Case 2

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 ? 6/121
• GAT
• (R) 17 mmHg
• (L) 19 mmHg

3
On examination

• Right Left

4

• What were the findings?
• Bilateral disc swelling L gt 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 38C
• 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 / Behcets / SLE/
  Wegeners / VKH

10
Fundal examination
striae
striae
hard exudates
subretinal fluid

• Right Left
• What do the photos show?
• Bilateral neurosensory detachments

11
FFA
Pinpoint hyperfluorescence
pooling

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

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 early control 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.