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Editor,—Vogt-Koyanagi-Harada (VKH) syndrome is an uncommon disorder characterised by panuveitis in association with neurological and cutaneous abnormalities including headache, tinnitus, vitiligo, poliosis, and alopecia. VKH syndrome almost always affects more heavily pigmented individuals, and is seen in women more than twice as often as men, typically in the third and fourth decades of life. The aetiology of VKH syndrome is unknown, although an autoimmune reaction to shared uveal, dermal, and meningeal melanocytes seems most probable.1
We describe here three patients with VKH syndrome who presented with angle closure glaucoma.
A 37-year-old Asian Indian woman presented with a 4 day history of bilateral eye pain, redness, decreased vision, and headache. Vision was right eye 6/36 and 6/6 left eye. Intraocular pressure was 22 mm Hg in the right and 27 mm Hg in the left eye. Anterior chamber examination showed marked shallowing bilaterally. Gonioscopy revealed closed angles. The optic discs were slightly oedematous with a cup to disc ratio of 0.2 bilaterally. The patient was placed on pilocarpine 2% and bilateral peripheral laser iridotomies were performed but with no change in anterior chamber depth. Three weeks after laser iridotomy the patient represented with decreased vision, moderate anterior chamber inflammation, and iris nodules. Fundus examination showed mild vitritis and massive exudative retinal detachments in each eye. The patient was diagnosed with VKH syndrome and improved quickly on local and systemic corticosteroids.
A 42-year-old Asian Indian man presented with decreased vision, eye redness, and severe headache of 4 days’ duration. Vision was 6/36 in the right and 6/60 in the left eyes. Intraocular pressure was 26 mm Hg bilaterally. Anterior chamber examination showed marked shallowing with no angle structures visible on gonioscopy in either eye. Fundus examination showed a cup to disc ratio of 0.3 bilaterally. The patient was placed on pilocarpine 2% and peripheral laser iridotomies were performed but with no change in anterior chamber depth. The patient returned 2 weeks later with severe headache, mildly elevated intraocular pressure, and moderate anterior chamber and vitreous inflammation with total exudative retinal detachments in each eye. The patient was diagnosed with VKH syndrome, and improved quickly on local and systemic corticosteroids.
A 16-year-old Asian Indian woman presented with decreased vision in each eye, severe headache, and tinnitus of 2 weeks’ duration. Vision was 2/60 in the right eye and counting fingers in the left. Intraocular pressure was 21 mm Hg in the right eye and 24 mm Hg in the left. Anterior chamber examination showed mild inflammation and marked shallowing. Scleral spur was barely visible by gonioscopy in each eye. Fundus examination revealed mild vitritis, moderate optic disc swelling with a cup to disc ratio of 0.2, and near total exudative retinal detachments bilaterally. The patient was diagnosed with VKH syndrome, and treated with local and systemic corticosteroids. The intraocular inflammation subsided and intraocular pressures normalised quickly, but the exudative retinal detachments resolved slowly leaving widespread retinal pigment epithelial atrophy and a vision of 2/60 bilaterally.
We described three patients with VKH syndrome who presented with angle closure glaucoma. Intraocular pressures were only mildly elevated despite gonioscopic angle closure in each patient. Moreover, laser iridotomies, although performed promptly in two patients, failed to produce anterior chamber deepening. While angle closure glaucoma is reported to be a rare presentation for VKH syndrome,2-10our three patients constituted about 3% of the total cohort of 98 patients with this disorder seen in the uveitis clinic at Aravind Eye Hospital in Madurai, India, between January 1993 and January 1996. Ophthalmologists should consider VKH syndrome in patients who present with angle closure glaucoma, particularly in populations known to be at risk, and in the setting of significant anterior or posterior chamber inflammation.
This work was supported by a grant from the De Loris Lange Foundation for International Eye Research.
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