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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

Case 2

Dr Chin Chee Fang

Medical Officer

Tan Tock Seng Hospital

madam t
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
on examination
On examination

Right Left

slide4
What were the findings?
  • Bilateral disc swelling L > R
    • (R) disc hyperaemic
  • What would be the next step?
  • Take a full history
further history
Further history
  • Over past 2 days, had bitemporal headache
  • a/w some neck stiffness
  • 2 weeks’ hx of tinnitus bilaterally
full examination
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
what is the next step
What is the next step?
  • CT brain
  • Lumbar puncture
investigations
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
differential diagnoses
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
fundal examination
Fundal examination

striae

striae

hard exudates

subretinal fluid

Right Left

  • What do the photos show?

Bilateral neurosensory detachments

slide11
FFA

Pinpoint hyperfluorescence

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

pooling

what is the likely diagnosis
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
slide14
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
what are the phases of vkh
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
slide16
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
management
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
management18
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
madam t19
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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