Multimodal Imaging of the White Dot Syndromes and Related Diseases

Abstract

The white dot syndromes encompass a group of rare posterior uveitis conditions that are characterized by outer retinal and/or choroidal hypopigmented lesions that are thought to be inflammatory in nature. The size, shape, and location of lesions in the fundus aid in differentiating these conditions. Multimodal imaging, including modalities such as fundus autofluorescence, optical coherence tomography, fluorescein angiography, and indocyanine green angiography, among others, has become integral in diagnosing and monitoring many of the white dot syndromes. Furthermore, multimodal imaging modalities have provided insights into the pathogenesis and exact sites within the retina and choroid affected by white dot syndromes.

Keywords: Multimodal imaging; Uveitis; White dot syndromes.

Figure 1

Multimodal imaging of a 63 year-old woman with birdshot chorioretinopathy (BCR). Note the deep hypopigmented lesions in the mid-periphery on color fundus photography (Color) and wide-field Optos pseudocolored (Optos Merge) images. These lesions are especially apparent on Optos 635 nm laser images that focuses on choroidal structures (Optos 635nm). Wide-field Optos fundus autofluorescence (Optos FAF) reveals patchy areas of hypoautofluorescence, some of which do not overlap with underlying birdshot lesions. Wide-field Optos fluorescein angiography reveals diffuse vascular leakage in the late frames (Optos Late FA).

Figure 2

Multimodal imaging of a 53 year-old woman with birdshot chorioretinopathy (BCR). On presentation, the hypopigmented birdshot lesions seen on color fundus photography (Color) appear as hypofluorescent spots on late indocyanine green angiography (Late ICGA). 6 months later, after receiving treatment with oral prednisone and mycophenolate mofetil, the lesions are essentially unchanged on color photographs, while late ICGA reveals substantially fewer dark spots.

Figure 3

Multimodal imaging of a 51 year-old woman with acute posterior multifocal placoid pigment epitheliopathy (APMPPE). At presentation, color fundus photography (Color) showed large hypopigmented placoid lesions in the macula. On fundus autofluorescence (FAF), some lesions were hypoautofluorescent (likely atrophic), some mixed hyper- and hypo-autofluorescent, and some had a surrounding hyperautofluorescent halo (likely active). On fluorescein angiography (FA), lesions were mostly hypofluorescent during early frames (Early FA) with staining on later frames (Late FA). On indocyanine green angiography, lesions were hypofluorescent early (Early ICGA) with persistence and expansion of hypofluorescence in late frames (Late ICGA).

Figure 4

Multimodal imaging of a 51 year-old woman with acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Color fundus photography (Color) and fundus autofluorescence (FAF) images are from the same patient as in figure 3, 15 months following presentation and after receiving a short course of oral prednisone. Note the pigmentary changes in areas of the prior placoid lesions on color images. On FAF 15 months after presentation, lesions were mostly hypoautofluorescent, suggesting atrophy. At presentation, optical coherence tomography (OCT) revealed outer retinal and RPE disruption corresponding to the lesions. Some restructuring of the outer retinal architecture is apparent 15 months after presentation. Infrared reflectance (IR) images are shown to demark the location of OCT B scans.

Figure 5

Multimodal imaging of a 67 year-old man with serpiginous choroiditis. During a period of quiescence, color fundus photography demonstrates a helicoid pattern of chorioretinal atrophy emanating from the optic nerve (Baseline Color), while fundus autofluorescence shows corresponding areas of hypoautofluorescence (Baseline FAF). 5 months later, despite immunosuppressive therapy, disease activity returned with a new area of retinal whitening on color fundus photography (5 months Color, arrow). The newly active lesion appeared mostly hyperautofluorescent on FAF. Optical coherence tomography demonstrated outer retinal loss and RPE disruption in the areas of lesions. An infrared reflectance (IR) image is shown to demark the location of OCT B scans.

Figure 6

Multimodal imaging of a 44 year-old man with serpiginous choroiditis. Color fundus photography shows a well-demarcated, geographic, white-to-yellow, chorioretinal lesions surrounding the optic nerve. Fluorescein angiography (FA) demonstrates hypofluorescence during the early frames (Early FA) with staining in late frames (Late FA).

Figure 7

Multimodal imaging of a 49 year-old woman with ampiginous chorioretinitis. At presentation, color fundus photography (Color) showed an extensive network of hypopigmented lesions in the macula and mid-periphery. On fundus autofluorescence (FAF), some lesions were hypoautofluorescent (likely atrophic), some mixed hyper- and hypo-autofluorescent, and some had a surrounding hyperautofluorescent halo (likely active). On fluorescein angiography (FA), lesions were mostly hypofluorescent during early frames (Early FA) with staining on later frames (Late FA). On indocyanine green angiography, lesions were hypofluorescent early (not shown) with persistence and expansion of hypofluorescence in late frames (ICGA). Optical coherence tomography demonstrates outer retinal loss and RPE disruption in the areas of lesions. Infrared reflectance (IR) images are shown to demark the location of OCT B scans.

Figure 8

Multimodal imaging of a 29 year-old woman with multifocal choroiditis and panuveitis (MCP). Color fundus photography (Color) demonstrates round chorioretinal lesions of various size scattered throughout the macula and periphery. On fundus autofluorescence (FAF), some lesions were hypoautofluorescent (likely atrophic), some mixed hyper- and hypo-autofluorescent, and some had a surrounding hyperautofluorescent halo (likely active). Fluorescein angiography (FA) showed early hypofluorescence (not shown) with late staining (Late FA). On indocyanine green angiography (ICGA), lesions were hypofluorescent early (Early ICGA) with persistence and expansion of hypofluorescence in late frames (Late ICGA). Optical coherence tomography (OCT) showed hyper-reflective outer retinal nodular lesions corresponding to clinically apparent lesions with more widespread disruption in the surrounding outer retinal architecture.

Figure 9

Multimodal imaging of a 17 year-old young man with multiple evanescent white dot syndrome (MEWDS). Note the multiple outer retinal white dots and foveal granularity on the color fundus photograph (Color). On fundus autofluorescence (FAF), lesions were hyperautofluorescent. Fluorescein angiography (FA) showed characteristic “wreath-like” hyperfluorescence of individual lesions that persisted into late frames (Late FA). Optical coherence tomography (OCT) showed disruption of the ellipsoid zone (arrows) corresponding to individual lesions at presentation (OCT Presentation) with reconstitution of these areas two months later after a course of oral prednisone (OCT 2 months). Infrared reflectance (IR) images are shown to demark the location of OCT B scans.

Figure 10

Multimodal imaging of a 35 year-old woman with acute zonal occult outer retinopathy (AZOOR). Color fundus photography (Color) revealed subtle peripapillary pigmentary abnormalities. Fundus autofluorescence (FAF) showed a sharply demarcated area of hypoautofluorescence with a hyperautofluorescent border. Optical coherence tomography (OCT) showed disruption of the outer retinal structures, including the ellipsoid zone and photoreceptor outer segments (arrow), in the area of FAF abnormalities.