This case presentation describes a 13-year-old female patient with diminished vision in both eyes for 6 months. She has a history of cataract surgery 2 months prior. On examination, she has uveitis, complicated cataracts, and vitreous opacity in both eyes. Based on her clinical features and investigations, she is diagnosed with Vogt-Koyanagi-Harada syndrome. She is started on high-dose intravenous steroids followed by oral steroids and immunosuppressants. Her vision improves but remains diminished at the time of discharge. Vogt-Koyanagi-Harada syndrome is a rare autoimmune condition affecting multiple organs including the eyes, skin, and central nervous
1. Case Preserntation
Dr Md Afzal Mahfuzullah
MBBS, MCPS, FCPS
Long term Fellow
Vitreo-Retina Department
Ispahani Islamia Eye Institute & Hospital
2. • Ms Shirin Akhter
• Age:13
• Student
Chief Complaints :
• Diminished of vision of both eyes for 6 month
• Headache for 8 months
• Tinnitus & vertigo for same duration
Past History
History of cataract surgery 2 month back
3. On first visit on 17th Sept 2014
R/E L/E
V/A 6/36 6/12
IOP (GAT) 11 mm of Hg 13 mm of Hg
Out come : Treated as Anterior uveitis & improved
4. On 2nd visit on 14 Oct, 2014
R/E L/E
V/A 6/60 6/24
IOP (GAT) 13mm of Hg 12mm of Hg
B Scan With in normal
limit
With in normal limit
Out come :Treated as Anterior uveitis & improved
5. On 3rd visit on 21 Dec 2014
R/E L/E
V/A 6/60 6/24
IOP (GAT) 13mm of Hg 12mm of Hg
B Scan mild vitreous
opacity
mild vitreous
opacity
Lens Complicated
cataract
Complicated
cataract
Vitreous Cells+ Cells+
Outcome :Treated as Anterior & Intermediate uveitis & improved
Advised for cataract extraction after 3 month
6. Surgery was performed on 25th February
• I/A with acrylic hydrophobic foldable PCIOL was
inserted .
• On Discharge:
R/E L/E
V/A 6/36 6/12
Pin Hole 6/18 6/9
7. On 4th visit on 7th April, 2015
Retina department
Right Eye Left Eye
Distance Vision 3/60
No improvement
with pinhole
1/60
No improvement
with pinhole
Near Vision N60 N60
Eyelids Normal Normal
Conjunctiva Congested Congested
Cornea Clear Clear
Anterior Chamber Cell +
Flare+
Cell +
Flare +
Iris Normal Normal
Pupil reaction 360 deg post synachae 360 deg post synachae
Lens Pseudophakia Complicated cataract
8. Cont. ocular exam
RIGHT EYE LEFT EYE
IOP 14 mmHg 12 mmHg
Fundus Media –
Vitreous opacity
No fundal view
Media –
Vitreous opacity
No fundal view
10. Neurological Examination:
• Other cranial nerves – Normal
• Motor System - Normal
• Sensory System – Normal
• Cerebellar system – Normal
Respiratory System:
• Normal breath sounds heard
• No added sounds
Cardiovascular System:
• S1 ,S2 heard
• No murmur
11. •Hb : 12 gm %
•ESR : 34 mm after 1 hr
•Platelet : 4.85 lakh/cumm
•RBC : 4.27 million/cumm
•RBS :98 mg/dl
• PCV : 29 %
•MCV : 68 fl
•MCH : 20 pg
• MCHC : 30
•TC: 12,300 cells/cumm
N : 87 % E : 4 % , L : 9 %
ANA : negative
RA : (8.5 Iu/ml )negative
CRP : within normal
limits
Mantoux Test – Negative
Chest X Ray – Normal
HLA B27 : Negative
VDRL : Negative
Investigations
12. B scan of both eye
• B scan
• L/E
• B scan
• R/E
15. Management from Retina department
Admission on 07-04-2015
• Started on Inj IV Methyl Prednisolone 500
mg in 250 ml D/A –for 3 days
• Oral Tab Prednisololne 30 mg
• Oral Tab Azathioprine 50 mg
• Review after 1 week.
Ref: Fang and Wang: Curr Eye Res 2008;33:517 (review). Read et al: Curr Opin
Ophthalmol 2000;11:437 (review).
16. Condition at time of discharge
10.04.2015
• Right eye - V/A 6/60
• Left eye- V/A 6/60
18. Discussion
• Vogt: 1906 one case
• Koyanagi: 1923 six cases
• Harada: 1926 posterior uveitis and pleocytosis of
CSF
Vogt-Koyanagi-Harada or VKH
19. Discussion Cont
Epidemiology:
• More prevalent in darker skinned ethnic groups
common
in Japan
in people from Mediterranean origin
in American Indians/Africans
in Indians
• 2nd to 4th decade
20. • Multisystem disease
• Chronic, bilateral, granulomatous panuveitis
• Associated with central nervous system, auditory and
integumentary manifestations
• Systemic disorder
Eyes/ears
Meninges
Skin
Ref :Moorthy et al: Surv Ophthalmol 1995; 39:265 (review)
Read et al: Am J Ophthalmol 2001;131:647
22. Prodromal Acute Uveitic Stage Convalsecent stage Chronic Recurrent
stage
Mimics viral
Infection
Bilateral blurring of
vision
Ocular pain
secondary to Ciliary
spasm
Vitiligo
Alopecia
Poliosis
43% in 1st three
months
52% in 1st six
months
Fever Multifocal Choroidtis Uveal
depigmentation
Sunset glow
Glaucoma
Cataract
Neurological
Symptoms
Multifocal
detachment
of the sensory retina
Exudative retinal
detachment
Foci of
hyperpigmentation
of RPE
Subretinal Fibrosis
23. Ocular Findings
• Bilateral disease
• Granulomatous panuveitis
• Anterior Segment involvement
Iris nodules and mutton fat KP’s in chronic or
recurrent disease
• Shallowing of the AC + IOP ↑
Inflammatory swelling of ciliary body
Pupillary block
Formation of AS chronic glaucoma
24. International Nomenclature Committee Revised Diagnostic
Criteria
• Complete VKH disease
• Incomplete VKH disease
• Probable VKH disease
Ref :New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20
25. Treatment
• Systemic corticosteroids:
– Intravenous pulse therapy
– Oral treatment (1mg/kg/day)
• Duration -inflammatory activity
• Slow taper over 1 year period
• Topical treatment for anterior uveitis
Ref: Fang and Wang: Curr Eye Res 2008;33:517 (review). Read et al: Curr Opin Ophthalmol 2000;11:437
(review).
Yamaki et al: Int Ophthalmol Clin 2002;42:13 (review).
26. Treatment Cont
• Slow taper over 1 year period or ~ inflammatory
activity
• Consider adding Immune suppressive to reduce side
effects of high dose steroids
27. Prognosis
• Visual prognosis is generally favorable.
• 87.5% achieved V/A. of ≥612
• High-dose systemic corticosteroids for >9 months with
slow tapering significantly improves the prognosis and
decreases risk of recurrence.
• Age older than 18 years is significantly associated with
the development of complications.
• visual prognosis is generally favorable in children.
• Ref: Al-Kharashi, Abu El-Asrar: Int Ophthalmol 2007;27:201
• Abu El-Asrar et al: Eye 2008;22:1124
28. Related Article
• The Vogt-Koyanagi-Harada syndrome: a rare differential diagnosis of
uveitis in childhood. A case report taking into account the revised
diagnostic criteria].
• Article in German] Klin Monbl Augenheilkd. 2005 Nov;222(11):919-22
• Rieger R1, Keitzer R, Pleyer U.
• Summery: The existing diagnostic criteria of VKH syndrome may prove to
be inadequate in diagnosing VKH syndrome, particularly at the onset of
the disease. Considering, that early diagnosis and treatment is crucial in
improving the outcome of the disease, current diagnostic criteria have
been revised. Recent retrospective data suggest a higher sensitivity for the
revised diagnostic criteria.
29. Related Article
• Vogt-Koyanagi-Harada Syndrome in a 10 Years Old Child
• Aalia R Sufi, Sumera Zargar, Tejit Singh,
• Department of Ophthalmology, Government Medical College, Srinagar, Jammu & Kashmir,
India
• Online J Health Allied Scs.2011;10(2):24
• Summery: Diagnosis of the Vogt-Koyanagi-Harada (VKH) syndrome,
specially in children is difficult due to the rarity of its occurrence in this
age group, the variable onset of clinical signs and symptoms in the course
of the disease and absence of diagnostic serological parameters
30. • Choroidal Thickness In Eyes With Recurrent
Vogt-Koyanagi-Harada Disease
Yutaka Imamura1, Kouhei Hashizume2, Takamitsu Fujiwara2, Shigeki Machida2, Masahiro
Ishida1 and Daijiro Kurosaka21Ophthalmology, Teikyo University School of Medicine, Kawasaki, Japan
2Ophthalmology, Iwate Medical University School of Medicine, Morioka, Japan
• Purpose: To investigate the change of choroidal thickness in eyes showing
recurrent inflammation during the follow-up of Vogt-Koyanagi-Harada disease
(VKH) after high-dose steroid therapy.
• Conclusions: Choroid becomes thick in eyes with relapsing VKH. Thickening of
choroid may be a hallmark of recurrence of inflammation during the follow-
up of VKH after high-dose steroid therapy.