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Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome

Received: 5 February 2021    Accepted: 14 February 2021    Published: 26 February 2021
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Abstract

Background: To present myriad and unique presentation of Vogt-Kayanagi-Harada Disease (VKHD) and Vogt-Koyanagi-Harada (VKH) resembling syndrome. Methods: This is observational series of four cases of VKH disease with variable unique visual presentation which ranged from blurring of vision, tinnitus, hot flushes on face and abdomen, presence of floaters with hedache, breathlessness and history of weight loss. The patient age ranged from 14-56 years. Symptoms developed acutely ranging from 4-12 weeks. Visual acuity varied from 1/60 to 6/6 on Snellen chart. A detailed ophthalmic evaluation, radio imaging and neuroimaging were done as needed on patients. FFA confirmed disc leakage, subretinal pooling of dye in posterior pole and pin point leaks in three cases. The fourth case revealed dilated capillaries, pin point leaks outside zone of hyperflourescence and presence of leopard spots.USG B-scan in same case showed choroidal thickening and an oval hypointense juxtapappilary lesion. OCT-macula revealed a convex retinal profile, thickening of retinal pigment epithelium choriocapillaries complex, multiple subretinal low reflective areas consisting with serous neurosensory detachment and hyper reflective spots in inner retinal areas consistent with findings suggestive of metastasis. All four patients presented in acute uveitic stage. Barring one patient, three patients had bilateral disease. Incomplete VKH disease was diagnosed in three cases. Metastatic intraocular carcinoma with multiorgan involvement with primary in cervix was diagnosed in one case. Two cases of VKH disease were in remission having responded well to oral steroids and immunesuppresive therapy. One case passed to chronic stage with visual deterioration. One case with multi-organ metastasis went on to receive chemotherapy, brachytherapy, whole body and pelvic radiation. Conclusions: VKH disease has variable presentation and a high degree of suspicion is needed to differentiate it from similar occurring malignant and non-malignant intraocular inflammation. Early staging of VKH disease helps in planning adequate treatment leading to greater improvement in disease outcome and simultaneously preventing progression to chronic stage.

Published in International Journal of Ophthalmology & Visual Science (Volume 6, Issue 1)
DOI 10.11648/j.ijovs.20210601.15
Page(s) 29-40
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

VKHD, Adolescent, Uveitis, Tinnitus, Revised Diagnostic Criteria, FFA, Immunesuppression, Choroid, Metastasis

References
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Cite This Article
  • APA Style

    Deepesh Arora, Kanika Gupta, Ankit Parasher, Deepak Anthwal, Niyaaz Ahmad, et al. (2021). Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome. International Journal of Ophthalmology & Visual Science, 6(1), 29-40. https://doi.org/10.11648/j.ijovs.20210601.15

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    ACS Style

    Deepesh Arora; Kanika Gupta; Ankit Parasher; Deepak Anthwal; Niyaaz Ahmad, et al. Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome. Int. J. Ophthalmol. Vis. Sci. 2021, 6(1), 29-40. doi: 10.11648/j.ijovs.20210601.15

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    AMA Style

    Deepesh Arora, Kanika Gupta, Ankit Parasher, Deepak Anthwal, Niyaaz Ahmad, et al. Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome. Int J Ophthalmol Vis Sci. 2021;6(1):29-40. doi: 10.11648/j.ijovs.20210601.15

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  • @article{10.11648/j.ijovs.20210601.15,
      author = {Deepesh Arora and Kanika Gupta and Ankit Parasher and Deepak Anthwal and Niyaaz Ahmad and Dinesh Sharma},
      title = {Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome},
      journal = {International Journal of Ophthalmology & Visual Science},
      volume = {6},
      number = {1},
      pages = {29-40},
      doi = {10.11648/j.ijovs.20210601.15},
      url = {https://doi.org/10.11648/j.ijovs.20210601.15},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijovs.20210601.15},
      abstract = {Background: To present myriad and unique presentation of Vogt-Kayanagi-Harada Disease (VKHD) and Vogt-Koyanagi-Harada (VKH) resembling syndrome. Methods: This is observational series of four cases of VKH disease with variable unique visual presentation which ranged from blurring of vision, tinnitus, hot flushes on face and abdomen, presence of floaters with hedache, breathlessness and history of weight loss. The patient age ranged from 14-56 years. Symptoms developed acutely ranging from 4-12 weeks. Visual acuity varied from 1/60 to 6/6 on Snellen chart. A detailed ophthalmic evaluation, radio imaging and neuroimaging were done as needed on patients. FFA confirmed disc leakage, subretinal pooling of dye in posterior pole and pin point leaks in three cases. The fourth case revealed dilated capillaries, pin point leaks outside zone of hyperflourescence and presence of leopard spots.USG B-scan in same case showed choroidal thickening and an oval hypointense juxtapappilary lesion. OCT-macula revealed a convex retinal profile, thickening of retinal pigment epithelium choriocapillaries complex, multiple subretinal low reflective areas consisting with serous neurosensory detachment and hyper reflective spots in inner retinal areas consistent with findings suggestive of metastasis. All four patients presented in acute uveitic stage. Barring one patient, three patients had bilateral disease. Incomplete VKH disease was diagnosed in three cases. Metastatic intraocular carcinoma with multiorgan involvement with primary in cervix was diagnosed in one case. Two cases of VKH disease were in remission having responded well to oral steroids and immunesuppresive therapy. One case passed to chronic stage with visual deterioration. One case with multi-organ metastasis went on to receive chemotherapy, brachytherapy, whole body and pelvic radiation. Conclusions: VKH disease has variable presentation and a high degree of suspicion is needed to differentiate it from similar occurring malignant and non-malignant intraocular inflammation. Early staging of VKH disease helps in planning adequate treatment leading to greater improvement in disease outcome and simultaneously preventing progression to chronic stage.},
     year = {2021}
    }
    

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  • TY  - JOUR
    T1  - Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome
    AU  - Deepesh Arora
    AU  - Kanika Gupta
    AU  - Ankit Parasher
    AU  - Deepak Anthwal
    AU  - Niyaaz Ahmad
    AU  - Dinesh Sharma
    Y1  - 2021/02/26
    PY  - 2021
    N1  - https://doi.org/10.11648/j.ijovs.20210601.15
    DO  - 10.11648/j.ijovs.20210601.15
    T2  - International Journal of Ophthalmology & Visual Science
    JF  - International Journal of Ophthalmology & Visual Science
    JO  - International Journal of Ophthalmology & Visual Science
    SP  - 29
    EP  - 40
    PB  - Science Publishing Group
    SN  - 2637-3858
    UR  - https://doi.org/10.11648/j.ijovs.20210601.15
    AB  - Background: To present myriad and unique presentation of Vogt-Kayanagi-Harada Disease (VKHD) and Vogt-Koyanagi-Harada (VKH) resembling syndrome. Methods: This is observational series of four cases of VKH disease with variable unique visual presentation which ranged from blurring of vision, tinnitus, hot flushes on face and abdomen, presence of floaters with hedache, breathlessness and history of weight loss. The patient age ranged from 14-56 years. Symptoms developed acutely ranging from 4-12 weeks. Visual acuity varied from 1/60 to 6/6 on Snellen chart. A detailed ophthalmic evaluation, radio imaging and neuroimaging were done as needed on patients. FFA confirmed disc leakage, subretinal pooling of dye in posterior pole and pin point leaks in three cases. The fourth case revealed dilated capillaries, pin point leaks outside zone of hyperflourescence and presence of leopard spots.USG B-scan in same case showed choroidal thickening and an oval hypointense juxtapappilary lesion. OCT-macula revealed a convex retinal profile, thickening of retinal pigment epithelium choriocapillaries complex, multiple subretinal low reflective areas consisting with serous neurosensory detachment and hyper reflective spots in inner retinal areas consistent with findings suggestive of metastasis. All four patients presented in acute uveitic stage. Barring one patient, three patients had bilateral disease. Incomplete VKH disease was diagnosed in three cases. Metastatic intraocular carcinoma with multiorgan involvement with primary in cervix was diagnosed in one case. Two cases of VKH disease were in remission having responded well to oral steroids and immunesuppresive therapy. One case passed to chronic stage with visual deterioration. One case with multi-organ metastasis went on to receive chemotherapy, brachytherapy, whole body and pelvic radiation. Conclusions: VKH disease has variable presentation and a high degree of suspicion is needed to differentiate it from similar occurring malignant and non-malignant intraocular inflammation. Early staging of VKH disease helps in planning adequate treatment leading to greater improvement in disease outcome and simultaneously preventing progression to chronic stage.
    VL  - 6
    IS  - 1
    ER  - 

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Author Information
  • Department of Vitreo-retina, Amritsar Eye clinic, Dehradun, India

  • Department of Paediatric Ophthalmology, Amritsar Eye Clinic, Dehradun, India

  • Department of MRI and CT Imaging, Suri Diagnostic and Imaging Centre, Dehradun, India

  • Department of Vitreo-retina, Arvind Eye Hospital, Coimbatore, India

  • Department of Medicine, Shri Guru Ram Rai Institute of Medical and Health Sciences and Shri Mahant Indiresh Hospital, Dehradun, India

  • Amritsar Eye Clinic, Dehradun, India

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