Application of OCT-Angiography to characterise the evolution of chorioretinal lesions in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Abstract

Purpose

The aim of this study was to determine a sequence of structural changes in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) using optical coherence tomography-Angiography (OCT-A) and comparing with other imaging modalities.

Methods

Patients with a new diagnosis of acute onset APMPPE referred to a regional specialist centre from October 2015 to October 2016 were included. Multimodal imaging employed on all patients from diagnosis included: fundus fluorescein angiography, indocyanine green angiography, fundus autofluorescence, spectral domain-OCT (SD-OCT) and OCT-A. All non-invasive imaging was repeated during follow-up.

Results

Ten eyes of 5 patients were included in the study, 3 males and 2 females, with a mean age of 26.2 years (range: 21-32) and a mean follow-up of 6.4 months (range: 2.6-13.3). All patients presented with bilateral disease and macular involving lesions. OCT-A imaging of the choriocapillaris was supportive of hypoperfusion at the site of APMPPE lesions during the acute phase of this condition with normalisation of choroidal vasculature during follow-up. Multimodal imaging consistently highlighted four sequential phases from presentation to resolution of active disease.

Conclusions

Multimodal imaging in patients with APMPPE in acute and long term follow-up demonstrates a reversible choroidal hypoperfusion supporting the primary inciting pathology as a choriocapillaritis. The evolution shows resolution of the ischaemia through a defined sequence that results in persistent changes at the level of the retinal pigment epithelium and outer retina. OCT-A was able to detect pre-clinical changes and chart resolution at the level of the choriocapillaris.