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Bilateral angle closure glaucoma: an unusual presentation of Vogt–Koyanagi–Harada syndrome
  1. Department of Ophthalmology, Rambam Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel
  1. Dr Y A Gelfand, Department of Ophthalmology, Rambam Medical Center, POB 9602, 31096 Haifa, Israel.

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Editor,—Vogt–Koyanagi–Harada (VKH) syndrome is a bilateral, diffuse, granulomatous uveitis associated with exudative retinal detachment and central nervous system, auditory, and dermatological involvement.1 It is a common cause of uveitis in Japan and, to a lesser extent, in parts of Latin America and the USA.

We report a case of VKH syndrome with an unusual presentation of bilateral secondary acute angle closure glaucoma (SAACG). Because of the rarity of this presentation of VKH syndrome (this being the first report, to our knowledge, of such a presentation outside the USA and Japan) we initially had difficulties in diagnosing and treating it in the optimal way. We thus stress the importance of clinicians being aware of such a VKH syndrome presentation, especially in those parts of the world where the disease is rare.


A 20-year-old white woman presented in April 1996 complaining of sudden onset of blurred vision in both of her eyes. Her past medical history and her family history were unremarkable.

Visual acuity was 6/24 in both eyes, improving to 6/12 with a pinhole. The corneas were slightly oedematous, without keratic precipitates. The anterior chambers were shallow, with +1 cells. The pupils were normal and reacted to light. Intraocular pressures were 26 mm Hg in the right eye and 29 mm Hg in the left eye. Gonioscopy revealed closed angles in both eyes. Owing to the corneal oedema and our reluctance to dilate the pupils at that stage, details of the posterior poles of the eyes could not be visualised with certainty.

The woman was thus diagnosed as suffering from bilateral SAACG and was treated with topical pilocarpine and timolol, combined with intravenous acetazolamide and oral glycerol. Since the intraocular pressures could not be controlled with this treatment, bilateral laser iridotomies were performed resulting in reduction of pressures to normal. This was accompanied by a progressive deepening of the anterior chambers.

At this stage we dilated the pupils and revealed +2 cells in the vitreous in both eyes. Ophthalmoscopy disclosed multiple areas of serous retinal detachment in the posterior poles, combined with bilateral inferior exudative retinal detachment (Fig 1). Fluorescein angiography demonstrated multifocal leakage of dye at the level of the retinal pigment epithelium in the early phase and late pooling of fluorescein under the areas of the detached retina in the late phase in both eyes (Fig 2).

Figure 1

Fundus photography, showing multiple areas of serous retinal detachment at the posterior pole combined with inferior exudative retinal detachment.

Figure 2

Fundus fluorescein angiogram (late phase), with pooling of fluorescein under the detached retina.

General physical examination, as well as neurological, dermatological, and auditory examinations were all normal. Lumbar puncture disclosed 127 lymphocytes per mm2 of cerebrospinal fluid, with normal protein levels. Results of other laboratory tests, including chestx ray, complete blood count, erythrocyte sedimentation rate, serum chemistry, liver enzymes, rheumatoid arthritis titre, blood serology, including serological syphilitic tests, and urine analysis were all within normal limits.

Based on the presence of bilateral iridocyclitis, posterior uveitis with bilateral exudative retinal detachment, and cerebrospinal fluid pleocytosis the diagnosis of VKH syndrome was made.2 The patient was treated with topical steroids and cycloplegia combined with 100 mg of oral prednisone daily, which was tapered down to 40 mg daily over the next month. During that period the retinal detachments resolved and the visual acuity improved to 6/6 in both eyes. A corneal endothelium cell count performed after the termination of the uveitic attack was normal.


Glaucoma occurs in 20% to 38% of patients with VKH syndrome.34 We are aware of only 12 reported cases of bilateral SAACG in VKH syndrome,4-6 of which in at least seven cases the glaucoma was the presenting symptom.

SAACG in cases of VKH syndrome is cyclocongestive in nature, resulting from ciliary body swelling6 which may be combined with choroidal effusion. Pilocarpine is contraindicated in the management of such cases, because it increases the dilatation of the uveal blood vessels (and by that increases intraocular inflammation and angle congestion) and causes further shallowing of the anterior chamber.7

As pointed out by Kimura et al,5 when pilocarpine is mistakenly prescribed for such cases, almost all patients will need an iridectomy or filtering operation. Since we did not diagnose the underlying disease initially and treated the patient with pilocarpine, we could not break the attack until bilateral YAG laser iridotomies were performed. This raises the suspicion that an additional element of pupillary block (possibly through an increased stickiness between the iris and the lens) was caused by the use of pilocarpine.

Corneal swelling is unusual in the presence of healthy corneal endothelium and mild anterior uveitis.8 A corneal endothelium cell count performed on the patient after the termination of the uveitic attack was normal. Thus, explanations for the mild corneal oedema noted on the patient’s presentation can only be speculative. It is not unreasonable to assume that the patient might have had a long standing asymptomatic anterior uveitis and that the sudden intraocular pressure rise resulted in mild corneal oedema.

In conclusion, in order to prevent unnecessary operations, pilocarpine should not be prescribed in SAACG due to uveitis. Since ultrasound is almost diagnostic in VKH syndrome3 and may provide additional information about posterior segment pathology in other predisposing diseases,9 it should be performed in suspected cases of SAACG before any medical iris manipulation is performed.


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