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Childhood VKH
- 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
- 9. General Examination : • Patient is cooperative, conscious, oriented Vital Parameters : • Blood pressure : 130/80 mm/Hg • Pulse : 84/min
- 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
- 13. Differential Diagnosis • VKH • Uveitis due to JIA • Posterior Scleritis
- 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
- 17. Discussion
- 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
- 21. Clinical course Four phases: • Prodromal • Acute uveitic • Convalescent or chronic • Chronic recurrent
- 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.