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The Official Scientific Journal of Delhi Ophthalmological Society
Bilateral Shallow Anterior Chamber And Transient Myopia As A Presenting Feature Of Vogt Koyanagi Harada Syndrome
Rahul Kumar Sharma, Abhishek Dagar, Vivek Kumar
Vitreo-Retina Department, Venu Eye institute & Research Center, New Delhi, India
Corresponding Author:
Rahul Kumar Sharma
MS, FICO
Fellow Vitreo-Retina Department, 
Venu Eye institute & Research Center
 New Delhi, India.
Email id: rahulsharma1253@gmail.com
Published Online: 30-APR-2018
DOI: http://dx.doi.org/10.7869/djo.357
Abstract
The Vogt-Koyanagi-Harada (VKH) syndrome is a rare systemic disorder of uveitis, dysacousia, vitiligo, premature greying of the hair, eyebrow and eyelashes, and meningoencephalitis. We report a case of a 36 year old type 2 diabetic patient who presented with sudden, painless, progressive blurring of vision in both eyes with shallow anterior chamber and transient myopia. He had history of headache, mild fever, malaise with stiffness in neck and back one week before blurring of vision. Exudative retinal detachment on fundus examination along with optical coherence tomography (OCT) features and fundus fluorescein angiogram (FFA) pattern confirmed our diagnosis as incomplete VKH. The patient responded well with steroid (systemic and topical) and cycloplegic drug. This case highlights the importance of history, careful ocular evaluation, judicious use of OCT and FFA in a patient presenting with bilateral shallow anterior chamber and transient myopia to narrow down the differential diagnosis as incomplete VKH.
Keywords : anterior chamber, transient myopia, Harada syndrome
Introduction

A 36 year old type 2 diabetic male patient presented with blurring of vision in both eyes since 4 days, which was sudden in onset, painless and progressive in nature in April 2017. There was no history of trauma, redness, pain in eye or similar episode in the past. He had history of headache, mild fever, malaise with stiff ness in neck and back one week before blurring of vision in both eyes. Family history revealed that his father was type 2 diabetic patient. 

The patient was orthoporic and had no history of wearing spectacles before this episode. At the time of presentation his best corrected visual acuity was 6/24 (Log MAR 0.6), N36 in both eye with myopic refraction. Anterior segment of both eyes had shallow anterior chamber (Figure 1 & 2) (Van Herrick grade 1) with 1+ inflammatory cell (Standardization of uveitis nomenclature grading {SUN}). Posterior segment had mild vitritis in both eyes. In both the eyes, applanation tonometry was 18 millimeters of mercury. Schwalbe’s line was visible on gonioscopy in both eyes and on indentation the angles opened up to scleral spur. 



Fundus examination in both eyes (Figure 3 & 4) showed multilobular areas of subretinal fluid pockets (exudative retinal detachment) at the posterior pole and mid periphery with creamy yellow choroidal lesions, clinically suggestive of exudative pathology. There were no signs of diabetic retinopathy. On laboratory investigations, complete blood count was within normal limits, erythrocyte sedimentation rate (ESR) was 20mm/hour, Mantoux skin test was negative (3x3 mm), human immune deficiency virus (HIV) and Treponemal pallidum haemagglutination assay (TPHA) were non reactive. Serum angiotensin converting enzyme (ACE) was 24 IU (normal). Chest X ray, HRCT Chest, USG of whole abdomen was normal. Optical coherence tomography at macula of both eyes showed multiple areas of neurosensory detachment with largest volume and subfield thickness at macula (Figure 5 & 6).Fundus fluorescein angiogram (FFA) of early and mid-phase showed multiple hyperfluorescent dots at the level of retinal pigment epithelium (Figure 7 & 8). These dots enlarged and stained the surrounding subretinal fluid. In the late phase of angiogram pooling of dye in the subretinal space was seen (Figure 9 & 10). Based on history, clinical findings, laboratory investigation and fundus fluorescein angiography, the patient was diagnosed as incomplete VKH and started on topical steroid and cycloplegic (atropine) with pulse therapy of intravenous methylprednisolone (IVMP) 1gm for 3 days followed by oral steroid. Immunosuppressive (azathioprine) was added with steroid tapering for long term control as well as to avoid side effects of steroids in a diabetic patient. The patient was shifted on insulin (subcutaneous) for better control of diabetes. After 3 doses of IVMP the anterior chamber became deep with resolution of anterior uveitis, vitritis, exudative retinal detachment (Figure 11 & 12). Optical Coherence Tomography (OCT) documented improvement as foveal contour started returning to normal with decreased subretinal fluid and flattening of neurosensory detachment (Figure 13 & 14).












On follow up examination, best corrected visual acuity improved to 6/9 in right eye and 6/6 in left eye and fundus examination showed totally resolved exudative retinal detachment, no vitritis and healed choroidal lesions with no recurrence on follow up (Figure 15 & 16). Foveal contour became normal on OCT, with minimal depressed parameters (Figure 17 & 18).







Differential diagnosis:
Uveal effusion syndrome
Multifocal choroiditis (secondary to sarcoidosis/tuberculosis)
White dot syndrome (MEWDS, APMPPE, PIC, MC with PU)
Bird shot Chorioretinopathy
Masquerades(lymphoma)

Discussion

VKH syndrome may be defined as a bilateral, chronic, granulomatous panuveitis associated with systemic manifestations that may affect the central nervous system (e.g., meningismus, headache); the inner ear (e.g., dysacousia, vertigo, tinnitus); and the integumentary system (e.g., vitiligo, alopecia, poliosis). The immune response in VKH syndrome most likely is directed against antigens associated with melanocytes present in all organ systems, including the choroid. VKH syndrome has been described as evolving in phases or stages, ranging from nonspecific neurological symptoms to devastating ocular inflammation. Long-term ocular complications include cataract, glaucoma, subretinal neovascularization, subretinal fibrosis, epiretinal membrane formation, macular and optic nerve atrophy, and pigmentary degeneration.1-5

The natural course of VKH syndrome is divided classically info four chronological stages or phases: prodromal, uveitic, convalescent or chronic, and recurrent.6

The prodromal phase precedes ocular symptoms and usually manifests with nonspecific neurologic and auditory symptoms. Neurologic symptoms generally include headache, nuchal rigidity, and low-grade fever; auditory symptoms consist primarily of vertigo, dysacousia, and tinnitus. CSF typically shows an increase in lymphocytes and other inflammatory cells. The clinical features and spinal tap findings during this phase often lead to a misdiagnosis of aseptic meningitis.5,7

The uveitic phase usually commences 3 to 5 days after the prodrome. In atypical patients, however, it may start several weeks after the initial phase. Ocular findings are the hallmark of the uveitic phase.5

VKH may cause shallow anterior chamber, angle closure and transient myopia.9 The inflammatory changes of VKH in the anterior segment, i.e; ciliary oedema and ciliochoroidal detachment, may exacerbate the shallow anterior chambers and narrow angles. Immunosuppressive agents and systemic corticosteroids during the uveitic phase are the mainstays of therapy. Topical corticosteroids and cycloplegics are helpful to control anterior segment inflammation.5 VKH associated with shallow anterior chamber may be misdiagnosed as acute primary angle closure.

Our patient presented with shallow anterior chamber and transient myopic refraction which on further clinical examination, OCT, FFA narrowed down our diagnosis as incomplete VKH(based on revised diagnostic criteria for VKH10). With prompt treatment with steroids (systemic and topical) and cycloplegic drugs, patient responded well. For differentiating from other inflammatory diseases, examination of the ocular fundus including OCT and FFA are very useful.

A patient presenting with bilateral shallow anterior chamber and myopic refraction, atypical or multilobular areas of subretinal fluid should raise a high level of suspicion for VKH, particularly when features are accompanied or preceded by neurological symptoms, general malaise or mild anterior chamber reaction or vitritis. Prompt diagnosis and treatment in the form of systemic immunosuppressant (steroid and immunosuppressive) is crucial to ensure a more rapid recovery and better visual prognosis. These patients should be counselled for long term follow up to prevent recurrences and complications.

References
  1. Rao NA. Mechanisms of inflammatory response in sympathetic ophthalmia and VKH syndrome. Eye (Lond) 1997; 11:213-6.
  2. Sakamoto T, Murata T, Inomata H. Class II Major histocompatibility complex on melanocytes of Vogt-Koyanagi-Harada disease. Arch Ophthalmol 1991; 109:1270-4.
  3. Bykhovskaya I, Thorne JE, Kempen JH, Dunn JP, Jabs DA. Vogt-Koyanagi-Harada Disease: clinical outcomes. Am J Ophthalmol 2005; 140:674-8.
  4. Camarena JH, Gonzalez LA, Shaikh M, García AR, Foster CS. Vogt- Koyanagi- Harada Syndrome. Contemporary Ophthalmology 2010;16:1-9.
  5. Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol 1995; 39:265–92.
  6. Damico FM, Kiss S, Young LH. Vogt-Koyanagi-Harada disease. Semin Ophthalmol 2005; 20:183-90.
  7. Sugiura S. Vogt-Koyanagi-Harada disease. Jpn J Ophthalmol 1978; 22:9-35. 
  8. Kamondi A, Szegedi A, Papp A, Seres A, Szirmai I. Vogt-Koyanagi- Harada disease presenting initially as aseptic meningoencephalitis. Eur J Neurol 2000; 7:19-22.
  9. Toimori S, Uyama M. Shallow anterior chamber and transient myopia as initial signs of Harada’s disease. Jpn J Clin Ophthalmol 1977; 31:1271-3.
  10. Read RW, Holland GN, Rao NA, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 2001; 131:647-52.
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Rahul Kumar Sharma, Abhishek Dagar, Vivek KumarBilateral Shallow Anterior Chamber And Transient Myopia As A Presenting Feature Of Vogt Koyanagi Harada Syndrome.DJO 2018;28:46-50
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Rahul Kumar Sharma, Abhishek Dagar, Vivek KumarBilateral Shallow Anterior Chamber And Transient Myopia As A Presenting Feature Of Vogt Koyanagi Harada Syndrome.DJO [serial online] 2018[cited 2018 Oct 24];28:46-50. Available from: http://www.djo.org.in/articles/28/4/Bilateral-Shallow-Anterior-Chamber-And-Transient-Myopia-As-A-Presenting-Feature-Of-Vogt-Koyanagi-Harada-Syndrome.html
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