Abstract

The white spot syndromes (WSS) are a group of diseases characterized by inflammation and dysfunction of the outer retina, retinal pigment epithelium, and the choroid or a combination of these. The diseases often present diagnostic and therapeutic challenges for clinicians and researchers. The etiologies of WSS remain unknown. This chapter discusses birdshot chorioretinopathy (BCR), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic serpiginous choroiditis (ISC), relentless placoid chorioretinitis (RPC), persistent placoid maculopathy (PPM), multifocal choroiditis (MFC)/punctate inner choroidopathy (PIC), multiple evanescent white dot syndrome (MEWDS), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Each disease has distinct features, but they do share some characteristics. Blurred vision, photopsias, visual field changes (including enlarged blind spots), floaters, and changes in contrast sensitivity can occur. Although these entities are thought to be inflammatory in nature, vitritis and iritis are not necessary findings. The white spots themselves may be subtle or prominent. Inflammatory multifocal chorioretinopathies may actually be a more descriptive term. Multimodal imaging has significantly advanced our knowledge about this group of diseases.

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