Vogt-Koyanagi-Harada syndrome presenting as bilateral simultaneous acute angle closure

Abstract

Presentation of bilateral simultaneous angle closure in a patient must alert one to look beyond the common "knee-jerk" diagnosis of acute primary angle closure (APAC). We describe a case with bilateral simultaneous acute angle closure with signs of anterior chamber inflammation with intraocular pressure of 60 mmHg. Based on the clinical details and ultrasonic findings of peripapillary choroidal thickening and supraciliary fluid, a diagnosis of Vogt-Koyanagi-Harada (VKH) disease was made. Our case highlights that every angle closure is not a primary angle closure requiring laser peripheral iridotomy. Detailed clinical examination with appropriate investigations could prevent misdiagnosis of APAC in VKH.

Introduction

Acute primary angle closure (APAC) is an ocular emergency which is traditionally managed with laser peripheral iridotomy (LPI). However, it rarely presents as an acute attack in both the eyes simultaneously. Such a presentation must always alert one to look beyond the common "knee-jerk" diagnosis of APAC. Bilateral acute angle closure (AAC) has been reported due to topical mydriatics in predisposed individuals, anticholinergic drugs, anticonvulsants like topiramate, following general anesthesia and spherophakia.^[1-3]

Vogt-Koyanagi-Harada (VKH) disease is a bilateral, chronic intraocular inflammation associated with neurological and cutaneous abnormalities including headache, tinnitus, vitiligo, poliosis, and alopecia.^[4,5] Decreased vision with headache is the most commonly presenting complaint and anterior chamber/vitreous cells are the most common sign of acute VKH.^[5] Association of glaucoma/ocular hypertension with VKH has been studied across Asian/Western population with reported prevalence of 10-62%.^[6-8] Rarely, it can manifest as an AAC.^[9-11] A 10-year retrospective review of VKH patients by Yang et al. revealed 8 (0.08%) cases which were misdiagnosed as APAC.^[9]

We describe a rare presentation of VKH as AAC along with a short literature review.

Case Report

A 52-year-old female presented with complaints of headache, decreased vision, and redness in both eyes for 1 week. Ocular examination at presentation revealed best-corrected visual acuity (BCVA) of 6/36 OU and intraocular pressure (IOP) of 52 and 62 mmHg on Goldmann applanation tonometry in the right and left eye, respectively. Both eyes showed mild ciliary flush with corneal edema, diffuse granulomatous keratic precipitates (kps), mild flare, and shallow anterior chamber, with sluggishly reacting pupils [Figure 1a]. Gonioscopy revealed bilateral closed angles in all four quadrants.

Fundus examination revealed blurring of the nasal optic disc margin [Figure 1b] with shallow retinal detachment in the inferior periphery in both eyes. There was no vitritis. In view of the suspicion of VKH (elderly female, inferior exudative detachment, and disc edema), B-scan ultrasonography (USG) was done which revealed choroidal thickening (2.1 mm) inthe peripapillary area [Figure 1c]. Ultrasound biomicroscopy(UBM) showed supraciliary fluid with closed angles in both theeyes [Figure 1d].

Keeping bilateral AAC in mind, she was questioned aboutdrug intake in the past. There was no history of psychotropicdrug intake, recent general anesthesia, or snakebite.

She was started on oral acetazolamide, timolol, and brimonidineeye drops in both eyes. IOP reduced to 36 mmHg in both eyes.

Based on the presence of bilateral granulomatous iridocyclitis,bilateral exudative retinal detachment, and ultrasonic findings ofperipapillary thickening, we made a presumptive diagnosis ofVKH disease and started the patient on oral steroids 1 mg/kgbody weight in combination with topical steroids betamethasone1 hourly and atropine 1% eye drops 3 times a day. Generalphysical examination, as well as neurological, dermatological,and auditory examinations for VKH syndrome, was all normal.

Within 3 days of therapy, BCVA improved to 6/9 in botheyes, IOP was 10 mmHg, with clear cornea and open angles[Figure 2a]. Fundus showed resolution of the exudativedetachment [Figure 2b] and the disc margins were clear. UBMshowed marked reduction in supraciliary fluid [Figure 2c, arrow].

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All antiglaucoma treatment could be stopped with controlledIOP, and immunosuppression therapy for VKH was initiated.

Discussion

Secondary angle-closure glaucoma due to supraciliary fluidcausing anterior rotation of the ciliary body may present as anAAC attack.^[1-3] Prompt recognition of the condition is imperativefor optimum management. It is important to understand thatthis condition requires steroids and cycloplegics in contrastto the usual treatment of pilocarpine and LPI in primary angleclosure. The latter treatment would prove to be disastrous due toexacerbated inflammation and further anterior movement of theiris-lens diaphragm.

In our patient, despite the presentation of a bilateral AAC,the recognition of granulomatous inflammation in anteriorchamber and increased choroidal thickening with exudativeretinal detachment supported the diagnosis of VKH. Promptmanagement resulted in resolution of the attack. We havepreviously reported two patients presenting with bilateral AAC,who were subsequently found to have acute retinal necrosis^[12]and tubercular granuloma,^[13] respectively, illustrating thatposterior segment inflammatory diseases can have unusualanterior segment manifestations.

Previously published reports on AAC in VKH found it tobe more common in females with associated uveal effusion.Rathinam et al. reported mildly elevated IOP with maximumof 27 mmHg in their series of three patients who did well withsteroids.^[11] Yang et al. retrospectively found 8 eyes of 486eyes VKH misdiagnosed as an angle closure where LPI wasperformed in three eyes which worsened the course until thecorrect diagnosis was established.^[9] UBM was performed in twopatients at 3 weeks, which showed fluid in supraciliary space.Our patient had 60 mmHg of IOP with evident supraciliaryfluid on UBM and choroidal thickening on USG. The presenceof bilateral kps with disc edema in an elderly female raised thesuspicion of VKH.

Glaucoma has been reported to be one of the most commonocular complications of VKH. Pandey et al. found 15.8%prevalence of glaucoma in retrospective review of 448 eyesdiagnosed with VKH over 23 years.^[7] The most commonmechanism was open-angle glaucoma in 46 eyes, (64.8%), angleclosure in 21 eyes (29.6%), and of combined mechanisms in theremainder (4 eyes, 5.6%). Only nine cases of 448 eyes presentedwith angle closure (four at presentation and five during followup)that was managed with LPI. UBM in these cases revealedsupraciliary fluid in only 5/9 (55%) cases.

Bilateral AAC is a rarely seen ocular condition. Variousreported etiologies are drug induced (sulfonamides,phenylephrine, ephedrine, and botulinum toxin), snakebite,uveal effusion, microspherophakia, and VKH.^[1-3,7] Although wedid consider drug induced secondary angle closure, literaturesearch revealed no reports of selective serotonin reuptakeinhibitors (SSRI) to be implicated in the pathogenesis and ourpatient used SSRI in the past. Mean duration of the onset of raisedIOP was 71 days, but earliest has been reported within 1 week ofdrug intake with topiramate.^[2] The presence of granulomatouskps, optic disc staining, and inferior exudative detachment tiltedthe diagnosis toward VKH disease.

Our case highlights the fact that every angle closure isnot a primary angle closure requiring LPI. This is especiallyimportant in countries such as India and China where PACGcomprises half of primary glaucoma encountered and there maybe an increased tendency to go ahead with LPI. In VKH, anautoimmune response is generated against uveal melanocytes,leading to uveal effusion. Secondary angle closure is due toinflammation as well as forward rotation of ciliary body and notpupillary block. Pilocarpine and LPI in such a scenario wouldincrease the dilatation of uveal blood vessels and the resultantangle congestion would be likely to complicate the conditionfurther.

Auxiliary investigations such as UBM and USG had amajor role in reaching diagnosis in such atypical presentation.However, to order these investigations, a high index of suspicionis required. Our case seeks to highlight the possibility of VKHpresenting as bilateral simultaneous AAC in the presence ofintraocular inflammation which responded well to topical andoral steroids, avoiding LPI.

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