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Year : 2019  |  Volume : 57  |  Issue : 2  |  Page : 160-162

A rare pediatric case of probable Vogt–Koyanagi–Harada syndrome

1 Department of Squint and Neuro-Ophthalmology, Regional Instititute of Ophthalmology and Government Ophthalmic Hospital, Chennai, Tamil Nadu, India
2 Department of Ophthalmology, Dr. MGR Medical University, Chennai, Tamil Nadu, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Malarvizhi Raman
RIO GOH, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_37_19

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We report a rare case of probable Vogt–Koyanagi–Harada (VKH) syndrome in a 10-year-old child who presented with visual acuity of only perception of light present in both eyes. The anterior segment examination showed cells, flare (4+), fine keratic precipitates over back of the cornea, posterior synechiae, posterior subcapsular cataract, and fundus showing hyperemic disc with blurred margins. B-scan showed shallow retinal detachment and choroidal thickening. Bilateral panuveitis with normal serological and systemic screening was favoring a diagnosis of probable VKH syndrome. The patient was treated with intravenous methylprednisolone followed by oral prednisolone. Visual acuity improved to 6/24 BE.

Keywords: Panuveitis, pediatric, Vogt–Koyanagi–Harada

How to cite this article:
Raman M, Vasumathi K, Anuradha A, Sheela S, Nisha C. A rare pediatric case of probable Vogt–Koyanagi–Harada syndrome. TNOA J Ophthalmic Sci Res 2019;57:160-2

How to cite this URL:
Raman M, Vasumathi K, Anuradha A, Sheela S, Nisha C. A rare pediatric case of probable Vogt–Koyanagi–Harada syndrome. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2020 Sep 23];57:160-2. Available from: http://www.tnoajosr.com/text.asp?2019/57/2/160/266391

  Introduction Top

Vogt–Koyanagi–Harada (VKH) disease is a chronic, bilateral, granulomatous panuveitis and exudative retinal detachment associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs.[1] The exact cause remains unknown, but evidence suggests that it involves a T-lymphocyte-mediated autoimmune process directed against antigens associated with melanocytes. Several studies demonstrated that tyrosinase family proteins are the antigens associated with VKH syndrome disease.[2],[3] VKH syndrome predominantly occurs in women in their third to fourth decades at the onset of illness. VKH syndrome can occur in pediatric population (about 3%, with an approximate incidence of one case/year), and the prognosis is variable.[4]

  Case Report Top

A 10-year-old child presented with defective vision in both eyes (BE). There was no history of penetrating intraocular trauma or surgery. On examination, visual acuity was only perception of light present in both eyes. Anterior segment examination showed [Figure 1] cells, flare (4+), fine keratic precipitates over the back of cornea with posterior synechiae, festooned pupil and posterior subcapsular cataract. Fundus examination [Figure 2] showed hazy media with a hyperemic disc with blurred margins. B-scan [Figure 3] showed vitritis, shallow retinal detachment with choroidal thickening. Fundus fluorescein angiography was deferred, considering the age of the child and hazy media. Investigations such as complete blood count, peripheral smear, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, TORCH screening, Mantoux test, high-resolution computed tomography chest, angiotensin-converting enzyme levels, and human leukocyte antigens (HLA) B27 were within normal limits. Bilateral panuveitis with serous shallow retinal detachment with normal serological and systemic screening was favoring a diagnosis of probable VKH syndrome. Hence, intravenous methylprednisolone 20 mg/kg/day for 3 days followed by maintenance dose of oral prednisolone of 1 mg/kg/day was given. After 1 week of steroids, visual acuity improved to 6/60, and spectral domain optical coherence tomography [Figure 4] showed bumpy elevations at the level of retinal pigment epithelium without serous detachment. At the end of 6 weeks, patient was started on oral prednisolone in maintenance dose of 40 mg. After steroid therapy, Visual acuity improved to 6/24 NIG, serous detachment settled, there was a decrease in choroidal thickening and dsappearance of the bumpy elevation in optical coherence tomography. Immunosuppressive, oral cyclosporine 5 mg/kg/day was started with tapering of oral prednisolone to 20 mg/day with periodic review with the pediatrician. After six months of presentation, the child is maintaining visual acuity of 6/24 NIG NIP with posterior subcapsular cataract. Fundus shows sunset glow appearance. Posterior synechiolysis with lensectomy and anterior vitrectomy has to be done for this patient.
Figure 1: Anterior segment showing posterior synechiae with posterior subcapsular cataract

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Figure 2: Fundus showing hazy media with hyperemic disc and blurred margin

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Figure 3: B-scan showing choroidal thickening with associated serous detachment

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Figure 4: Optical coherence tomography showing bumpy elevation at retinal pigment epithelium without serous detachment

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  Discussion Top

VKH syndrome is more common among oriental and dark-skinned individuals. There is a clinical association between HLA DRB1*0405 and HLA DRB1*0410 and VKH.[5] The clinical course of VKH syndrome in children is more aggressive than in adults. The microstructural changes in the retina and the retinal pigment epithelium such as undulations and bumps corresponding to the choroidal striations have been reported.[6],[7] Glaucoma is the most common complication in VKH syndrome because of immune-mediated damage in melanin-bearing cells in the trabecular meshwork.[8],[9] Long-term systemic steroids pose considerable risks in childhood, and they do not adequately control the intraocular inflammation or prevent the ocular complications of VKH syndrome. Immunosuppressants such as methotrexate, cyclosporine, and mycophenolate have to be considered for treating VKH syndrome.[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol 1995;39:265-92.  Back to cited text no. 1
Kobayashi H, Kokubo T, Takahashi M, Sato K, Miyokawa N, Kimura S, et al. Tyrosinase epitope recognized by an HLA-DR-restricted T-cell line from a Vogt-Koyanagi-Harada disease patient. Immunogenetics 1998;47:398-403.  Back to cited text no. 2
Yamaki K, Gocho K, Hayakawa K, Kondo I, Sakuragi S. Tyrosinase family proteins are antigens specific to Vogt-Koyanagi-Harada disease. J Immunol 2000;165:7323-9.  Back to cited text no. 3
Read RW, Rechodouni A, Butani N, Johnston R, LaBree LD, Smith RE, et al. Complications and prognostic factors in Vogt-Koyanagi-Harada disease. Am J Ophthalmol 2001;131:599-606.  Back to cited text no. 4
Shindo Y, Inoko H, Yamamoto T, Ohno S. HLA-DRB1 typing of Vogt-Koyanagi-Harada's disease by PCR-RFLP and the strong association with DRB1*0405 and DRB1*0410. Br J Ophthalmol 1994;78:223-6.  Back to cited text no. 5
Ikewaki J, Kimoto K, Choshi T, Nagata M, Motomura Y, Tamura K, et al. Optical coherence tomographic assessment of dynamic macular changes in patients with Vogt-Koyanagi-Harada disease. Int Ophthalmol 2011;31:9-13.  Back to cited text no. 6
Gupta V, Gupta A, Gupta P, Sharma A. Spectral-domain cirrus optical coherence tomography of choroidal striations seen in the acute stage of Vogt-Koyanagi-Harada disease. Am J Ophthalmol 2009;147:148-53.e2.  Back to cited text no. 7
Jampel HD, Jabs DA, Quigley HA. Trabeculectomy with 5-fluorouracil for adult inflammatory glaucoma. Am J Ophthalmol 1990;109:168-73.  Back to cited text no. 8
American Academy of Ophthalmology. Intraocular inflammation and uveitis. In: Textbook of Ophthalmology. Ch. 6. Non infectitious (autoimmune) ocular inflammatory disease panuveitis: American Academy of Ophthalmology; 2011-2012. p. 189.  Back to cited text no. 9
Brad bowling, Kanski's Clinical Ophthalmology – Uveitis vogt koyanagi harada syndrome Elsevier publication eighth edition 2016. p. 416.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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