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Year : 2021  |  Volume : 9  |  Issue : 1  |  Page : 27-29

An unusual presentation of Vogt-Koyanagi-Harada disease

Department of Vitreo Retina, Sankara Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission31-Oct-2019
Date of Decision29-Oct-2020
Date of Acceptance15-Dec-2020
Date of Web Publication10-Apr-2021

Correspondence Address:
G Geetha
Sankara Eye Hospital, Sathy Road, Sivanandapuram, Coimbatore - 641 035, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_84_19

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We report a rare case of bilateral Vogt-Koyanagi-Harada (VKH) disease coexisting with sub hyaloid hemorrhage in one eye, in a 59-year-old female who presented with diminution of vision in both eyes, right more than left. The anterior segment examination was within normal limits. Fundus examination and imaging revealed features typical of VKH in one eye and these features masked by subhyaloid hemorrhage in the other eye. Varied differential diagnoses appropriately ruled out, subjective and clinical improvement with systemic steroids hits the bull's eye diagnosis of probable VKH with early disease manifestation coexisting with subhyaloid hemorrhage secondary to valsalva maneuver.

Keywords: Subhyaloid hemorrhage, valsalva manoeuvre, Vogt-Koyanagi-Harada disease

How to cite this article:
Keerthhi Dhevi R S, Geetha G, Shanker M P, Anusha V. An unusual presentation of Vogt-Koyanagi-Harada disease. J Clin Ophthalmol Res 2021;9:27-9

How to cite this URL:
Keerthhi Dhevi R S, Geetha G, Shanker M P, Anusha V. An unusual presentation of Vogt-Koyanagi-Harada disease. J Clin Ophthalmol Res [serial online] 2021 [cited 2022 Aug 13];9:27-9. Available from: https://www.jcor.in/text.asp?2021/9/1/27/313480

Serous retinal detachment is characterized by fluid accumulation in the potential space between the macula and underlying retinal pigment epithelium (RPE). The differential diagnosis for serous retinal detachments can be divided into vascular, inflammatory, neoplastic, and congenital etiologies.[1] A subhyaloid hemorrhage is an intraocular collection of blood that remains contained in a self-created, previously nonexistent space, usually between the posterior limiting layer of the vitreous and the retina, and can lead to sudden and severe loss of vision when it occurs in the macula.[2] It is important to differentiate a malignant and potentially lethal condition from a benign condition, as timely intervention could prevent significant morbidity and mortality.

Here, we present a rare case of bilateral multifocal serous retinal detachment which was masked by subhyaloid hemorrhage in one eye, necessitating thorough evaluation and appropriate treatment.

  Case Report Top

A 59-year-old nondiabetic, nonhypertensive female presented with a 10-day history of gradual progressive blurry vision in both eyes and a further sudden drop in vision in her right eye (OD) for 3 days. She denied a history of ocular trauma, surgery, fever, weight loss, skin rashes, hearing changes, tinnitus, and prior vision problems. She gave no history suggestive of tuberculosis (TB), autoimmune disorders, or malignancy. She gave a history of an episode of upper respiratory infection 5 days before her symptoms which subsided on itself. At the initial visit, the best-corrected visual acuity (BCVA) in the OD was 1/60 and in the left eye (OS) 3/60. Pupillary function, intraocular pressure, and biomicroscopic examination were normal. OD fundoscopy showed disc hyperemia with boat-shaped subhyaloid hemorrhage centered at the fovea and superficial flame-shaped hemorrhages around the disc. OS showed vitreous cells 1+, disc hyperemia, and multiple yellowish subretinal lesions throughout the posterior pole [Figure 1]. B-scan ultrasonography revealed diffuse low-to-medium reflective thickening of the posterior choroid and mild vitreous opacities in OS and localized elevation of blood in the subhyaloid space in the OD. There was no evidence of peripapillary retinochoroidal thickening in both eyes. A fundus fluorescein angiography (FA) of OD showed disc staining and boat-shaped blocked fluorescence centered over macula suggestive of subhyaloid hemorrhage blocking the details of the underlying posterior pole and OS revealed focal areas of delay in choroidal perfusion in the early phase, multifocal areas of pinpoint leakage, large placoid areas of hyperfluorescence in the late phase, pooling of dye within subretinal space, and optic disc staining [Figure 2]. Optical coherence tomography showed preretinal hemorrhage masking the underlying details in the OD and serous detachment in the OS. Inspite of the typical presentation of Vogt-Koyanagi-Harada (VKH), considering the atypical presentation with subhyaloid hemorrhage, superficial retinal hemorrhages in one eye and serous detachment in other eye, possibilities of inflammation, infection, blood dyscrasias, and metastasis were sought. Laboratory investigations including complete hemogram, erythrocyte sedimentation rate, peripheral smear, Mantoux and Quantiferon-TB gold were sought, to rule out blood dyscrasias, and infections including TB. Possibility of metastasis was ruled out with physician opinion and positron emission tomography-computed tomography scan. The patient was started on oral steroids after ruling out TB. One week later, her BCVA was 6/24 in OD and 6/18 in OS. The patient was symptomatically better. On examination, the subhyaloid hemorrhage in OD had resolved, revealing the scattered hemorrhages and multifocal sensory detachment. OS showed resolving sensory detachment [Figure 3]. We continued her on tapering dose of steroids. On 2-month follow-up, her BCVA is 6/12 in OD and 6/9 in OS.
Figure 1: (a) Fundus photo of the right eye showing disc hyperemia with subhyaloid hemorrhage and superficial hemorrhages around the disc. (b) The left eye showing disc hyperemia and multiple yellowish subretinal lesions throughout the posterior pole. (c and d) Demonstrates the optical coherence tomography changes

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Figure 2: (a) Early phase fluorescein angiogram of the right eye showing sub hyaloid hemorrhage and left eye revealing multifocal areas of pinpoint leakage. (b) Late phase fluorescein angiogram showing disc staining in both eyes and pooling of dye in the left eye

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Figure 3: (a) Fundus photo 1 week later showing resolved subhyaloid hemorrhage in the right eye, revealing the scattered hemorrhages and multifocal sensory detachment. (b) showing resolving sensory detachment in the left eye. (c and d) Demonstrates the optical coherence tomography changes

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The subjective and clinical improvement with steroids and the absence of other pathology as the cause for unilateral subhyaloid hemorrhage zeroed in the diagnosis of bilateral VKH, with the possibility of Valsalva or posterior vitreous detachment (PVD) induced subhyaloid hemorrhage that possibly coexisted with the condition. The presence of superficial hemorrhages helped us zero into the diagnosis of Valsalva-induced hemorrhage.

  Discussion Top

Serous macular detachment is a separation of the neurosensory layer from RPE occurring at the macula. Although the exact mechanism of development is not known, it may occur in retinal or choroidal vascular leakage with RPE dysfunction.[3] It occurs in a variety of benign and malignant conditions including chronic central serous chorioretinopathy, posterior scleritis, multifocal choroiditis, uveal melanoma, metastatic tumor, blood dyscrasias including anemia, leukemia, thrombocytopenic purpura, severe hypertensive choroidopathy, VKH disease, and systemic diseases such as myxedema and multiple myeloma.[4]

Subhyaloid hemorrhage has been reported in hypertensive and diabetic retinopathy, blood disorders, retinal macroaneurysm, chorioretinitis, metastases, TB, trauma, Valsalva maneuvers, Terson's syndrome, and secondary to PVD.[2]

Ocular manifestation may be the first sign of a critical systemic disease such as blood dyscrasias and metastases.[5],[6] Prompt recognition of the disease is important for early systemic treatment and restoration of visual function.

In our case, the coexistence of the unilateral subhyaloid hemorrhage confused the picture opening up varied differentials. With general and specific investigations the diagnosis was zeroed into bilateral probable VKH with early manifestation with subhyaloid hemorrhage secondary to Valsalva retinopathy in one eye.

VKH being a diagnosis of exclusion was confirmed based on the typical features on imaging and the revised diagnostic criteria.[7] The disc staining in both eyes on FA, revealed it as a bilateral pathology since the other typical FA findings were obscured by the overlying hemorrhage in the OD. The subjective and clinical improvement with steroids confirmed the inflammatory etiology as VKH. With other etiologies ruled out, the presence of sub hyaloid hemorrhage and superficial hemorrhages confirmed the diagnosis of Valsalva retinopathy coexisting with VKH.

Valsalva retinopathy manifests as preretinal hemorrhage secondary to rupturing of superficial retinal vessels from physical exertion. Valsalva maneuver is characterized by a sudden rise in intrathoracic or intraabdominal pressure against a closed glottis, leading to a rapid rise of intravenous pressure within the eye, causing retinal vessels to spontaneously rupture.[8],[9]

What makes our case interesting is the coexistent subhyaloid hemorrhage unilaterally, confusing the typical bilateral pattern of VKH, urging us to search for possibilities and exclude threatening etiologies before concluding it as inflammation and starting on steroids.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Simmons-Rear A, Yeh S, Chan-Kai BT, Lauer AK, Flaxel CJ, Smith JR, et al. Characterization of serous retinal detachments in uveitis patients with optical coherence tomography. J Ophthalmic Inflamm Infect 2012;2:191-7.  Back to cited text no. 1
Alarfaj MA, Hasen AA, Al-Yami SS. Traumatic subhyaloid macular hemorrhage with complete resolution following Neodymium-Doped Yttrium Aluminium Garnet Laser. Am J Ophthalmol Case Rep 2018;9:85-7.  Back to cited text no. 2
Uvaraj T, Mathew M. Serous macular detachments are not always benign. Kerala J Ophthalmol 2018;30:200.  Back to cited text no. 3
  [Full text]  
Yaghoubi G, Heydari B, Yaghoobi MA. Headache in a 27-year-old man: Bilateral serous retinal detachment. J Neurosci Rural Pract 2011;2:68-70.  Back to cited text no. 4
[PUBMED]  [Full text]  
Kim J, Chang W, Sagong M. Bilateral serous retinal detachment as a presenting sign of acute lymphoblastic leukemia. Korean J Ophthalmol 2010;24:245-8.  Back to cited text no. 5
Di Mattina AJ, Fazelat A, Santaro A. Exudative retinal detachment reveals metastatic bladder cancer. Optom Vis Sci 2016;93:549-54.  Back to cited text no. 6
Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: Report of an international committee on nomenclature. Am J Ophthalmol 2001;131:647-52.  Back to cited text no. 7
Kim KY, Yu SY, Kim M, Kwak HW. Macular hole formation after pars plana vitrectomy for the treatment of Valsalva retinopathy: A case report. Korean J Ophthalmol 2014;28:91-5.  Back to cited text no. 8
García Fernández M, Navarro JC, Castaño CG. Long-term evolution of Valsalva retinopathy: A case series. J Med Case Rep 2012;6:346.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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