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Case 2

Case 2. Dr Chin Chee Fang Medical Officer Tan Tock Seng Hospital. Madam T. 43 Chinese female No PMHx of note Presented with 2 weeks’ hx of left blurring of vision Associated with fatigue & some irritation VA with glasses (R) 6/6 (L) 6/18 → 6/12 +1 GAT (R) 17 mmHg (L) 19 mmHg.

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Case 2

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  1. Case 2 Dr Chin Chee Fang Medical Officer Tan Tock Seng Hospital

  2. Madam T • 43 Chinese female • No PMHx of note • Presented with 2 weeks’ hx of left blurring of vision • Associated with fatigue & some irritation • VA with glasses • (R) 6/6 • (L) 6/18 → 6/12+1 • GAT • (R) 17 mmHg • (L) 19 mmHg

  3. On examination Right Left

  4. What were the findings? • Bilateral disc swelling L > R • (R) disc hyperaemic • What would be the next step? • Take a full history

  5. Further history • Over past 2 days, had bitemporal headache • a/w some neck stiffness • 2 weeks’ hx of tinnitus bilaterally

  6. Full examination • Colour (Ishihara) 15/15 BE • Left mild RAPD • Visual fields : Bilateral central scotoma • Temperature 38°C • BP / heart rate normal • WCC 9.8 x 109/L, neutrophils 70.7% • ESR 4mm/hr, CRP 0.5

  7. What is the next step? • CT brain • Lumbar puncture

  8. Investigations • CT brain : Normal • Lumbar puncture CSF results : • Opening pressure unknown (manometer not available on ward) • Cell count : 180/µL • Neutrophils : 9%, Lymphocytes 91% • Glucose : 2.9 mmol/L (serum glc 4.9) • Protein 0.7g/L (0.1-0.4) • CSF gram stain & c/s –ve • Mantoux –ve

  9. Differential diagnoses? • LP showed CSF pleocytosis with lymphocyte dominance • Infectious Causes • Viral – entero / herpes / HIV • TB • Neurosyphilis • Rare : Brucellosis, Cryptococcus • Non-infectious Causes • Aseptic meningitis • Drugs – NSAIDs / (intrathecal methotrexate) • Systemic diseases – Sarcoid / Behcet’s / SLE/ Wegener’s / VKH

  10. Fundal examination striae striae hard exudates subretinal fluid Right Left • What do the photos show? Bilateral neurosensory detachments

  11. FFA Pinpoint hyperfluorescence • What does this frame show? (at 3 minutes) • Multiple pinpoint hyperfluorescences at level of RPE with pooling • Disc leakage pooling

  12. OCT of left macula

  13. What is the likely diagnosis? Acute Vogt-Koyanagi Harada (VKH) • Differentials : • Posterior scleritis • Intraocular lymphoma • Idiopathic uveal effusion syndrome • Sympathetic ophthalmia • Neurosyphilis • Sarcoidosis • Bilateral diffuse uveal melanocytic proliferation • Metastatic carcinoma • Toxaemia of pregnancy / eclampsia

  14. VKH • Idiopathic, bilateral, granulomatous, panuveitis. • a/w systemic manifestations • Usually males of Oriental origin • Thought to be related to aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. • Linked to HLA-DR4, HLA-Dw53 • Vogt-Koyanagi • Cutaneous lesions & anterior uveitis • Harada • Neurological features & exudative RD

  15. What are the phases of VKH? • Prodromal • Fever, headache, meningism, cranial nerve palsies • Tinnitus / vertigo • CSF pleocytosis (resolves within 8 wks) • Uveitic (Acute) • Convalescent • Dalen-Fuchs • ‘Sunset glow’ fundus • Perilimbal vitiligo (Sugiura sign), vitiligo of eyebrows & lashes • Chronic-recurrent • Chronic granulomatous AAU • Posterior synechiae / cataract / glaucoma / CNV

  16. Uveitic / Acute VKH • p/w acute BOV • Granulomatous AAU – Vogt-Koyanagi • Posterior uveitis - Harada • Disc oedema • Multifocal choroiditis with sensory detachments • Detachments coalesce into exudative RD • Sometimes high IOP due to rotation of iris-lens diaphragm

  17. Management • Early aggressive treatment with systemic steroids • IV methylprednisolone (1g/day) x 3days • Oral prednisolone 1 mg/kg/day • Most require therapy for at least 6 months to a year • Rapid improvement of serous RD noted following pulse corticosteroid therapy • Supports an early therapeutic mechanism related to improved permeability of capillaries & of the blood-retinal-barrier than an anti-inflammatory / immunosuppresive action. (Yamanaka et al, AJO 2002 Sept) • Immunosuppressive therapy • eg cyclosporine, azathioprine, methotrexate, cyclophosphamide

  18. Management • Biologics e.g. IFN- – alternative in corticoresistant/ corticodependent forms of disease (Touitou et al, J Fr Ophthalmol, 2005 Jan) • If anterior uveitis : topical steroids & cycloplegics • Prognosis • Visual loss from cataracts / glaucoma / CNV • Can be prevented with earlycontrol of inflammation & not discontinuing Rx before 3 months

  19. Madam T • Commenced on oral prednisolone (1mg/kg) 60 mg om. • Now on decreasing dose. • Currently (2 weeks post-presentation), right macula flat, left exudative RD improved. • Symptomatically better.

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