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Multimodal imaging during active and inactive disease in patient 1.. (A) Fundus photograph in patient 1 during active disease shows fovea-threatening cream-colored placoid lesions. (B) FAF reveals a heterogeneous pattern of central hypoautofluorescent lesions with surrounding stippled hyperautofluorescence. (C) Late stage FA demonstrates late leakage of the placoid lesions without foveal involvement. (D) OCT shows outer retinal architectural disruption. (E) Fundus photography one month later reveals RPE hyperpigmentation and resolution of cream-colored lesions. (F) FAF at this time point demonstrates hypoautofluorescent lesions with surrounding stippled hyperautofluorescence. (G) Late stage FA shows staining with resolved areas of early blockage and late leakage. (H) OCT demonstrates partial restoration of the ellipsoid zone.
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Funduscopic examination in the left eye revealed concern for an enlarging cream-colored placoid lesion threatening the fovea (Fig 6).
PMC7446910_02
Multimodal imaging demonstrates lesions are more apparent on ICG-A than other imaging modalities in patient 3.. Initial fundus photograph of the right eye (A) shows multifocal cream-colored lesions with leakage on FA (B). FAF (C) shows hyperautofluorescence only partially corresponding to the area of choroidal disease, while ICG-A (D) outlines hypocyanescent lesions that appear larger than visualized on fundus photography or FAF. In the left eye, a fundus photograph (E) shows choroidal lesions, minimal FA hyperfluorescence (F) and minimal changes on FAF (G). ICG-A (H) highlights the choroidal lesions that are more numerous than seen on fundus exam or FAF.
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FAF demonstrated hyperautofluorescent lesions surrounded by a hypoautofluorescent ring in the right eye and no abnormal hyperfluorescence in the left eye (Fig 4).
PMC7446910_03
Fundus changes in APMPPE during active and inactive stages of disease.. (A) Fundus photo of the left eye of patient 5 demonstrates multiple cream-colored placoid parafoveal lesions indicative of active disease. (B) Five days after treatment with high-dose prednisone, the lesions decreased in size with the interval development of overlying RPE hyperpigmentation. (C) One month later, fundus photo of the left eye revealed RPE pigmentary changes with complete resolution of the previously noted cream-colored lesions.
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On initial evaluation, funduscopic examination revealed several pigmented areas of RPE scarring in the right eye and 8-10 cream-colored lesions in the posterior pole of the left eye (Fig 1).
Multimodal imaging during a recurrence of inflammation in patient 5.. Fundus photos of the left eye in patient 5 demonstrated multiple new cream-colored lesions near the fovea (A). FAF (B) showed stippled mixed hyperautofluorescent and hypoautofluorescent lesions and FA (C) revealed late hyperfluorescence. ICG (D) demonstrated hypocyanescent lesions that corresponded to the clinically visible lesions. OCT (E) displayed areas of IS-OS disruption.
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Approximately one year later, the patient presented with a new scotoma in the left eye, and funduscopic examination revealed new cream-colored deep lesions near the fovea in the left eye (Fig 3).