En Face Optical Coherence Tomography and OCT Angiography in the Pathoanatomy of Inflammatory Macular Disease

Summary

En face OCT and OCTA in pathoanatomy of inflammatory macular disease, including MEWDS, MFC, AZOOR, APMPPE, syphilis, serpiginous, and VK.

Abstract

Purpose: To review the pivotal role of en face optical coherence tomography (OCT) and OCT angiography (OCTA) in elucidating the pathoanatomy, diagnostic markers, and clinical management of inflammatory macular diseases. These noninvasive modalities provide depth-resolved, layer-specific visualization of structural and vascular alterations, enhancing our understanding of disease mechanisms and facilitating monitoring of disease activity and therapeutic response.

Methods: This perspective article synthesizes recent advances in multimodal imaging (MMI) with emphasis on en face OCT and OCTA across the spectrum of posterior noninfectious and infectious uveitic disorders. Representative diseases include multiple evanescent white dot syndrome (MEWDS), punctate inner choroidopathy (PIC), and idiopathic multifocal choroiditis (iMFC), acute zonal occult outer retinopathy (AZOOR), acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and its chronic variants, serpiginous choroiditis (SC), acute syphilitic posterior placoid chorioretinopathy (ASPPC), and Vogt-Koyanagi-Harada (VKH) disease. Imaging signatures were reviewed in relation to pathophysiology, differential diagnosis, and clinical management.

Results: En face OCT delineates disease-specific topographic patterns: “dots and spots” in MEWDS, hyperreflective plaques in PIC/iMFC, trizonal maps in AZOOR, sharply demarcated placoid lesions in APMPPE, geographic patterns in SC, and concentric hyperreflective “fingerprint” rings in VKH. OCTA provides complementary vascular insights, distinguishing preserved vs impaired choriocapillaris (CC) flow and revealing disease-driven ischemia. MEWDS typically demonstrates preserved CC perfusion, in contrast to the flow deficits of APMPPE, relentless placoid chorioretinitis, and SC. In PIC/iMFC, OCTA enables detection of inflammatory choroidal neovascularization (iCNV), while in ASPPC and VKH, it captures reversible or persistent CC flow voids following appropriate systemic therapy. Structural-vascular correlation permits differentiation between ischemic and nonischemic disorders, recognition of masquerades, and monitoring of subclinical disease activity.

Conclusions: En face OCT and OCTA have redefined the diagnostic and management landscape of inflammatory macular diseases by providing rapid, reproducible, and layer-specific structural-vascular information. Their disease-specific signatures enhance accuracy, support treatment decisions, and improve monitoring of progression and complications such as inflammatory CNV. Integration of these tools into routine multimodal imaging protocols is essential, while future research should prioritize standardization of acquisition and interpretation criteria, quantitative biomarkers, and expanded use of widefield technologies to capture peripheral and longitudinal disease dynamics.