Laser surgery in glaucoma

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Anterior uveitis Granulomatous type IOP
rise Transillumination defect Viral anterior
uveitis?
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Posterior scleritis Good general health No
rheumatoid disease Probably idiopathic ?
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• 52 year-old white male
• VA loss RE
• fascicular VFD
• swollen disc and disc hemorrhage
• AION ?

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Retinitis fociImmunocompetent mangranular
aspect hazy vitreousviral retinitis ?
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100 µV/div
Nightblindness Severe VF constriction OU Almost
flat ERG OU Hereditary retinal dystrophy ?
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The great imitator

• Philippe Kestelyn, MD, PhD, MPH
• Department of Ophthalmology
• Ghent University Hospital
• Belgium

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The great imitator Lecture for the British
Medical Society in 1879
Jonathan Hutchinson
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Pubmed search

• The great imitator strikes back
• The return of the great imitator
• The new great imitator (Lyme disease)

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Great imitators

• Internal medicine syphilis/ Lyme disease
• Dermatology sarcoidosis/syphilis
• Family medicine depression
• Urgency medicine CO intoxication
• Ophthalmology syphilis/ Lyme disease

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SyphilisEpidemiology

• 12 million new cases of syphilis worldwide each
  year
• uncommon in Europe
• serious problem in developing countries
• serious sequelae / risk of congenital infection

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Syphilis

• sexually transmitted disease caused by Treponema
  pallidum
• penetrates intact mucous membranes and abraded
  skin
• incubation from 10 to 90 days
• spread through the lymphatics to the bloodstream
• hematogenous dissemination
• 3 stages primary, secondary and tertiary
  syphilis
• 70 of patients remain in latent stage after
  secondary stage
• 30 go on to develop tertiary syphilis

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Congenital Syphilis

• transplacental transmission of T. pallidium first
  3 months
• preventable!
• intrauterine death or serious congenital
  abnormalities
• generalized rash, jaundice, rhinitis
• osteochondritis and X-ray abnormalities of bones
  in gt 90
• chorioretinitis often present
• DD rubella, CMV, toxoplasmosis
• diagnosis FTA-ABS (IgM)

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Late congenital syphilis

• silent infection at birth
• after 2 years
• interstitial keratitis in 20
• Hutchinsons triad
• interstitial keratitis
• notched thin upper incisors with abnormal spacing
• deafness

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Ocular involvement in the different stages of
syphilis

• Primary syphilis conjunctival chancre (rare)
• Secondary syphilis anterior and posterior
  involvement with pronounced inflammation
• iritis (roseolae)
• acute syphilitic posterior placoid
  chrioretinitis
• inner retinal punctate lesions
• retinal necrosis
• Tertiary stage chronic anterior and posterior
  uveitis (chronic and mild vitritis, vasculitis,
  pigment epitheliopathy)

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Anterior segment involvement in syphilis

• starts as unilateral iritis
• contralateral eye involved in 50 of patients
• from mild nongranulomatous to severe
  granulomatous
• often notion of skin rash (secondary stage)
• resistant to corticosteroid treatment

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Luetic anterior uveitis

• 65-year-old male
• bilateral anterior uveitis
• resistant to topical steroid treatment
• history of allergic skin reaction

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Luetic anterior uveitis
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Posterior segment complications of syphilis

• posterior scleritis
• vitritis
• vasculitis
• venous and arterial occlusive disease
• chorioretinitis ,
• retinal necrosis
• acute syphilitic posterior placoid
  chorioretinitis
• punctate inner retinopathy
• retinal detachment with choroidal effusion
• pseudoretinitis pigmentosa
• macular edema, neuroretinitis
• papillitis, optic perineuritis

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Posterior scleritis in a TP seropositive patient
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Luetic perioptic neuritis
Bilateral papilledema in a young woman with
normal visual acuity Normal intracranial pressure
(imaging, lumbar puncture) LP confirms syphilitic
etiology
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Luetic papillitis

• 52 year-old white male
• VA loss RE
• fascicular VFD
• nocturnal sweats
• skin rash 2 months ago
• VDRL , RPR
• LP ?protein, VDRL pleocytosis,

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Luetic vitreitis
A young African patient complains of unilateral
hazy vision Serologic tests for syphilis are
positive Lumbar puncture confirms asymptomatic
neurosyphilis penicillin G IV
After treatment
Before treatment
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Luetic retinitis (HIV-)
A 32-year- old white male patient complains of
hazy vision in the left eye no general health
problems, but syphilis serology strongly positive
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Before and after treatment
Full recovery of visual acuity after penicillin G
therapy
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Syphilis in patients with HIV infection

• recognition of concurrent infection mandatory
• accelerated course of syphilis
• greater likelyhood of posterior uveitis,
  bilateral disease and neurosyphilis
• treatment failures more common
• serologic tests less reliable
• neurosyphilis treatment for all patients ?

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HIV and ocular syphilis

• Bilateral disease
• Accelerated course and extensive tissue
  destruction (retinalk necrosis)
• False negative serology (indirect test)

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Bilateral Luetic Uveitis post Rx
PPLE
PPRE
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Bilateral Luetic Uveitis
Inf perif LE
PPRE
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Bilateral Luetic Uveitis post Rx
FFA Inf perif LE
FFA PPRE
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Full-Field Flash ERG
Scotopic
Maximal
Photopic
30Hz Flicker
RE
LE
20 µV/div
100 µV/div
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Bilateral luetic uveitisLaboratory results

• HIV positive 624 CD4 cells /microliter
• Toxoplasmosis IgG - IgM -
• Epstein-Barr IgG 260 IU/ml IgM -
• CMV IgG gt 2000 IgM 2.0 (PCR negative)
  
• HSV IgG 1700 IgM -
• VZV IgG 1600 IgM
• RPR negative

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Prozone phenomenon

• disequilibrium between antibody and antigen
  levels
• ? present in less than 1 of patients with
  secondary syphilis
• ? false negative test

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Another presentation of syphilitic posterior
uveitis
Middle aged man with mild visual impairment and
bilateral inflammation
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Leopard-spot like lesions on FA in the
cicatricial phase
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• Acute syphilitic placoid pigment epitheliopathy
• first described by Gass
• considered to be pathognomonic for syphilis
• leopard spots on FA in the cicatricial phase

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A 3rd rather typical presentation of posterior
syphilitic involvement...
Middle aged man with mild visual impairment and
bilateral inflammation
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Syphilitic punctate inner retinitis in
immunocompetent gay men.
Wickremasinghe et al. Ophthalmology
1161195-1200, 2009.
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Non-specific tests for syphilis

• cardiolipin from beef heart detects anti-lipid
  IgG and IgM formed in patients in response to
• lipoidal material released from cells damaged by
  the infection
• lipids in the surface of T. pallidum
• VDRL (venereal disease research lab)
• RPR (rapid plasma reagin test)
• decline after effective AB therapy (indicator)
• false positive results

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Specific tests

• detection of antibodies to specific treponemal
  antigens
• FTA-ABS (fluorescent treponemal antibody
  absorption)
• TPHA (T. pallidum hemagglutination assay)
• become positive earlier and stay positive
  throughout life
• cannot be used as indicators of therapeutic
  response

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Treatment of ocular syphilis

• same treatment regimen as for neurosyphilis
• 18 to 24 million units of penicilllin G IV/day
  for 2 weeks
• doxycycline 100 mg orally BID for 30 days
• tetracycline 500 mg QID orally for 30 days
• corticosteroids may be added once effective
  antibiotherapy has been started

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Endemic treponematoses

• Genus treponema 4 human pathogens
• T. pallidum, subspecies pallidum venereal
  sypilis
• T. pallidum, subspecies endemicum endemic
  syphilis or bejel
• T. pallidum, subspecies pertenue yaws
• Treponema carateum pinta

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Endemic treponematoses Common features

• Primary and secondary lesions
• After latency some patients develop laate-stage
  disease
• Cutaneous manifestations prominent
• Penicillin drug of choice
• No serologic tests at present can differentiate
  endemic trepanomatoses from each other or from
  venereal syphilis

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Endemic treponematoses Important differences

• Target population
• Young children versus neonates and adults
• Mode of transmission
• Hand-to-hand or fomites versus sexual or
  transplacental
• Tertiary and systemic involvement
• Rare versus common
• Optic atrophy and uveitis described in endemic
  syphilis (Tabara)

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Take home

• The great imitator is still there
• Syphilis serolgy is mandatory in all patients
  presenting with unexplained intraocular
  inflammation
• It is an inexpensive and reliable tool to unmask
  the great imitator
• If not recognized in time, syphilitic retinitis
  may destroy the retina in a short time period
  (HIV patients)
• Excellent prognosis with early and adequate
  treatment

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Thank you !