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 Table of Contents  
Year : 2022  |  Volume : 34  |  Issue : 1  |  Page : 65-67

A rare case of Vogt–Koyanagi–Harada disease presenting simultaneously with pituitary macroadenoma

Department of Ophthalmology, Institute of Medical Sciences and SUM Hospital, SOA (Deemed to be) University, Bhubaneswar, Odisha, India

Date of Submission17-Jun-2021
Date of Decision24-Jun-2021
Date of Acceptance28-Jun-2021
Date of Web Publication21-Apr-2022

Correspondence Address:
Dr. Pradeep Kumar Panigrahi
Department of Ophthalmology, Institute of Medical Sciences and SUM Hospital, SOA (Deemed to be) University, 8-Kalinga Nagar, Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_144_21

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A 45-year-old female presented with sudden painful loss of vision in both eyes of 1-week duration. Clinical findings and imaging studies suggested Vogt–Koyanagi–Harada (VKH) disease. She was started on systemic steroids. Nonresolution of periocular pain and presence of color vision defects prompted us to do neuroimaging study which detected a pituitary macroadenoma. The patient underwent surgical removal of the mass lesion. There was a complete resolution of VKH features with systemic steroids.

Keywords: Macroadenoma, pituitary, steroids, Vogt–Koyanagi–Harada disease

How to cite this article:
Panigrahi PK. A rare case of Vogt–Koyanagi–Harada disease presenting simultaneously with pituitary macroadenoma. Kerala J Ophthalmol 2022;34:65-7

How to cite this URL:
Panigrahi PK. A rare case of Vogt–Koyanagi–Harada disease presenting simultaneously with pituitary macroadenoma. Kerala J Ophthalmol [serial online] 2022 [cited 2022 Aug 15];34:65-7. Available from: http://www.kjophthal.com/text.asp?2022/34/1/65/343670

  Introduction Top

Vogt–Koyanagi–Harada (VKH) disease is an idiopathic inflammatory disease characterized by bilateral granulomatous panuveitis.[1] Ocular findings in the acute stage of the disease include multifocal neurosensory detachment, choroidal detachment, and optic disc hyperemia or swelling.[2] Prolonged immunosuppression forms the cornerstone of therapy. I report a rare case of VKH disease presenting with pituitary macroadenoma simultaneously.

  Case Report Top

A 45-year-old female presented with sudden onset painful loss of vision in both eyes (BE) of 1-week duration. The patient gave a history of having headache and eye ache for the last 2 months. There was a history of fever, flu-like symptoms, and increasing intensity of periorbital pain 1 week prior to vision loss. She had been diagnosed elsewhere as a case of posterior uveitis in BE and had been advised systemic steroids. Best-corrected visual acuity (BCVA) in BE was 20/200, N36. Anterior segment was within normal limits in BE. Pupils were reacting sluggishly to light bilaterally. Intraocular pressure was 16 and 18 mmHg in the right eye (RE) and left eye, respectively. Fundus examination revealed 1 + cells in vitreous, significant disc edema, and exudative macular detachment bilaterally [Figure 1]. A pocket of subretinal fluid was noted superior to the optic disc in RE also. The patient had undergone optical coherence tomography (OCT) elsewhere which showed pockets of neurosensory detachment with intervening septae in BE. The fluid within the pockets appeared hyperreflective which was suggestive of high fibrin content. Fundus fluorescein angiography showed early pinpoint hyperfluorescence in the macular area in BE with increasing leakage from these points as the angiography proceeded. Bilateral disc leakage was noted [Figure 2]. Ultrasound examination showed lens echo, few low reflective dot echoes, increased choroidal thickness, and absent T-sign in BE.
Figure 1: (a and b) Color fundus photograph of the right and left eye showing disc edema and macular neurosensory detachment

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Figure 2: (a and b) Fundus fluorescein angiography showing disc edema and early pinpoint hyperfluorescent lesions in the macular area bilaterally. (c and d) Fundus fluorescein angiography images showing a progressive increase in disc and macular leakage bilaterally in the late phases of angiography

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We diagnosed it as a case of VKH disease in BE. Routine blood investigations, blood sugar, and baseline investigations for sarcoidosis, tuberculosis, and syphilis were normal. The patient was started on intravenous methylprednisolone (1 g mixed in 300 ml normal saline) for 3 days. After completion of pulse steroid therapy, the patient was started on oral steroids (Tablet Omnacortil 1 mg/kg after heavy breakfast), proton-pump inhibitors, and calcium supplements. The patient came for review 2 weeks later. She reported mild improvement in vision, but there was no relief from the periocular pain. BCVA in BE was 20/120, N24. Pupils were sluggishly reacting to light in BE. Color vision done using Ishihara's chart was defective in BE. Fundus examination showed decreasing subretinal fluid in the macular area but a minimal reduction in disc edema. At this point of time, we advised magnetic resonance imaging (MRI) of the brain to rule out any other possible etiology responsible for disc edema. MRI scans revealed a well-defined lobulated heterogeneously enhancing hyperintense lesion (12 mm × 13 mm) in the sellar and suprasellar region with chiasmal compression suggestive of pituitary macroadenoma [Figure 3]. The patient was immediately referred to the neurosurgery department where she was advised surgical removal of the adenoma. The patient underwent successful removal of the tumor through the transsphenoidal approach 3 weeks following initial presentation.
Figure 3: Axial (a) and coronal (b) magnetic resonance imaging scans of the brain showing a well-defined hyperintense lobulated lesion in the sellar and suprasellar region suggestive of pituitary macroadenoma

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Six weeks following the presentation, BCVA in BE was 20/80, N12. Disc edema and exudative macular detachment had resolved. Few hard exudates were noted in the macular area bilaterally [Figure 4]a and [Figure 4]b. OCT showed complete resolution of neurosensory detachment [Figure 4]c and [Figure 4]d. Oral steroids were slowly tapered over a period of 6 months. At final follow-up, 6 months after presentation BCVA remained stable at 20/60, N12 in BE. Bilateral disc pallor suggestive of optic atrophy was noted in BE. Macula was dry bilaterally.
Figure 4: (a and b) Color fundus photograph of the right and left eye showing resolved disc and macular edema with few hard exudates in the macular area bilaterally. (c and d) Optical coherence tomography images of the right and left eye showing resolved neurosensory detachment bilaterally

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  Discussion Top

Pituitary adenomas are usually benign tumors. Clinically nonfunctioning pituitary adenomas are the most common subtype which are characterized by the absence of clinical evidence of hormonal secretion.[3] There were no clinical signs of hormonal secretion in the present case. Visual symptoms produced depend on the size of the lesion. Pituitary macroadenomas (>1 cm) can lead to ocular symptoms such as field defects, eye pain, headache, blurring of vision, and double vision.[4] Compression of optic nerves, if not relieved early, can lead to optic atrophy and permanent vision loss.[5]

The present case presented with clinical and angiographic features of VKH disease. Patients with VKH disease also present with headache and periocular pain which usually get resolved soon with the initiation of systemic steroid therapy. Nonresolution of periocular pain 2 weeks after steroid therapy and the presence of color vision defects prompted us to go for neuroimaging study. Although there was resolution of disc edema and neurosensory detachment, the final visual acuity was poor. This could be due to optic atrophy induced by tumor compression. Simultaneous presentation of VKH disease and pituitary adenoma has not been reported earlier. However, the association seems more coincidental rather than causal in this case.

  Conclusion Top

The present case highlights the importance of performing additional investigation in all cases where the response to treatment is suboptimal. Early diagnosis and treatment of newly detected diseases can help salvage vision in such cases.

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

Fang W, Yang P. Vogt-koyanagi-harada syndrome. Curr Eye Res 2008;33:517-23.  Back to cited text no. 1
Katsuyama A, Kusuhara S, Awano H, Nagase H, Matsumiya W, Nakamura M. A case of probable Vogt-Koyanagi-Harada disease in a 3-year-old girl. BMC Ophthalmol 2019;19:179.  Back to cited text no. 2
Ntali G, Wass JA. Epidemiology, clinical presentation and diagnosis of non-functioning pituitary adenomas. Pituitary 2018;21:111-8.  Back to cited text no. 3
Kitthaweesin K, Ployprasith C. Ocular manifestations of suprasellar tumors. J Med Assoc Thai 2008;91:711-5.  Back to cited text no. 4
Abouaf L, Vighetto A, Lebas M. Neuro-ophthalmologic exploration in non-functioning pituitary adenoma. Ann Endocrinol (Paris) 2015;76:210-9.  Back to cited text no. 5


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


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