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Year : 2014  |  Volume : 2  |  Issue : 3  |  Page : 108-110

Unilateral acute angle-closure glaucoma as an unusual presentation of Vogt–Koyanagi–Harada disease: A diagnostic challenge

Department of Ophthalmology, Vitreoretinal Diseases and Uveitis Division, Alexandria University, Alexandria, Egypt

Date of Web Publication7-Sep-2015

Correspondence Address:
Dr. Mohsen Ahmed Abou Shousha
Department of Ophthalmology, Alexandria Faculty of Medicine, Division of Vitreoretinal Diseases and Uveitis, 6 Chamblion Street, Elazareeta, Alexandria
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Source of Support: Nil, Conflict of Interest: None declared.

DOI: 10.4103/2347-5617.164630

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Vogt–Koyanagi–Harada syndrome is an inflammatory multisystem disorder with ocular, cutaneous and neurological manifestations occurring more commonly in certain dark-skinned ethnic groups such as Asians, patients of Middle-Eastern heritage, and hispanics. Only a few reports have described a masquerade presentation of acute angle closure as an initial manifestation for this uveitic entity, which is typically characterized by panuveitis with serous retinal detachments. This has almost always led to misdiagnosis, unwarranted surgical interventions, and a delay of appropriate timely therapy. In this report, we describe an unusual case of acute unilateral Harada disease presenting as acute angle-closure glaucoma that has remitted following institution of appropriate therapy.

Keywords: Glaucoma, harada, uveitis

How to cite this article:
Shousha MA, Amin RM. Unilateral acute angle-closure glaucoma as an unusual presentation of Vogt–Koyanagi–Harada disease: A diagnostic challenge. Egypt Retina J 2014;2:108-10

How to cite this URL:
Shousha MA, Amin RM. Unilateral acute angle-closure glaucoma as an unusual presentation of Vogt–Koyanagi–Harada disease: A diagnostic challenge. Egypt Retina J [serial online] 2014 [cited 2022 Sep 29];2:108-10. Available from: https://www.egyptretinaj.com/text.asp?2014/2/3/108/164630

  Introduction Top

Vogt–Koyanagi–Harada is a multisystem autoimmune disorder typically characterized by panuveitis with serous retinal detachments. It is often associated with neurologic and cutaneous manifestations including headache, hearing loss, tinnitus, poliosis, and vitiligo. Ophthalmologists should consider VKH syndrome in susceptible patients who present with angle-closure glaucoma as a rare presentation of this uveitic entity. The elevated intraocular pressure (IOP) does not respond to anti-glaucoma or laser therapy, but resolves following treatment with corticosteroids. A major drop of vision inconsistent with the mild to moderate elevation of IOP and the very narrow-angle in these cases is an important clue for diagnosis. In this report, we describe a case of acute Harada disease presenting as unilateral acute angle-closure glaucoma with onset following sildenafil aided sexual intercourse.

  Case Report Top

A previously healthy 32-year-old male patient presented with acute onset of headache and diminution of vision in his left eye 12 h prior to the presentation. He reports 50 mg sildenafil citrate (Viagra®) intake 6 h prior to his visual complaints. On examination, visual acuity (VA) of the affected eye was 0.05 with mild corneal haze, mid-dilated vertically oval pupil, with shallow anterior chamber (AC) (Schaffer Grade 2), 0.5+ AC cells, and IOP of 32 mmHg. VA and IOP in the right eye were 1.0 and 16 mmHg respectively with no AC inflammation and a normal AC depth [[Figure 1] - Panel a and b]. A diagnosis of acute angle closure glaucoma was entertained at that time, and anti-glaucoma measures were instituted including cidamex 250 mg tablets 3 times daily, brimonidine eye drops three times daily and combined timolol maleate 0.5% and dorzolamide eye drop twice daily, which resulted in improvement of corneal edema 48 h later, a mild drop of IOP to 27 mmHg, however, no change in AC depth and failure to relieve the patient's symptoms and visual loss.
Figure 1: Slit lamp photo of the right and left eyes (Panel a and b respectively) showing a shallow anterior chamber (AC) with vertically oval mid-dilated pupil and peripheral iridocorneal contact in the left eye and a normal pupil and AC depth in the right eye. Panel c: Ciliary body swelling with supraciliary fluid collection in combined a/b-scan ultrasonography of the left eye. optical coherence tomography (OCT) showing subfoveal neurosensory detachment (Panel d). Panel e: Anterior segment OCT demonstrating peripheral iridocorneal contact. Panel f: A color fundus photo of the left eye showing mulifocal areas of subretinal fluid collection. Panels g and h display early and late frame fluorescein angiography findings in the left eye at 59 s and 3 min 12 s respectively. Notably, there are early hyperfluorescent dots over the posterior pole with late pooling of the dye in areas of neurosensory detachments

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  Investigations Top

B-scan ultrasonography was done, and revealed ciliary body swelling and choroidal thickening with supraciliary fluid pooling in the left eye [[Figure 1] - Panel c]. Now that the media haze decreased, we opted to do a fluorescein angiography (FA), which revealed early hyperfluorescent dots over the posterior pole and late pooling of the dye in areas of neurosensory detachments, a picture consistent with acute Harada disease. Optical coherence tomography at that time revealed a neuroepithelial detachment at the fovea [[Figure 1] - Panel d-h].

  Treatment Top

Oral systemic steroids was started at a dose of 1 mg/kg prednisolone for 1 week with a drop of IOP to 20 mmHg, deepening of the AC (Schaffer 4), resolution of AC inflammation, however the fundus examination showed a massive inferior exudative bullous retinal detachment [[Figure 2] - Panel a-d] that only started to resolve slowly over 2 weeks after performing a posterior subtenon triamcinolone acetonide 40 mg injection.
Figure 2: Panels a and b showing inferior bullous exudative retinal detachment through fundus biomicroscopy and increased subretinal fluid in follow-up optical coherence tomography 1 week after starting systemic steroid therapy. Panels c-e demonstrating normal anterior chamber (AC) depth, round pupil and normal color fundus photo of the left eye 1 month through treatment. The AC angle is wide open (Panel d). Normal foveal contour and anatomical morphology has been restored (Panel f)

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  Outcome and Follow-up Top

On follow-up 2 months later, there was the complete absorption of subretinal fluid with the restoration of foveal contour and improvement of VA to 0.9 [[Figure 2] - Panel e and f]. During 12 months of follow-up off all therapy, there were no recurrences and IOP was well controlled at 12 mmHg.

  Discussion Top

Few reports described acute Harada disease misdiagnosed initially for acute attacks of angle closure glaucoma in susceptible ethnic groups.[1],[2] The majority of these patients had undergone unwarranted interventions including laser iridotomies, with one patient undergoing enucleation in one eye after failed trabeculectomies.[1] The mean IOP in those patients was 32.9 mmHg. All patients had a shallow AC at presentation, a narrow or closed angle, and complaints of headache and sudden drop of vision in both eyes. B-scan ultrasonography and FA supported the diagnosis. After corticosteroid treatment, the IOP elevation resolved with deepening of the AC and improved VA.

Another two separate reports describe acute angle closure 24 h following sildenafil citrate aided sexual intercourse in two patients.[3],[4] The mechanism of angle closure glaucoma caused by sildenafil citrate; a medication prescribed for erectile dysfunction, is not well understood or proven, but of note was the fact that our patient has had this particular presentation 6 h after sildenafil intake. Apart from transient blurry vision, photophobia, and difficulty in discriminating blue-green light spectrum, previous studies have not shown other drug-induced changes in the anterior segment or the IOP attributed to Sildenafil.[5] We cannot prove a causal relation between Sildenafil intake and our patient's presentation and more is needed to further explore this particular presentation of angle closure glaucoma following the drug intake.

This report of acute angle-closure glaucoma as an initial manifestation of unilateral acute Harada disease draws attention to the spectrum of atypical presentations of certain uveitis entities in susceptible ethnic groups and is the first report to our knowledge of such a presentation following aided sexual intercourse with sildenafil citrate intake.

  Learning Points/Take Home Messages Top

  • Atypical presentations of certain common uveitis entities in susceptible ethnic groups should be included on the differential when meeting such patients
  • Acute VKH at presentation can rarely mimic acute attacks of angle closure glaucoma due to ciliary body swelling and suprachoroidal fluid accumulation. The inconsistency between the amount of IOP elevation and the degree of visual loss, as well as AC depth, can raise suspicion toward the correct diagnosis
  • Given the global variation in incidence, epidemiology, and patterns of uveitis in different parts of the world, knowledge of such data is helpful when caring for patients with intraocular inflammation.

  References Top

Yang P, Liu X, Zhou H, Guo W, Zhou C, Kijlstra A. Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset. Clin Experiment Ophthalmol 2011;39:639-47.  Back to cited text no. 1
Yao J, Chen Y, Shao T, Ling Z, Wang W, Qian S. Bilateral acute angle closure glaucoma as a presentation of Vogt-Koyanagi-Harada syndrome in four Chinese patients: A small case series. Ocul Immunol Inflamm 2013;21:286-91.  Back to cited text no. 2
Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexual intercourse. Acta Ophthalmol Scand 2007;85:229-30.  Back to cited text no. 3
Lee WJ, Seong M. Bilateral simultaneous acute angle closure glaucoma following sexual intercourse aided by sildenafil citrate. J Korean Ophthalmol Soc 2011;52:1123-7.  Back to cited text no. 4
Laties AM, Fraunfelder FT. Ocular safety of Viagra, (sildenafil citrate). Trans Am Ophthalmol Soc 1999;97:115-25.  Back to cited text no. 5


  [Figure 1], [Figure 2]

This article has been cited by
1 Vogt-Koyanagi-Harada disease associated with anterior ischemic optic neuropathy in a young woman presenting as acute angle closure glaucoma
Sagarika Patyal,Ritesh Narula,Mithun Thulasidas
Indian Journal of Ophthalmology. 2020; 68(9): 1937
[Pubmed] | [DOI]


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