Clinical and multimodal imaging characteristics of acute Vogt–Koyanagi–Harada disease unassociated with clinically evident exudative retinal detachment

Abstract

The purpose of this study was to describe the clinical and multimodal imaging findings in acute Vogt-Koyanagi-Harada (VKH) disease without clinically evident exudative retinal detachment (ERD). We retrospectively reviewed the charts of 18 patients (36 eyes), diagnosed with acute VKH disease without clinically evident ERD. All patients underwent complete ophthalmic examination, fundus photography, optical coherence tomography (OCT), B-scan ultrasonography, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Of 18 patients, twelve (66.7 %) were female and 6 (33.3 %) were male. Mean age was 39 years (range, 23-60). Ten patients had been referred with an erroneous diagnosis of primary optic nerve disorder (8; 44.4 %) or isolated anterior uveitis (2; 11.1 %). Anterior chamber or vitreous inflammatory reaction was noted in 22 eyes (61.1 %), each. Fundus findings included optic disc swelling in 30 eyes (83.3 %), retinal striae in 20 eyes (55.5 %), and yellowish deep lesions in 3 eyes (8.3 %). OCT showed a shallow, localized subclinical ERD in 18 eyes (50 %), and retinal pigment epithelial folds in 23 eyes (63.9 %). B-scan ultrasonography showed diffuse, low- to medium-reflective choroidal thickening in all eyes. FA disclosed delayed choroidal perfusion in at least one eye of all patients (100 %), mild pinpoint leakage in 21 eyes (58.3 %), optic disc hyperfluorescence in 35 eyes (97.2 %) and choroidal folds in 13 eyes (36.1 %). ICGA findings included delayed choroidal perfusion in 24 eyes (66.7 %), decrease in the number of large choroidal vessels in 36 eyes (100 %), fuzzy choroidal vessels in 35 eyes (97.2 %), and hypofluorescent dark dots in 28 eyes (77.8 %). The association of bilateral optic disc edema with retinal striae and intraocular inflammatory reaction highly suggests acute VKH disease. A multimodal imaging approach including fundus photography, OCT, B-scan ultrasonography, FA, and ICGA provides important clues for the definite diagnosis and help differentiate VKH disease from primary optic nerve disorders.

Multi-modal imaging and anatomic classification of the white dot syndromes

“…Enhanced depth imaging (EDI) OCT reveals a thickened choroid and can be used to monitor serous retinal detachments. Hypofluorescent pinpoint dots at the early phase of FA followed by multiple focal areas of leakage and subretinal dye accumulation at the late phase can be seen [15–17]. In chronic VKH, peripheral FAF abnormalities are seen.…”

Vogt–Koyanagi–Harada syndrome – current perspectives

“…Unilateral and bilateral optic disc hyperemia (Figure 3) without visual field loss is a frequent presentation in acute VKHD55–57 and is the most common finding in acute VKHD patients with no SRD at presentation 58…”

“…In addition to older age at presentation, optic disc swelling is found to correlate significantly with the morphology of the optic nerve (a smaller cup-to-disc ratio and a higher disc–macula distance to disc diameter) 57. Swollen optic discs may be the only presenting sign and should be differentiated from other conditions,56 such as optic neuritis,59 diabetic papillopathy,58 central retinal vein occlusion, or anterior ischemic optic neuropathy (AION). Extensive optic disc swelling can itself lead to further optic nerve pathology, and patients can develop AION following the resolution of the optic disc swelling and SRD.…”

A super-resolution method-based pipeline for fundus fluorescein angiography imaging

“…Second, FFA is still the most commonly used method to plan laser treatment (photocoagulation) in clinical settings [ 60 ]. Third, for clinical research involving multimodal imaging, OCT, FFA and other modalities are often used cooperatively [ 61 ]. In fact, considering the characteristic of OCT images, we also wonder if our proposed SR-based pipeline method can be used to the enhance OCT images, which can be a potential research direction for future work.…”