PMC10083237_01
Patient 1. (A) Right eye revealed yellow-white placoid lesions in the posterior pole and extending into the mid-periphery. Some appeared gray and healed, and others appeared more white and active (superior nasally). Left eye (not shown) looked similar to the right eye. (B,C) Autofluorescence revealed bilateral hypofluorescent lesions with hyperfluorescent edges. (D) Early fluorescein angiography (FA) showed early hypofluorescence of lesions. (E) FA showed late staining of the lesions. (F) ICG showed hypofluorescence of the lesions. (G) Late ICG showed continued hypofluorescence of the lesions.
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F2
Her fundus exam revealed yellow-white and gray placoid lesions in the posterior pole extending into the mid-periphery in both eyes (Figure 2A). Fundus autofluorescence (FAF) showed hypofluorescent lesions with hyperfluorescent edges (Figures 2B,C). On FA, both eyes demonstrated early hypofluorescence and late staining of the lesions (Figures 2D,E). On ICG, the lesions appeared hypofluorescent in both eyes in early and late phases (Figures 2F,G).
Patient 1. (A) Ultrawide-field fundus color imaging over time. Right eye only. The left eye demonstrated similar findings. The placoid lesions increased in quantity over time from initial presentation, 1 month, and 4 months to 14 months after presentation. (B) Fundus autofluorescent studies over time. Right eye only. The left eye demonstrated similar findings. The hypo/hyperautofluorescent lesions increased in quantity over time from initial presentation, 1 month, 2 months, 3 months, and 4 months to 14 months after presentation.
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F3
At 1, 2, 3, and 4 months after the presentation, more yellow-white and gray placoid lesions were noted on fundus color pictures (Figure 3A) that appeared hypo/hyperautofluorescent on FAF in both eyes (Figure 3B). Fourteen months later, a new symptomatic choroiditis lesion was noted at the macula (Figure 3A) that showed hyperautofluorescence (Figure 3B) with outer retinal hyperreflectivity on SD-OCT.
PMC10083237_02
Patient 2. (A,B) Ultrawide-field fundus color imaging showed yellow-white placoid lesions in the posterior pole and extending into the mid-periphery. (C,D) Placoid lesions with central hypofluorescence and peripheral hyperfluorescence. (E,F) SD-OCT imaging demonstrated thickened retina, disrupted foveal contour, subfoveal and hyper-reflective lesions of the outer retina layers (red arrows) in both eyes. (G,H) After IV methylprednisolone, the SD-OCT demonstrated a resolution of subretinal fluid in the right eye, but the hyperreflective alterations persisted in the outer retina in the left eye and the EZ layer at the macula.
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F4
The fundus exam was significant for bilateral yellow-white choroidal lesions scattered in the posterior pole and extending to the periphery in both eyes with macular involvement in the right eye (Figures 4A,B). FAF imaging showed lesions with central hypoautofluorescence and peripheral hyperautofluorescence (Figures 4C,D). SD-OCT demonstrated thickened retina, disrupted foveal contour, focal subfoveal fluid, and hyper-reflective lesions of the outer retina layers in both eyes (Figures 4E,F). APMPPE chorioretinal lesions (Table 2). After completion of 2 days of IV methylprednisolone, SD-OCT demonstrated a resolution of subretinal fluid in the right eye, but the hyperreflective alterations persisted in the outer retina in the left eye as well as those involving the EZ layer in the macula, bilaterally (Figures 4G,H).
Patient 2. Fluorescein angiography: (A) early hypofluorescence with late (B) hyperfluorescence of the placoid lesions.
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F5
Oral FA showed early hypofluorescence with late hyperfluorescence of the fundus lesions indicative of active inflammation (Figures 5A,B).
Patient 2. Eight months after the initial visit, on 40 mg/2 weeks adalimumab, visual acuity was drastically improved to 20/40 in the right eye eccentrically. (A,B) Ultrawide-field fundus color imaging showed more yellow-white placoid lesions in the posterior pole and extending into the mid-periphery compared to the initial presentation; (C,D) Placoid lesions with central hypofluorescence and peripheral hyperfluorescence.
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F6
Optos fundus pictures, however, showed additional active placoid subretinal lesions in the periphery of both eyes (Figures 6A,B) that appear hyperautofluorescent with a hypofluorescent center (Figures 6C,D).
PMC10083237_03
Patient 3. (A,B) Fundus pictures showing posterior retinal lesions occurring simultaneously with macular involvement. (C-F) At 12 and 36 months, the lesions look older, healing pigmented but more numerous and widespread.
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F7
Table 2). Ten days after the initial presentation, a new activity was detected in the fellow eye in the form of a macular chorioretinal lesion (Figure 7A). Unfortunately, due to multiple missed appointments and treatments, when the patient was reviewed 12 months after the initial presentation, the chorioretinal lesions had progressed within the posterior pole of both eyes (Figures 7C,D). His visual acuity was stable at worse than 20/200 in both eyes, but new lesions continued to develop in the mid-periphery of both eyes (Figures 7E,F).
Patient 3. (A) At presentation, spectral-domain and optical coherence tomography angiography (SD-OCT) showed right hyperreflective lesions of the outer retinal involving the retinal pigment epithelium, the ellipsoid zone, and outer nuclear layers. (B) At 12 months, progression of the macular retinal pigmentary inflammatory lesions with inflammation of the outer retinal and sub- and intraretinal deposits and focal photoreceptor/RPE atrophy.
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F8
SD-OCT showed right hyper-reflective lesions of the outer retinal involving the RPE, EZ, external limiting membrane (ELM), and ONL layers and new hyperreflective lesions on SD-OCT at the macula in the left eye (Figure 8A). On SD-OCT, the lesions were hyper-reflective from occupying space from the choroid to the outer retina through the inner retina and associated with thinning of the retina (Figure 8B).
Patient 3. (A,B) Lesions were hypofluorescent at the early phase of FA, (C,D) with late leakage, and (E-G) Hypofluorescent on all phases of ICG.
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F9
The lesions were hypofluorescent in the early phase of FA (Figures 9A,B) with late hyperfluorescence (Figures 9C,D) and hypofluorescent in the early to late phases of ICG (Figures 9E-G).
Patient 3. (A,B) At last follow-up, intermediate phase of fluorescein angiography (FA) of both eyes showing hypofluorescence of the lesions in the posterior pole with hyperfluorescent borders and extensive widespread new lesions with staining.
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F10
The patient was restarted on monthly infliximab infusions, and due to the continued progression of lesions 3 years after the initial presentation (Figure 10), the patient was switched to adalimumab (40 mg/1 week), which finally stopped additional new lesions.
PMC10083237_04
Patient 4. (A,B) At presentation, fundus photographs of both eyes showing extensive white/grey placoid lesions in the posterior pole and extending into the periphery, with preretinal heme OD. Lesions appear mostly atrophic and inactive. (B,C) At 5 month follow up, there appears to be no obvious progression of disease or active lesions.
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F11
The fundus examination revealed yellow and gray placoid lesions (of varying stages/pigmentation) in the posterior pole and extending into the mid-periphery in both eyes, as well as preretinal hemorrhage in the right eye (Figures 11A,B).
Patient 4. (A,B) At presentation, fundus autofluorescence (FAF) of both eyes showing extensive hypofluorescent lesions with very few scattered lesions with hyperfluorescent edges. (C,D) At 5 month follow up, there appears to be no obvious new lesions/progression or active hyperfluorescent lesions.
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F12
FAF showed extensive hypofluorescent lesions with very few scattered lesions with hyperfluorescent edges (Figures 12A,B). FAF (Figures 12C,D) off oral prednisone. FA showed regression of small vessel vasculitis and no evidence of active lesions (Figures 13C,D).
Patient 4. (A,B) At presentation, late phase fluorescein angiography (FA) of both eyes showing diffuse late staining of the lesions, with extensive small vessel vasculitis/ferning. There is no neovascularisation of the disc or retina. (C,D) At 5 month follow up, there was regression of the mid-peripheral and peripheral small vessel vasculitis, with no evidence of recurrence of neovascularisation of the disc.
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F13
On FA, both eyes demonstrated diffuse late staining of the lesions, with extensive small vessel vasculitis/ferning and regression of the disc neovascularization previously noted in the right eye (Figures 13A,B). SD-OCT revealed extensive EZ layer and outer retinal loss in the parafovea of the right eye and extensive EZ/outer retinal loss involving the fovea in the left eye (Figures 13AB). FAF (Figures 12C,D) off oral prednisone. FA showed regression of small vessel vasculitis and no evidence of active lesions (Figures 13C,D).