Abstract
Background: To present myriad and unique presentation of Vogt-Kayanagi-Harada Disease (VKHD) and Vogt-Koyanagi-Harada (VKH) resembling syndrome. Methods: This is observational series of four cases of VKH disease with variable unique visual presentation which ranged from blurring of vision, tinnitus, hot flushes on face and abdomen, presence of floaters with hedache, breathlessness and history of weight loss. The patient age ranged from 14-56 years. Symptoms developed acutely ranging from 4-12 weeks. Visual acuity varied from 1/60 to 6/6 on Snellen chart. A detailed ophthalmic evaluation, radio imaging and neuroimaging were done as needed on patients. FFA confirmed disc leakage, subretinal pooling of dye in posterior pole and pin point leaks in three cases. The fourth case revealed dilated capillaries, pin point leaks outside zone of hyperflourescence and presence of leopard spots.USG B-scan in same case showed choroidal thickening and an oval hypointense juxtapappilary lesion. OCT-macula revealed a convex retinal profile, thickening of retinal pigment epithelium choriocapillaries complex, multiple subretinal low reflective areas consisting with serous neurosensory detachment and hyper reflective spots in inner retinal areas consistent with findings suggestive of metastasis. All four patients presented in acute uveitic stage. Barring one patient, three patients had bilateral disease. Incomplete VKH disease was diagnosed in three cases. Metastatic intraocular carcinoma with multiorgan involvement with primary in cervix was diagnosed in one case. Two cases of VKH disease were in remission having responded well to oral steroids and immunesuppresive therapy. One case passed to chronic stage with visual deterioration. One case with multi-organ metastasis went on to receive chemotherapy, brachytherapy, whole body and pelvic radiation. Conclusions: VKH disease has variable presentation and a high degree of suspicion is needed to differentiate it from similar occurring malignant and non-malignant intraocular inflammation. Early staging of VKH disease helps in planning adequate treatment leading to greater improvement in disease outcome and simultaneously preventing progression to chronic stage.
Highlights
Vogt-Koyanagi-Harada (VKH) disease is a bilateral granulomatous uveitis often associated with exudative retinal detachment and with extraocular manifestations, such as pleocytosis in the cerebrospinal fluid and in some cases vitiligo, poliosis, alopecia and dysacussis [1]
We present a series of four cases diagnosed with incomplete VKH disease in our hospital with variable clinical features described in detail (Table 1)
A 38 year old North Indian male presented for the first time with history of tinnitus followed by bilateral decrease in vision of eight weeks duration
Summary
Vogt-Koyanagi-Harada (VKH) disease is a bilateral granulomatous uveitis often associated with exudative retinal detachment and with extraocular manifestations, such as pleocytosis in the cerebrospinal fluid and in some cases vitiligo, poliosis, alopecia and dysacussis [1]. Bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined) a) Early manifestations of disease (1) Evidence of diffuse choroiditis (with or without anterior uveitis, vitreous inflammatory reaction, or optic disk hyperaemia) which may manifest as (a) focal areas of subretinal fluid, or (b) bullous serous retinal detachments b) Late manifestations of disease (1) History suggestive of prior presence of early findings noted in 3a and either (2) or (3) below, or multiple signs from 3. Integumentary finding (not preceding onset of central nervous system or ocular disease) a) Alopecia, or b) Poliosis, or c) Vitiligo
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