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Review

Vogt–Koyanagi–Harada syndrome – current perspectives

, &
Pages 2345-2361 | Published online: 24 Nov 2016

Figures & data

Figure 1 Color photograph of a patient in the chronic recurrent phase of the VKHD showing acute anterior uveitis and mild poliosis (white arrow).

Abbreviation: VKHD, Vogt–Koyanagi–Harada disease.
Figure 1 Color photograph of a patient in the chronic recurrent phase of the VKHD showing acute anterior uveitis and mild poliosis (white arrow).

Figure 2 Color retinal photographs of a patient during the acute phase of VKHD.

Notes: (A) Multifocal choroiditis lesions of variable size representing along the superior arcade (black arrow). (B) The posterior pole depicting a hyperemic optic disc (white arrow), multifocal choroiditis (black arrow), and multiple areas of SRDs (red arrows).
Abbreviations: VKHD, Vogt–Koyanagi–Harada disease; SRDs, serous retinal detachments.
Figure 2 Color retinal photographs of a patient during the acute phase of VKHD.

Figure 3 Color retinal photographs of right (A) and left eye (B) showing swollen and hyperemic optic discs (white arrows), with choroidal folds disc (black arrows), in the acute phase of VKHD.

Abbreviation: VKHD, Vogt–Koyanagi–Harada disease.
Figure 3 Color retinal photographs of right (A) and left eye (B) showing swollen and hyperemic optic discs (white arrows), with choroidal folds disc (black arrows), in the acute phase of VKHD.

Figure 4 Color retinal photographs of right (A) and left eye (B) during the convalescent phase of VKHD, showing sunset glow fundus with pale optic discs (white arrows) and bright-orange choroids (black arrows). Note the peripapillary atrophy (gray arrow) and macular scaring (blue arrow) in (A).

Abbreviation: VKHD, Vogt–Koyanagi–Harada disease.
Figure 4 Color retinal photographs of right (A) and left eye (B) during the convalescent phase of VKHD, showing sunset glow fundus with pale optic discs (white arrows) and bright-orange choroids (black arrows). Note the peripapillary atrophy (gray arrow) and macular scaring (blue arrow) in (A).

Figure 5 Color retinal photograph displaying sunset glow fundus with a numerous small choroidal depigmented atrophic lesions (black arrow) and hyperpigmented lesions (white arrow) in the retinal periphery during the convalescent phase of VKHD.

Abbreviation: VKHD, Vogt–Koyanagi–Harada disease.
Figure 5 Color retinal photograph displaying sunset glow fundus with a numerous small choroidal depigmented atrophic lesions (black arrow) and hyperpigmented lesions (white arrow) in the retinal periphery during the convalescent phase of VKHD.

Figure 6 Color retinal and FFA photographs of a patient in the acute phase of VKHD showing (A) hyperemic optic disc with deep yellow lesions of variable size and multiple SRD, (B) early FFA showing delayed choroidal filling, (C) mid phase of FFA showing pinpoint hyperfluorescent leakage at the RPE level (D) with pooling of the dye in the subretinal space in the late phase of the angiogram.

Abbreviations: FFA, fundus fluorescein angiography; VKHD, Vogt–Koyanagi–Harada disease; SRD, serous retinal detachment; RPE, retinal pigment epithelium.
Figure 6 Color retinal and FFA photographs of a patient in the acute phase of VKHD showing (A) hyperemic optic disc with deep yellow lesions of variable size and multiple SRD, (B) early FFA showing delayed choroidal filling, (C) mid phase of FFA showing pinpoint hyperfluorescent leakage at the RPE level (D) with pooling of the dye in the subretinal space in the late phase of the angiogram.

Figure 7 Color retinal photograph of the right eye showing swollen and hyperemic optic discs (A) and FFA showing hot disc (B) (white arrows), in the acute phase of VKHD.

Abbreviations: FFA, fundus fluorescein angiography; VKHD, Vogt–Koyanagi–Harada disease.
Figure 7 Color retinal photograph of the right eye showing swollen and hyperemic optic discs (A) and FFA showing hot disc (B) (white arrows), in the acute phase of VKHD.

Figure 8 FFA photograph of a patient in the acute phase of VKHD showing optic disc leakage and numerous hyperfluorescent pinpoint foci of leakage at the level of RPE leading to the classic “starry sky” appearance.

Abbreviations: FFA, fundus fluorescein angiography; VKHD, Vogt–Koyanagi–Harada disease; RPE, retinal pigment epithelium.
Figure 8 FFA photograph of a patient in the acute phase of VKHD showing optic disc leakage and numerous hyperfluorescent pinpoint foci of leakage at the level of RPE leading to the classic “starry sky” appearance.

Figure 9 Color retinal (A) and FFA (B) photographs of a VKHD patient with macular CNV (white arrows).

Abbreviations: FFA, fundus fluorescein angiography; VKHD, Vogt–Koyanagi–Harada disease; CNV, choroidal neovascularization.
Figure 9 Color retinal (A) and FFA (B) photographs of a VKHD patient with macular CNV (white arrows).

Figure 10 ICG photographs of the right eye of a patient in the acute phase of VKHD showing (A) patchy hypofluorescence during the early angiographic phase, (B) large choroidal stromal vessel hyperfluorescence with fuzzy choroidal vessels in the early phase, (C) hypofluorescent dark dots during the intermediate phase of angiography, and (D) diffuse choroidal hyperfluorescence in the late phase.

Abbreviations: ICG, indocyanine green angiography; VKHD, Vogt–Koyanagi–Harada disease.
Figure 10 ICG photographs of the right eye of a patient in the acute phase of VKHD showing (A) patchy hypofluorescence during the early angiographic phase, (B) large choroidal stromal vessel hyperfluorescence with fuzzy choroidal vessels in the early phase, (C) hypofluorescent dark dots during the intermediate phase of angiography, and (D) diffuse choroidal hyperfluorescence in the late phase.

Figure 11 Short wavelength light (blue) FAF photographs of eyes of a patient with chronic VKHD.

Notes: (A) Right eye showing areas of hypoautofluorescence (black arrow) corresponding to RPE atrophy and loss. (B) Left eye showing areas of hyperautofluorescence (white arrow) corresponding to RPE proliferation.
Abbreviations: FAF, fundus autofluorescence; VKHD, Vogt–Koyanagi–Harada disease; RPE, retinal pigment epithelium.
Figure 11 Short wavelength light (blue) FAF photographs of eyes of a patient with chronic VKHD.

Figure 12 OCT scan of the macula of a patient in the acute phase of VKHD before and after starting corticosteroid treatment.

Notes: (A) SRD (white arrow) and a mild fluctuation in the ILM (red arrow). (B) Resolution of SRD with treatment (white arrow).
Abbreviations: OCT, optical coherence tomography; VKHD, Vogt–Koyanagi–Harada disease; SRD, serous retinal detachment; ILM, internal limiting membrane.
Figure 12 OCT scan of the macula of a patient in the acute phase of VKHD before and after starting corticosteroid treatment.

Table 1 Complications associated with VKHD