New staging of Acute Posterior Multifocal Placoid Pigment Epiteliopathy by using multimodal imaging

Abstract

Purpose : Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare disease involving the choriocapillaris, retinal pigment epithelium and retina. Although the pathoaetiology remains unknown, inflammation of the choriocapillaris is the likely primary insult. The aim of this study by using multimodal imaging was to determine a sequence of structural changes during evolution and course of APMPPE.

Methods : All patients with a new diagnosis and acute presentation of APMPPE seen at our regional care centre from October 2015 to October 2016 were included. Multimodal imaging employed on all patients from diagnosis included: fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), spectral domain-optical coherence tomography (SD-OCT) and OCT-Angiography (OCT-A). Non-invasive imaging was repeated during follow-up.

Results : Five patients were included in the study, 3 males and 2 females, with a mean age of 26.2 (range: 21-32) years and a mean follow-up of 4.69 (range: 2.3-10.3) months. All patients presented with bilateral disease and macular involving lesions, accompanied by significant headache and a viral-prodrome prior to presentation. Multimodal imaging has consistently highlighted 4 sequential stages during APMPPE (see legend).

Conclusions : The use of multimodal imaging for the follow-up of patients with APMPPE supports the increasing evidence of a choriocapillaritis. The evolution shows resolution of ischaemia but a defined sequence that results in permanent changes at the level of the retinal pigment epithelium (RPE).

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

 

Multimodal imaging in APMPPE was able to define 4 stages. Stage 1: Choriocapillaris hypoperfusion disclosed through OCT-A and confirmed with ICGA and early FFA phenotype. Stage 2: Evidence of classic active lesion on the FFA with early hypofluorescence and late hyperfluorescence, persistent hypocyanescence lesion through ICGA frames, hypoperfusion on the OCT-A and disorganisation and hypereflectivity of the outer retinal layers on the SD-OCT. Stage 3: Thinning and disruption of outer retinal layers on the SD-OCT, but persistent ischemia of the choriocapillaris on the OCT-A. Stage 4: Remaining retinal thinning in the SD-OCT at the lever of outer retina with hyporeflectivity at the RPE and hypoautofluorescent lesions, but normalised choriocapillaris on the OCT-A.