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Editor,—Vogt–Koyanagi–Harada (VKH) disease is a chronic panuveitis associated with poliosis, alopecia, vitiligo, dysacousia, and meningeal signs.1 In VKH, optic disc involvement is not unusual and disc oedema is one of the hallmarks of the disease.2 However, severe visual loss, altitudinal visual field defect, and sector filling defect on fluorescein angiography that suggest anterior ischaemic optic neuropathy (AION) are not usual presentations. We describe a case of VKH masquerading AION.
The patient was a 68 year old man who had decreased right visual acuity. He had systemic hypertension and multiple small brain infarction. On initial examination, the visual acuity was 20/50 in the right eye and 20/30 in the left. On the next day, his visual acuity declined to 20/100 in the right eye and 2/200 in the left. Slit lamp examination showed no signs of ocular inflammation but fundus examination revealed swelling of the both optic discs. Eight days later, keratic precipitates and cells in the anterior chamber were found. Multiple serous retinal detachments in the both eyes occurred on the 14th days. Enlargement of the Marriote blind spot and central scotoma were demonstrated by Goldmann visual field examination. Pleocytosis of the cerebrospinal fluid and human leucocyte antigen (HLA) type DR4 were found. Computed tomography showed small infarctions in the bilateral basal ganglia and the pons.
Fluorescein angiography (FA) showed a wedge-shaped filling defect in the optic disc of the left eye in the early phase and later leakage of the dye from both optic discs (Fig 1A). On the day 8, bilateral hyperfluorescence in the posterior pole typical of VKH was observed. Indocyanine green (ICG) videoangiography showed filling delay of choroidal circulation.3 Choroidal vessels were indistinct and speckled hypofluorescence on the diffuse background fluorescence was seen in the early to late phase (Fig 1B). Three months later, the fundus showed depigmentation typical of VKH. The visual function of the right eye showed some recovery but visual acuity in the left eye was 15/200 and no recovery in the visual field defect was observed.
In this case, at the early stage of disease, signs and symptoms suggestive of AION were found. Typical features of VKH emerged on later examinations. Although disc oedema is a common finding in VKH,2 severe visual loss, sector filling defect, and visual field loss are unusual in VKH. Sector filling defect in the disc on FA and altitudinal visual field defect are more compatible with AION. Whether AION was caused independently of VKH or inflammatory cells in the choroid4 caused circulatory disturbances is hard to determine because the patient was at high risk of circulatory disorders. But disturbed choroidal circulation by inflammatory cells in VKH3 may support the latter idea. Indeed, Perry and Font5 reported pathology of severe cases of VKH. In their cases, infiltration of the leptomeninges by lymphocytes and mild to moderate arachnoidal proliferation were found. Severe visual loss from optic nerve involvement in VKH is an important but not well documented complication of VKH.
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