Case Series of Bilateral Idiopathic Multiple Pigment Epithelial Detachments

Abstract

Purpose : Pigment Epithelial Detachments (PEDs), elevations of the Retinal Pigment Epithelium (RPE) above the Bruch's membrane, are a common finding in retinal disease. They appear in Age-Related Macular Degeneration (AMD) and its variants (e.g., Polypoidal Choroidal Vasculopathy – PCV), several disorders of the RPE and other conditions. Here we describe the findings in three patients (2 males, 1 female) with presumed Idiopathic Multifocal Pigment Epithelium Detachments (IMPED), a condition first identified by Gass and colleagues¹ and thus far described in only five patients in the literature.

Methods : A retrospective case series of three patients with the presumed rare diagnosis of IMPED.

Results : The three patients, in their forties, presented to the Hadassah Retinal Degenerations clinic between the years 2015-2018. All exhibited multiple bilateral PEDs on clinical examination and on wide-field fundus imaging, in the absence of drusen. We confirmed the classification of the lesions as PEDs by OCT. Notably, the patients demonstrated preserved visual acuity and an otherwise normal ophthalmic examination. Electrophysiological testing demonstrated normal retinal and RPE function by full-field electroretinography and electrooculography, respectively. Angiographic studies excluded the diagnoses of NVAMD and PCV. The patients remained stable during the course of follow-up, which was 7, 3.5 and 0.9 years for the study group (including revision of previous imaging studies), during which time we did not note any dynamic changes, either clinically or by ancillary testing.

Conclusions : While in themselves PEDs usually do not necessitate treatment, dynamic changes of PEDs have been associated with possibility of disease progression in AMD and were shown to herald a significant bleeding episode in PCV patients. Here we demonstrate that IMPED, despite being exceedingly rare, appears to represent a distinct clinical entity, seen in relatively young patients. The long-term implications and prognosis of these lesions are not known and warrant further follow-up.

1 Gass. et al. Bilateral idiopathic multifocal retinal pigment epithelium detachments in otherwise healthy middle-aged adults: a clinicopathologic study. Retina 2005; 25:304-310

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

Representative patient demonstrating multiple bilateral PEDs
Top row - Standard fundus camera
Rows 2-3 - wide field fundus imaging and autofluorescence
Rows 4-5 - OCT imaging of both eyes