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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 60  |  Issue : 4  |  Page : 312-314

Gleaming in the gloom—A striking bilateral multimodal imaging canvas of vogt-koyanagi-harada syndrome in a patient with poor adherence to therapy


1 Department of Glaucoma and Research, Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
2 Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
3 Department of Optometry and Visual Science, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
4 Department of Vitreo-Retinal Surgery, Mahathma Eye Hospital Private Limited, Trichy, Tamil Nadu, India
5 Vitreo-Retinal Fellow, Aravind Eye Care System, Coimbatore, Tamil Nadu, India

Date of Submission11-May-2022
Date of Decision17-Aug-2022
Date of Acceptance21-Aug-2022
Date of Web Publication19-Dec-2022

Correspondence Address:
Prasanna Venkatesh Ramesh
Mahathma Eye Hospital Private Limited, No. 6, Tennur, Seshapuram, Trichy – 620 017, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_47_22

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  Abstract 


Vogt-Koyanagi-Harada (VKH) syndrome is a rare bilateral granulomatous panuveitis typically affecting the more pigmented races, and women more than men. VKH syndrome is theorized due to a T-cell-mediated autoimmune reaction against antigens related to melanin and melanocytes, that may happen due to cutaneous or viral triggers. Along with the clinical evaluation, it is mandatory to utilise additional investigative modalities such as fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), optical coherence tomography (OCT) and ultrasonography, which will help pick up pathognomonic signs, that can be missed on clinical evaluation. In this manuscript, we have reported striking multimodal images of a VKH patient with poor adherence; and have emphasized early detection, initiation of therapy without delay, aggressive therapy, good compliance, very slow tapering of oral steroids and use of immunosuppressants for a good visual prognosis.

Keywords: Adherence, FFA, ICGA, Multimodal imaging, VKH syndrome


How to cite this article:
Ramesh PV, Ramesh SV, Devadas AK, Ray P, Balamurugan A, Bavaharan B, Balasubramaniam P. Gleaming in the gloom—A striking bilateral multimodal imaging canvas of vogt-koyanagi-harada syndrome in a patient with poor adherence to therapy. TNOA J Ophthalmic Sci Res 2022;60:312-4

How to cite this URL:
Ramesh PV, Ramesh SV, Devadas AK, Ray P, Balamurugan A, Bavaharan B, Balasubramaniam P. Gleaming in the gloom—A striking bilateral multimodal imaging canvas of vogt-koyanagi-harada syndrome in a patient with poor adherence to therapy. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2023 Feb 8];60:312-4. Available from: https://www.tnoajosr.com/text.asp?2022/60/4/312/364248



A 35-year-old female patient came to the outpatient department with a sudden loss of vision in her left eye (OS). On evaluation, the right eye (OD) was within normal limits [Figure 1]a with best corrected visual acuity (BCVA) of 20/20, and OS revealed a normal anterior segment with multiple pockets of subretinal fluids (SRF) all over the posterior pole [Figure 1]b, with BCVA of 20/60. Optical coherence tomography (OCT) revealed multiple SRF pockets, increased choroidal thickness and retinal pigment epithelial (RPE) undulations [Figure 1]c and [Figure 1]d with all classical features of Vogt-Koyanagi-Harada (VKH) syndrome. A set of tailored blood investigations were done to rule out other systemic causes and was found to be within normal limits. The patient was a known diabetic on treatment, with no other systemic illness or history of trauma. As the VKH syndrome was in its acute phase, intravenous (IV) methylprednisolone 1 gram over 1 hour was administered under a physician's supervision for 3 days, after contraindications to it were ruled out, followed by high-dose systemic oral prednisolone 60 mg/day.
Figure 1: (a) Colour fundus photograph of the right eye (OD) showing normal findings. (b) Color fundus photograph of the left eye (OS) showing multiple pockets of subretinal fluids (SRF) all over the posterior pole. (c) Optical coherence tomography (OCT) of the macula shows multiple pockets of SRF (red asterisks) over the posterior pole with retinal pigment epithelium (RPE) undulations. (d) OCT of ONH shows SRF collection pockets (red asterisk) surrounding the disc with RPE undulations

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Following therapy, there was a resolution of VKH features [Figure 2]a but subtle RPE undulations [Figure 2]b and disc oedema [Figure 2]c developed in OS, which were not present on the previous visit. Fundus fluorescein angiography (FFA) with indocyanine green angiography (ICGA) was performed [Figure 3] and [Figure 4] during the next visit. The patient also started developing bilateral anterior uveitis with cells 2+ and flare 1+, for which she was started on topical steroids along with systemic oral prednisolone 60 mg/day (i.e., 1 mg/kg body weight as the weight of the patient was 60 kg). The BCVA improved to 20/20 in OS with a resolution of signs at 2-month follow-ups. However, the patient had poor medication adherence and she subsequently developed multiple SRF pockets in OD [Figure 5]. The patient was advised to restart her medications and was put additively on an immunosuppressive agent (Azathioprine 50 mg/day).
Figure 2: (a) Colour fundus photograph of OD post systemic steroid therapy showing resolving features of VKH (red arrow) with disc oedema (green arrow) at 2 weeks follow-up. (b) OCT macula of OD showing resolving features of VKH with subtle RPE undulations at 2 weeks follow-up post systemic therapy. (c) OCT of ONH showing disc oedema at 2 weeks follow-up post systemic therapy

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Figure 3: Fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) at 4-week follow-up period after systemic therapy. (a) FFA (left side) of OD shown in venous phase and ICGA (right side) of OD showing multiple hypofluorescent dark spots and fuzzy vascular pattern of large stromal vessels. (b) FFA (left side) of OS in arteriovenous phase and ICGA (right side) showing multiple hypofluorescent dark spots and fuzzy vascular pattern of large stromal vessels

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Figure 4: FFA and ICGA at 4 weeks follow-up period after systemic therapy. (a) FFA (left side) of OD shows few pinpoint leakages in the macula, and ICGA (right side) of OD shows multiple hypofluorescent dark spots. (b) FFA (left side) of OS shows multiple pinpoint leakages in the retina and leakage from the disc, and ICGA (right side) of OS shows multiple hypofluorescent dark spots

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Figure 5: (a) Colour fundus photograph of OD showing recurrent VKH features (red arrow) at 2 months follow-up after systemic therapy. (b) Colour fundus photograph of OS showing resolving disc oedema (green arrow). (c) OCT macula OD showing VKH features (red asterisk) at 2 months follow-up after systemic therapy. (d) OCT macula of OS showing resolved features of VKH at 2 months follow-up after systemic therapy

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In this manuscript, we have reported a striking multimodal imaging canvas of a case of VKH syndrome, which developed in OS initially and later involved OD due to poor adherence to medications. Immunosuppressants were added later in this case; however, some clinicians carry an opinion to start immunosuppressants with steroids at the initial presentation of VKH syndrome. Also, as the patient was a known diabetic, an early start with an immunosuppressant with intravitreal dexamethasone implant is another school of thought for treating this patient, thus reducing the systemic dosage of steroids.

Nevertheless, the patient showed a good prognosis when compliant with therapy. The visual prognosis of VKH is dependent on the duration and number of recurrent episodes of inflammation, the number of complications, age at disease onset, the median duration of the disease and time of initiating therapy.[1],[2],[3],[4] Better the visual acuity at presentation, more likely the final visual acuity is to improve, as seen in this case.[4],[5]

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest



 
  References Top

1.
Gupta V, Gupta A, Bambery P, Radotra BD, Panday SS. Vogt-koyanagi-harada syndrome following injury-induced progressive vitiligo. Indian J Ophthalmol 2001;49:53.  Back to cited text no. 1
    
2.
Priya D, Sudharshan S, Biswas J. Management of a rare presentation of Vogt-Koyanagi-Harada disease in human immunodeficiency virus/acquired immunodeficiency disease syndrome patient. Indian J Ophthalmol 2017;65:413-6.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Dutta Majumder P, Shah A, Kaushik V. Tofacitinib in Vogt-Koyanagi-Harada disease. Indian J Ophthalmol 2020;68:1938-9.  Back to cited text no. 3
    
4.
Nagpal MP, Bhatt KJ, Mehrotra NS, Goswami SD. Angiographic and spectral domain optical coherence tomography features in case of Vogt-Koyanagi-Harada disease. Indian J Ophthalmol 2015;63:162-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Mohan H, Gupta AN. Vogt-Koyanagi syndrome. Indian J Ophthalmol 1967;15:117-9.  Back to cited text no. 5
  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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