and Review of Literature

Abstract

Purpose: Description of longitudinal follow-up of 13 patients diagnosed with Idiopathic Multifocal Pigment Epithelial Detachments (IMPED), a rare condition first described by Gass et al., in 2005.

Methods: This retrospective case series included 13 patients in the first to sixth decades of life who presented with multiple bilateral PEDs, confirmed by Optical Coherence Tomography. Ancillary imaging included fundus autofluorescence and angiography to exclude alternative diagnoses such as Central Serous Chorioretinopathy and Polypoidal Choroidovasculopathy. Electrophysiology testing, including full-field electroretinography (FFERG) and electrooculography (EOG), assessed both retinal and pigment epithelium function. Visual acuity (VA) was documented over a mean follow-up of 6.5 years (range: 0.1-26.4 years).

Results: All patients demonstrated stable VA during follow-up. Electrophysiology was normal in tested patients (FFERG: 9/13, EOG: 7/13). Genetic testing (10/13) was non-diagnostic. Two patients (including a patient previously receiving anti-VEGF without exudation) exhibited outer retinal atrophy. One patient developed a choroidal neovascularization (CNV) during follow-up, which resolved with intravitreal aflibercept.

Conclusion: IMPED should remain a diagnosis of exclusion, confirmed after sufficient follow-up demonstrating no progression. While our study implies a generally favorable prognosis, continuous monitoring is necessary to assess long-term risks, including potential complications such as the development of CNV and/or atrophy.

Keywords: Age-Related Macular Degeneration; Case Series; Drusen; Idiopathic Multifocal Pigment Epithelial Detachments; Imaging.