Grand Ward Round Bilateral retinal swelling

1
Grand Ward RoundBilateral retinal swelling

• Dr Heng Li Wei
• Dept of Ophthalmology, TTSH
• 27th December 2007

2
History

• 41yo Chinese male, sales assistant
• No PMH. No prev ocular Sx.
• Not on any long term meds but occasional takes
  TCM.
• Presented on 25/10/07 c/o
• - Bilateral progressive BOV x 5-6 mths.

3
Examination

• VR 6/60, BCVA 6/24
• VL 6/24, BCVA 6/24
• No RAPD
• BE anterior segment NAD.
• IOP normal.
• Fundus photos

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Fundus Photos
5
Examination

• Posterior segment
• - occ vitreous cells seen
• - bilateral cystoid macula oedema
• - no vasculitis
• - optic discs pink, no swelling, CDR 0.4.
• - no other areas of neurosensory detachment.

6

• Differential diagnosis?
• Referred to Uveitis team 4 days later.
• OCT done.
• H/S FFA/ICG done
• - diffused generalized sick RPE with leakages
• - subtle central retinal vasculitis changes
• - advanced CMO.

7

• Impression
• - 1. ? VKH ( Harada type)
• - 2. r/o infective causes TB, syphilis, HIV.
• Investigations?

8
Investigations
9

• Further history unmarried, denies abnormal
  sexual activities, ? previous MSP.
• TPPA Reactive.
• Imp BE syphilitic posterior uveitis with CMO.
• Referred to ID to r/o neurosyphilis and kiv for
  IV penicillin.

10

• Patient was admitted to CDC under ID on 1/11/07
  and started on
• - IV penicillin 4MU 4hrly ( total of 2 wks)
• - G. pred forte tds BE.
• Lumbar puncture done 2/11/07
• - opening pressure 14cm H2O.
• - CSF sent for cell count, glucose protein
  level, VDRL, LIA syphilis IgG.

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IV Penicillin Day 7

• VR 6/18 VL 6/24
• CMO decreased. No active vitritis.
• Was started on T. prednisolone 40mg od and
  Famotidine, cont on G Pred Forte tds.

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IV Penicillin Day 12

• CSF - glucose 3.1mmol/L, protein 0.50g/L
• - VDRL non-reactive
• - LIA syphilis IgG positive
• VR 6/12 VL 6/15

13

• Imp 1. CMO slow resolution with T. pred
• 2. Neurosyphilis on IV Penicllin x 2 weeks.
• Started on IV Methylprednisolone 500mg bd x 3
  days.
• IV Methylpred and IV Penicillin course completed
  and pt was discharged with tapering dose of oral
  pred G. pred forte tds.

14

• Last r/v on 6/12/07
• CMO much decreased.
• OCT - R 234 microns L 226 microns.
• Next TCU on 31/12/07.

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Cystoid Macula Oedema

• Signs
• - loss of foveal depression
• - retinal thickening
• - multiple cystoid areas in sensory retina.
• FFA
• - AV phase parafoveal hyperfluorescence due to
  early leakage
• - late phase petaloid pattern of
  hyperfluorescence.

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Causes of CMO

• Vascular eg. DR, CRVO/BRVO, radiation
  retinopathy
• Inflammatory eg. Posterior uveitis, scleritis,
  Behcets dz, toxoplasmosis, CMV retinitis,
  birdshot retinochoroidopathy etc.
• Post-cataract Sx risk factors -gt PCR, vitreous
  loss, diabetics, ACIOL.

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Causes of CMO

• ERM
• Vitreo-macula traction
• Retinal dystrophies eg. RP, gyrate atrophy,
  dominantly inherited CMO.
• Drug-induced eg. topical latanoprost1, systemic
  nicotinic acid2
• 1 Moroi SE et al. Cystoid macular edema
  associated with latanoprost therapy in a case
  series of patients with glaucoma and ocular
  hypertension. Ophthalmology. 1999
  May106(5)1024-9
• 2 Dajani HM et al. Optical coherence tomography
  findings in niacin maculopahy. Can J
  Ophthalmology 2006 Apr41(2)197-200.

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Rare causes of bilateral CMO

• POEMS Syndrome3
• ( Polyneuropathy, organomegaly, endocrinopathy,
  monoclonal gammopathy, skin changes)
• - with bilateral optic disc oedema and bilateral
  CMO, anasarca and elevated serum VEGF.
• Post electric current strike4
• 3 Chong DY et al. Optic disc edema, cystoid
  macular edema, and elevated vascular endothelial
  growth factor in a patient with POEMS syndrome. J
  Neuroophthalmol. 2007 Sep 27(3) 180-3.
• 4 Manrique-Cerrillo M et al. Bilateral macular
  cysts secondary to electric current strike. A
  case report. Arch Soc Esp Oftalmol 2004
  Jan79(1)37-9.

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Syphilis

• STD caused by the spirochaete Treponema pallidum.
• Congenital or Acquired

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Acquired Syphilis - stages

• Primary
• - painless genital ulcer (chancre) and regional
  lymphadenopathy
• Secondary
• - 6-8wks after chancre
• - generalised lymphadenopathy, constitutional
  symptoms.
• - symmetrical maculopapular rash on trunk, palms
  and soles.
• - condylomata lata in anal region
• - mucous patches in mouth, pharynx, genitalia
• - meningitis, nephritis, hepatitis may occur.
• Latent
• - resolution of 2o syphilis, last for years.
• Tertiary
• - CVS aortitis with aneurysm, aortic
  regurgitation.
• - Neurosyphilis, tabes dorsalis
• - Gummata in various organs.

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Ocular Syphilis

• Usu occurs in secondary tertiary stages.
• Primary
• - conj chancre
• Secondary/Tertiary
• - Orbit lids -gt rash, orbital periostitis,
  dacryocystitis, madarosis
• - Ant Seg -gt conjunctivitis, IK, episcleritis,
  scleritis, uveitis
• - Post Seg -gt chorioretinitis, neuroretinitis,
  retinal vasculitis
• - Neuro-Oph -gt optic neuritis, CN palsies, optic
  neuropathy, pupils abnormalities, VF defects.

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CMO as primary sign of neurosyphilis

• 1st report of bilateral CMO as primary sign of
  neurosyphilis, based on FFA.
• Neurosyphilis dx based on CSF serology, cell
  count.
• Good VA recovery with systemic steriods combined
  with antitreponemal therapy.
• Martin NF et al. Cystoid macular edema as primary
  sign of neurosyphilis.Am J Ophthalmol. 1979
  Jul88(1)28-31.

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Ocular Syphilis

• Multiple ocular manifestations.
• Ocular syphilis may be initial presentation of
  syphilis, w/o obvious systemic manifestations. -gt
  early diagnosis Rx of syphilis is important as
  good response.1
• Co-infection with HIV common. 2,4
• Test for neurosyphilis. 3,6
• Rx same as for neurosyphilis -gthigh dose
  parenteral penicilline G 12-24 million units/day
  for 10-14 days. 4,5,6

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References

• Hong MC et al. Ocular uveitis as the initial
  presentation of syphilis. J Chin Med Assoc. 2007
  Jul 70(7) 274-80.
• Trans TH et al. Syphilitic uveitis in patients
  infected with human immunodeficiency virus.
  Graefes Arch Clin Exp Ophthalmol. 2005 Sep
  243(9) 863-9.
• Ormerod LD et al. Syphilitic posterior uveitis
  correlative findings and significance. Clin
  Infect Dis. 2001 Jun 1532(12)1661-73.
• Browning DJ. Posterior segment manifestations of
  active ocular syphilis, their response to a
  neurosyphilis regimen of penicillin therapy, and
  the influence of human immunodeficiency virus
  status on response. Ophthalmology 2000
  Nov107(11) 2015-23.
• Deschenes J. Acquired ocular syphilis diagnosis
  and treatment. Ann Ophthalmol 1992 Apr24(4)
  134-8.
• Kiss S et al. Ocular manifestations and treatment
  of syphilis. Semin Ophthalmol 2005 Jul-Sep20(3)
  161-7.

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Syphilis Lab tests

• Serologic nontreponemal tests
• Venereal Disease Research Laboratory (VDRL) and
  rapid plasma reagin (RPR).
• Detect antibodies directed against cardiolipin
  (lecithin) cholesterol antigens.
• Biologic false ve in IVDA, acute infection, SLE,
  pregnancy.
• High titer (gt116) indicates active disease.
• VDRL titer reflects the systemic activity of the
  disease.
• Reactive and weakly reactive tests should be
  confirmed with fluorescent treponemal antibody
  absorption (FTA-ABS).

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Syphilis Lab tests contd

• Serologic treponemal tests
• Micro-hemagglutination T pallidum (MHA-TP),
  FTA-ABS, T pallidum particle agglutination (TPA).
  
• Highly sensitive and specific tests.
• Titers are not correlated with clinical activity.
  
• FTA-ABS test
• - not useful in monitoring response to therapy
  as it remains positive for life
• - 98 sensitive, even in latent syphilis.

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Lumbar puncture for neurosyphilis

• For asymptomatic neurosyphilis.
• Pts with positive syphilitic serologic tests
  should have CSF tests for VDRL titers, cell
  count, total protein.
• Indicative of neurosyphilis
• - elevated WBC count with lymphocytes
  predominance, or
• - elevated total protein, even w/o positive CSF
  VDRL test.