The posterior uveitis stage refers to within 2 weeks after uveitis attack. Sudden bilateral blurring and decrease of vision are the most common symptoms. The majority of patients present with decreased vision simultaneously in both eyes. In the remaining patients, the interval between uveitis onsets of both eyes is usually from 1 to 2 weeks. Diffuse choroiditis is the characteristic finding. Most patients present with exudative retinal detachment and optic disc swelling (Fig. 9.1). No anterior reaction is observed in this stage. Fundus fluorescein angiography (FFA) shows numerous punctate hyperfluorescent dots in the early phase and subretinal dye pooling in the late phase (Fig. 9.2a). Optic disc staining is also a common finding. Indocyanine green angiography (ICGA) typically shows multiple hypofluorescent dots and dark areas corresponding to serous retinal detachment (Fig. 9.2b). Exudative retinal detachment disclosed with optical coherence tomography (OCT) is a rule in this stage (Fig. 9.2c). Tinnitus, hearing loss, and meningismus may be still present in this stage.

The anterior uveal involvement stage usually begins from 2 weeks after the uveitis attack. This stage usually lasts for 1.5 months. In this stage, choroiditis is still observed in most patients. A mild to moderate anterior uveitis characterized by flare and cells in the anterior chamber and dust-keratic precipitates (KP) is the prominent manifestation. Granulomatous anterior uveitis is very rarely seen in this stage. Mild vitreous inflammatory reaction and optic disc swelling are also noted. Tinnitus and hearing loss are the most common extraocular symptoms, and integumentary changes including poliosis, alopecia, and vitiligo are observed in some patients in this stage (Fig. 9.3).

Without appropriate and prompt treatment, VKH patients may eventually develop recurrent chronic granulomatous anterior uveitis. During this stage, active choroiditis normally resolves and “sunset glow” fundus ensues as a result of depigmentation of the retinal pigment epithelium (RPE) cells and the choroid (Fig. 9.4). In some patients, the sclera tissue can be observed because of severe depigmentation of the choroid (Fig. 9.5). Dalen-Fuchs nodules, which manifest as small, round to oval active lesions in the midperiphery of the fundus, chorioretinal atrophy, and migration of RPE cells are the other common findings in the posterior segment (Fig. 9.4). Anterior uveitis is a very striking feature and typically presents with mutton fat KP, Koeppe nodules, Busacca nodules, and significant anterior chamber reaction (Fig. 9.6). Window defects due to RPE cell damages and blockade of fluorescence arising from migration of retinal RPE cells are common FFA findings. Ultrasound biomicroscopy may show the cells in the anterior chamber as thickened and swollen ciliary body as well as ciliochoroidal detachment in patients in this stage (Fig. 9.7). Ocular complications including cataract, secondary glaucoma, subretinal fibrosis, and choroidal neovascularization are observed in this stage. Poliosis, alopecia, and vitiligo are common extraocular findings.