Download Posterior Uveitis

Posterior Uveitis
Joseph Sowka, OD, FAAO, Diplomate
Symptoms
 Blurred vision
 Floaters
 Pain and photophobia seen in anterior uveitis is not likely to occur
Signs
 Mild anterior segment inflammation
 External eye typically white and quiet, depending upon etiology
 Anterior uveitis
 Typically asymptomatic spill-over from posterior chamber
 Occasionally granulomatous
 Vitritis
 Infiltrates
 Vascular sheathing
 Candlewax drippings
 Retinal scarring and RPE hyperplasia
 Fuzzy fundus lesions
 Inflammatory cell aggregation
 Snow balls and snow banks
 Periphlebitis
 Peripheral retinal neovascularization with attendant complications
 CME
 Retinitis
 Chorioretinitis
 Choroiditis
Types of Posterior Uveitis
 Traumatic/ surgical
 Infectious (syphilis, toxoplasmosis, etc)
 Infiltrative (sarcoidosis)
 Idiopathic (pars planitis)
Pars Planitis
 True intermediate/ posterior uveitis
 Younger patients
 Chronic
 May be asymptomatic
 Blurred vision
 Visual acuity ranges from 20/20 to no perception of light, with a mean range of 20/4020/50
 Cataracts
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 Vitreous debris
 CME
Pars planitis is typically bilateral, with both eyes affected in 85% of the cases
Seems to have an association with Crohn’s disease and, especially, multiple sclerosis
 45% positive association with between pars planitis and multiple sclerosis. You must
give strong consideration to ordering MRI of the brain in patients with pars planitis,
especially if the patient is in a high risk group
Vitreal cells
Retinal inflammatory exudates (snowballs) and periphlebitis
Inferior snowbanking of exudates
 While vitreous snowballs and snow banks are frequently encountered, they are by no
means present in every eye with pars planitis and need not be present to make this
diagnosis
 Exacerbations and remissions
 May last for years
Generally benign
 Treatment should be conservative and often involves only periodic monitoring, especially
if vision is only minimally disturbed by vitritis and CME
 This disease has a good prognosis with a final mean visual acuity for patients of
20/30-20/40 in 90% of cases
 There are exacerbations and remissions and typically this disorder runs a very long
course. Inflammatory mediators will increase vasopermeability of retinal capillaries
resulting in posterior segment inflammatory cells as well as CME.
Pars Planitis: Complications
 Posterior subcapsular cataracts (both from the disease itself and the treatment)
 Posterior vitreous detachment
 Frequent cause of rare PVD in young patients
 Neovascularization (mostly posterior segment)
 Attendant complications of vitreous hemorrhage and tractional RD
 Glaucoma (steroid induced, POAG, secondary inflammatory)
Pars Planitis: Treatment
 Observation
 Periocular, intravitreal, and systemic corticosteroids have all been employed, as well as other
immunosuppressive drugs. However, once a commitment to use systemic steroids is made,
typically they are used for months. With this treatment come the possible attendant
complications of steroid induced cataracts and glaucoma.
 Topical steroids only if there is concomitant anterior inflammation
 Anterior uveitis in pars planitis is not true anterior uveitis, but a spill over from the
posterior segment. These patients are typically asymptomatic.
 In severe or unresponsive cases, transscleral cryoretinopexy or thermal laser
photocoagulation can be directed against the snowbanks to destroy the inflamed areas along
with the infiltrates. These treatments can reduce intraocular inflammation, increase visual
acuity, and decrease dependence upon systemic steroids. Vitrectomy can also be used to
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clear the vitreous of both cells and hemorrhage
Clinical Pearl: When encountering a true PVD in a young patient, look for vitreal cells and
other signs of pars planitis.
Toxoplasmosis
 Number one cause of posterior uveitis
 Number one cause of focal chorioretinitis
 Caused by toxoplasma gondii
 Obligate intracellular protozoan parasite
 Retinal
 Hematogenous spread to eye
 Neural
 Congenital- passed from mother to child transplacentally after acquiring it during pregnancy
 Most common mode of transmission
 40% likelihood of fetal involvement
 Acquired (must consider AIDS)
 HIV testing needed
 Often without associated scarring
 Cat feces and undercooked meat are vectors
 Sporozoite (cat)
 Tachyzoit (proliferative form in humans)
 Bradyzoit (encysted and dormant)
 Bradyzoit sits in the NFL
 Bradyzoit usually sit near old scars and may remain viable for 25 yrs
 Immunosuppression can reactivate a bradyzoit
 May spontaneously reactivate without immunosuppression
 When active, toxoplasmosis produces a retinitis that appears as
 " Headlights in a fog " due to overlying focal vitritis
 Arteritis
 Periphlebitis
 Lesions heal within 3 weeks to 6 months
 Affects the posterior pole
 Vitritis usually located near an old scar- diagnostic
 Encystic organisms latent near old scar
Toxoplasmosis: Ocular Findings
 PVD
 CME
 Retinochoroiditis
 Scarring
 Arteritis
 Vasculitis
 Papillitis (totally destroys vision)
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Vitritis
RD
Toxoplasmosis: Other Thoughts
 Activity for 4-6 months
 Diagnosed by ELISA Toxoplasmosis titre
 Self limiting, but often treated
 Lesions which must be treated include large lesions (> 3DD), severe vitritis with vision
loss, and juxtafoveal or peripapillary lesions
 Results in chorioretinal scarring which may be visually disabling
Toxoplasmosis: Treatment
 Often simply monitored if vision not threatened and patient has healthy immune system (i.e.,
Not HIV/AIDS)
 Triple sulfonamide drugs
 Sulfadiazine 1 gm PO QID or Bactrim (trimethoprim 160 mg/sulfamethoxazole- 1 Double
Strength (DS) or 2 tabs BID) x 6 weeks (most common treatment)
 Bactrim DS every third day has shown to significantly reduce ocular toxoplasmosis
recurrences.
 Pyrimethamine (Daraprim- anti-parasitic)
 Causes bone marrow suppression which can be averted with folic acid supplementation
 25 mg PO QD x 6 weeks with Folic acid 5 mg Q2 days
 Clindamycin 250 mg QID
 Toxic and can cause colitis
 Spiramycin
 Steroids
 Prednisone 40 mg QD (only use prednisone in conjunction with the above meds- never
alone). Begin antimicrobial therapy for a few days first. Generally not used unless vision
significantly threatened.
Clinical Pearl: Though we have long known how to treat toxoplasmosis, it is not clear that
we should treat toxoplasmosis. There is a lack of evidence based medicine that identifies
treatment benefits. Controlled studies are clearly needed.
Clinical Pearl: Not every black spot on the retina is a toxoplasmosis scar, despite what
other optometrists tell you.
Clinical Pearl: The key diagnostic sign of toxoplasmosis is an active vitritis with a
"headlights in a fog appearance" adjacent to an area of old scarring.
Clinical Pearl: When encountering active toxoplasmosis, especially in a young patient,
strongly consider HIV testing.
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Toxocariasis
 Nematode - parasitic
 Puppies, eating dirt (geophagia), eating fecal matter (coprophagia) are the vectors
 Occurs in children and is usually unilateral
 Larvae travel in blood and lymph fluid
 Two forms: Never seen together
1. Ocular
 Ages 7-8
 Neuroretinitis
 Vitritis
 Papillitis
 RPE changes
 Elevated granuloma
 Decreased vision
 Leukocoria
 Chronic endophthalmitis
2. Systemic
 Ages 2-5
 ELISA
 Photocoagulation; cryo
 Corticosteroids (oral) for inflammation
 Closely related is the disease caused by the blackfly- onchocerciasis
Ocular Histoplasmosis Syndrome
 Fungal disease: Histoplasma capsulatum
 Associated with bird (pigeon, chicken) feces
 Actually in soil fertilized by bird feces
 Actually found in bat feces
 Ohio - Mississippi River Valley (or any river valley region)
 Inhaled fungus
 Inhaled mycelial spores of Histoplasma capsulatum
 These spores undergo transformation to the yeast phase in the lung, and from here it is
disseminated via the bloodstream to the rest of the body (including the eye where it
causes choroidal infection)
 Flu-like illness
 Retinal lesions reactivate 10-30 yrs later
 Affects ages 20-50
 Rare in patients of African descent
 Circumpapillary choroidal scarring
 Peripheral atrophic Histo spots & peripheral scars
 Punched-out lesions
 Large (1 DD) or small
 Hypo- or-hyperpigmented
 Foci of previously present inflammatory reaction
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 Site of infection with Histoplasma organism
Macular compromise
 Granulomatous inflammatory mass
Diagnosis is made by presence of peripapillary scarring and at least one peripheral Histo spot
Invisible choroiditis
 Not a fundus finding because it is not visible. May possibly be seen on FA
 Due to an accumulation of inflammatory cells at an inflammatory focus
 Will eventually result in an atrophic Histo spot
Ocular Histoplasmosis Syndrome: Maculopathy
 Macular granuloma
 Bruch's disruption
 Choroidal neovascularization
 4th most common cause of CNVM
 Sub-RPE hemorrhage with subsequent disciform scarring
 Lipid exudate
 Differential diagnosis
 Multifocal choroidopathy
 Acute posterior multifocal placoid pigment
 Multiple evanescent white dot syndrome
 Retinal pigment epithelialitis
 Serpiginous choroiditis
 Diffuse unilateral subacute neuroretinitis
Clinical Pearl: Ocular Histoplasmosis Syndrome can look exactly like multifocal
choroidopathy with one exception: OHS never causes cells to appear in the vitreous
because it is purely a choroiditis.
Clinical Pearl: Many peripheral spots look like Histo spots. To confirm the suspected
diagnosis in these cases, look for associated peripapillary scarring.
Ocular Histoplasmosis Syndrome: Treatment
 Routine f/u when inactive
 Home amsler to monitor for neovascularization
 Oral, depot steroids when active
 Some advocate that steroids are ineffective
 Photocoagulation for juxtafoveal neo
 Laser tx is mainstay for Histo
 30% recurrence rate for neo regrowth
 Risk factors are younger age and females
 Neo can spontaneously involute without treatment
 PDT commonly used
 Anti-angiogenic drugs are used as well
 60% of untreated patients develop 20/200 or worse vision
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30% chance of fellow eye involvement within 7 yrs
Clinical Pearl: Treatment isn’t directed at the cause of Histoplasmosis, but rather at the
neovascular maculopathy using standard methods.
Sarcoidosis
 Idiopathic disseminated granulomatous disease
 Non-caseating granulomas
 May have lid and conjunctival granuloma
 Multisystemic
 Females > males
 Blacks >>> Whites
 Ages 20-60 yrs
Sarcoidosis: Ocular Manifestations
 Keratoconjunctivitis sicca with lacrimal gland involvement by granuloma
 Granulomatous anterior uveitis
 Mutton fat KP’s and posterior synechiae
 Periphlebitis
 Candlewax drippings
 More common than retinal granulomas
 Adjacent ‘puff balls’
 Vitritis/retinitis
 Peripheral vascular occlusion and neovascularization
 CME
 From inflammation
 Optic neuropathy
 Inflammatory, infiltrative, or compressive optic neuropathy
 Disc edema
Sarcoidosis: Diagnosis
 Hilar adenopathy (enlarged pulmonary lymph nodes) on chest x-ray (CXR)
 Angiotensin converting enzyme (ACE)
 Gallium scan
 Uptake of gallium only in sarcoidosis after gallium is injected into venous system (uptake
in salivary and lacrimal glands and hilar lymph nodes)
 Conjunctival biopsy or biopsy of skin granulomas
Sarcoidosis: Treatment
 Recognition of sarcoid in differential diagnosis
 Oral steroids and Periocular steroids
Clinical Pearl: Sarcoidosis should be high on your list of differential diagnoses when
encountering retinal periphlebitis.
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Eales' Disease
 Difficult to classify
 Vascular occlusion secondary to posterior segment inflammation
 Retinal periphlebitis
 Idiopathic bilateral periphlebitis affecting retinal veins
 Healthy young men in 20's-30's
 25% have hearing/balance difficulties
 Patient may complain of floaters, but is typically asymptomatic early in the disease
 Possibly with associated anterior uveitis
 Obscure inflammatory reaction to antigens
 High association to tuberculoprotein sensitivity
 Retinal findings include perivascular sheathing and capillary non-perfusion
 Sufficient capillary non-perfusion can lead to retinal neovascularization with attendant
complications
 Vitritis occurs overlying periphlebitis
 Retinal telangiectasias may occur with resultant retinal/macular edema
 Vision reduction can come from retinal/macular edema, vitreous hemorrhage, tractional
retinal detachment
 In late stages/recovered stages, there is often perivascular scarring/RPE hyperplasia
Eale’s Disease: Management
 Rule out tuberculosis
 FA if neovascularization is suspected
 Focal photocoagulation if macular edema develops from telangiectasias or PRP for
retinal/disc neovascularization
 Often, the disease self limits
Clinical Pearl: Two key features of Eale's disease are venous peri-phlebitis and venous
obstruction.
Clinical Pearl: Eales’ disease targets healthy, young males.
Clinical Pearl: Suspect Eale’s Disease in cases of posterior segment neovascularization,
vitreous hemorrhage, tractional detachment in young, healthy males with no history of
diabetes.
Clinical Pearl: Suspect Eale’s disease when encountering perivascular scarring.
Syphilis:
 Caused by spirochetal bacteria Treponema pallidum
 Possible ocular findings:
 Salt-n-pepper fundus (like RP)
 Multifocal area of RPE atrophy
 Uveitis (anterior and/or posterior)
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 Periphlebitis
 Vasculitis
 Choroiditis
 Retinitis
 Papillitis or retrobulbar neuropathy
 Vitritis
 Intraretinal hemorrhage
 Peripheral neovascularization and attendant complications
Diagnosis:
 Non-specific (RPR, VDRL) and specific (FTA-ABS, MHA-TP) tests
Management:
 Systemic antibiosis
Clinical Pearl: Syphilis should be high on your list of differential diagnoses when
encountering retinal periphlebitis.
Clinical Pearl: Whenever you see retinal periphlebitis, vasculitis, or candlewax drippings,
immediately think of sarcoidosis and syphilis.
Behcet’s Syndrome:
 Painful aphthous oral/genital ulcers
 Arthritis
 Conjunctivitis, anterior uveitis
 Endophthalmiitis
 Arteritis
 Periphlebitis
 Necrotizing retinitis
 Retinal edema, exudation
Endophthalmitis
 Post-surgical
 Post-penetrating foreign body
 Indwelling catheter as portal of entry for microbes
 Rarely endogenous
 Fungi
 Extremely inflamed eye
 Hypopyon
 Staphylococcus; streptococcus; pseudomonas
 Poor prognosis
 Intravitreal injections of antibiotics
 Eye may be totally lost
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“The White Dot Syndromes”
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
 Bilateral with accompanying vision loss (often severe)
 Often with viral prodrome several weeks antecedent
 Floaters, mild eye pain, metamorphopsia and/or scotomas
 Young healthy adults
 May have inflammations elsewhere
 May have concurrent episcleritis
 HLA B27 association
 Yellow - white placoid lesions
 Cream colored lesions
 May be gray
 Discrete and flat
 May have associated disc swelling (rare)
 May have overlying vitritis
 May have shallow subretinal fluid over large lesions
 Lesions fade within days to leave RPE mottling
 RPE and inner choroid affected
 Likely a choroidal vasculitis, post viral autoimmune disorder, or may be related to
spirochete disease
 FA: early hypofluorescence due to blockage, then late staining
 Blockage and accumulation in RPE
 Resolves within 4 weeks
 Recurrence uncommon, but if it does recur, it will do so within 3 months typically
 No tx
 Prognosis excellent
 Pts. generally recover 20/40 or better
 May have a (rare) associated CNS vasculitis which is life threatening and requires systemic
steroids
 Px must be instructed to return immediately if they develop severe HA or other
neurological symptoms
Birdshot Retinochoroidopathy
 Vitiliginous
 Chronic disease in healthy middle-aged patients (females)
 Bilateral decreased vision (20/50).
 Floaters, blurred vision, nyctalopia, field defects
 Multiple depigmented, creamy spots in posterior pole following vessels
 When fresh, there is ‘substance’, but becomes more atrophic later
 RPE hyperpigmentation
 Chronic lesions can become confluent and spread to the macula
 Vitritis
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Disc edema
Retinal vasculitis
Epiretinal membrane formation
CME
Autoimmune disease
(+) HLA A29 highly correlated
Oral steroids
NVD; NVE
FA: filling delay and vessel leakage.
Prognosis is poor due to chronicity
Multiple Effervescent White Dot Syndrome (MEWDS):
 Likely a group of disorders
 Healthy young patients (typically females)
 Viral prodrome
 Unilateral, rapid with vision loss
 20/200
 Photopsia with or within field loss very diagnostic
 Markedly enlarged blind spot without a fundus correlate to explain the field loss
 White dots within the outer photoreceptor level, particularly the macular area
 Old lesions manifest as tiny orange dots in the fovea
 ¼-1/3rd DD
 Macular granularity
 Vitritis, periphlebitis
 FA: early hyperfluorescence of punctate lesions in “wreath appearance”. Late staining of
punctate lesions & leakage of ONH.
 No known treatment
 White spots disappear over days to weeks
 Vision typically returns (1-10 weeks), but field defects may remain
 No RPE residual irregularities
Multifocal Choroiditis
 Pseudo-Histoplasmosis
 Young myopic females
 Blurred vision and blind spot enlargement
 Multiple small white round lesions surrounded by pigment in posterior pole
 Punctate inner choroidopathy
 Presence of vitreous cells differentiates this from true Histoplasmosis
 Prognosis good - Responds well to steroids
Clinical Pearl: There are posterior uveitic syndromes such as toxoplasmosis and some
white dot syndromes that are visually recognizable. However, the majority of posterior
uveitis syndromes present with signs and symptoms of posterior inflammation which do not
necessarily identify the causative condition.
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