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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 l.jpg

Case 2

Dr Chin Chee Fang

Medical Officer

Tan Tock Seng Hospital


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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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On examination

Right Left


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  • What were the findings?

  • Bilateral disc swelling L > R

    • (R) disc hyperaemic

  • What would be the next step?

  • Take a full history


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Further history

  • Over past 2 days, had bitemporal headache

  • a/w some neck stiffness

  • 2 weeks’ hx of tinnitus bilaterally


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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


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What is the next step?

  • CT brain

  • Lumbar puncture


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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


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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


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Fundal examination

striae

striae

hard exudates

subretinal fluid

Right Left

  • What do the photos show?

    Bilateral neurosensory detachments


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FFA

Pinpoint hyperfluorescence

  • What does this frame show? (at 3 minutes)

  • Multiple pinpoint hyperfluorescences at level of RPE with pooling

  • Disc leakage

pooling



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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


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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


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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


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  • 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


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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


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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


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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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