Download - Diseases of the Uvea and Vitreous
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Diseases of the Uvea & Vitreous
Vicente Victor D. Ocampo, Jr., MD, DPBO
Pamantasan Ng Lungsod Ng Maynila
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Objectives
• To discuss and differentiate the various diseases of the uveal tract
• To discuss and differentiate the various diseases of the vitreous
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Uveal Tract
• Iris
• Ciliary Body
• Choroid
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Uveal Tract
• Pigmented vascular middle coat
• From optic disc to pupil
• Blood supply from ophthalmic artery– CB & iris – Long ciliary arteries (2)– Choroid – Short ciliary arteries (10-20)
• Mesodermal in origin; CB & iris have neuro-ectodermal components also
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Physiology
• Iris – regulates size of pupil
• Ciliary body– secretes aqueous humor– controls accommodative power of the eye
• Choroid – provides nourishment for RPE and outer retina
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Uveitis
• Inflammation of uveal tract
• Compartmentalization due to separate distribution of blood supply
• Severity: Iris < CB < Choroid due to vascularity and cellularity
• May also involve adjacent structures like sclera,retina,vitreous,optic nerve
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Classification of Uveitis
• Anatomical– Anterior
– Intermediate
– Posterior
– Panuveitis
• Clinical– Acute
– Chronic
– Recurrent
• Etiological– Exogenous– Endogenous
• Pathological– Granulomatous– Non-granulomatous
• Pathophysiology– Infectious– Inflammatory
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Onset
• Sudden – pain,redness,photophobia
• Insidious – painless, white eye
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Duration
• Limited – 3 mos or less
• Persistent > 3 mos
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Clinical Course
• Acute– sudden onset, limited duration– many cells, severe flare
• Recurrent– repeated attacks separated by periods of
inactivity w/o tx of at least 3 mos
• Chronic– persistent inflammation w/in 3 mos after d/c of
tx
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Pathology
• Granulomatous– chronic,insidious– large,mutton-fat kp's– ++ ps– ++ busacca nod– ++ koeppe nod– TB,VKH,SO, sarcoid
• Non-granulomatous– acute– smaller kp's– less likelihood fr ps– (-) busacca nod– +/- koeppe nods– most ant uveitides
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Infectious Uveitis • Etiology
– Bacterial: TB, syphilis,staph– Fungal: Histoplasmosis, coccidiomycosis– Viral: HSV, HZV, CMV– Parasitic: Toxoplasmosis, toxocariasis
• Due to perforating injury (exogenous), infection in eye (ocular), or emboli from body (endogenous)
• Suppuration of uvea, retina, vitreous leads to endophthalmitis
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Nomenclature
• Uveitis – inflammation of uveal tract
• Iritis – inflammation confined to anterior chamber
• Iridocyclitis – spill over to rentrolental space
• Keratouveitis – spill over to cornea
• Sclerouveitis – sclera + iris
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Markers of Inflammation
• Cells• Flare• Keratic Precipitates• Iris Nodules
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Cells and Flare
• Cells – sign of active inflammation• Aqueous flare – proteinaceus materials
leaking from damaged iris blood vessels
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Keratic Precipitates• cellular aggregates that form on the corneal
endothelium
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Iris Noodules• Bussaca
– in iris stroma– granulomatous
• Koeppe– pupil margin
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Acute Anterior Uveitis
• Pain, redness, photophobia
• Several days to weeks
• Acute & unilateral
• Recurrences common
• 50-70% idiopathic
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Signs of Acute Anterior Uveitis
• Non-pigmented KP’s• AC cells & flare• Ciliary injection• Fibrin• Hypopyon• Hypotony
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HLA-B27 Assoc’d Uveitides
• HLA-B27 –genotype in short arm Chrom6• 50-60% in acute iritis px• Seronegative spondyloarhtropathies
– Ankylosing Spondylitis– Reiter Syndrome– IBD– Psoriatic Arthritis– Post-infectious or Reactive Arthritis
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Ankylosing Spondylitis
• 88% HLA-B27 positive
• Chronic backache & stiffness during 2nd-3rd decades
• Sacroiliitis, sclerosis, narrowing of joint space
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Reiter’s Syndrome• Non-specific
urethritis, polyarthritis, conjunctivitis/iritis
• 85-95% HLA-B27 positive
• Keratoderma blennorhagicum. Circinate balanitis
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Signs of Chronic Anterior Uveitis
• Band K• Posterior synechiae• Pigmented KP’s• Cataract• Glaucoma
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Juvenile Rheumatoid Arthritis
• Arthritis in a child < 16 y.o– Mean age : 6 y.o.
• RF (-)• Low-grade uveitis
– White eye
• Chronic, recurrent course
• Vision-threatening– Close follow-up
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High Risk Factors
• Girls > Boys
• Pauciarticular (80-90%)
• Wrist-sparing, affects lower extremity
• ANA(+)
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Intermediate Uveitis
• Intraocular inflammation predominantly involving the vitreous & peripheral retina
• Not part of a specific disease entity
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Epidemiology
• 4-15 % of uveitis px
• Up to 25% of uveitis in children, mostly pars planitis
• Usually 2nd to 4th decades of life
• No sex or race predilection
• Familial intermediate uveitis
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Pars Planitis
• Subset of intermediate uveitis
• White opacity over pars plana & ora serrata (snowbank)
• Often with worse vitritis, more severe macular edema, & worse visual prognosis
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Signs & Symptoms
• BOV & floaters• Rarely w/ pain,
redness & photophobia
• Bilateral
• Always w/ vitritis• Snowballs • Snowbanks• Minimal AC rxn
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Snowballs
• White & yellow • Epithelioid cells &
multinucleated giant cells which are not found in the uvea
• Inferior vitreous
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Snowbanks• Yellowgray
exudates
• Usually in inferior ora
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Vascular Abnormalities
• Perivasculitis
• Whitish retinal infiltrates
• Neovasc
• Cyclitic membrane
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Complications
• CME (30%)• ODE (50%)• Vit Hem (3%)• Glaucoma (8%)• Cataract (46%)• RD (5%)• Synechiae (25%)
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Prognosis
• More vitritis & macular edema w/ snowbanks.
• Severity of disease related to visual outcome.
• Only 5% remission.
• Burn-out eventually.
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Causes of Posterior Uveitis
• Focal Retinitis– Toxoplasmosis– Onchocerciasis– Cysticercosis– Masquerade Syndrome
• Mulitfocal Retinitis– Syphilis– Herpes Simplex virus– CMV– Sarcoidosis– Masquerade– Candidiasis– Meningococcus
• Focal Choroiditis– Toxocariasis– TB– Nocardiosis– Masquerade Syndrome
• Multifocal Choroiditis– Histoplasmosis– SO– VKH– Sarcoidosis– Serpiginous Choroidopathy– Birdshot Choroidopathy– Masquerade Syndrome
(metastatic tumor)
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Ocular Toxoplasmosis
• 30-50% of posterior uveitis
• Intracellular protozoan Toxoplasma gondii
• Def host: cat; Intermediate host: human
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– Ingestion of encysted form (bradyzoite) w/c has predilection for cardiac, muscular, & neural tissue (e.g. retina); may lie dormant
– Rupture of cyst releases tachyzoite/ trophozoite w/ reactivation of infection
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• Floaters,BOV
• Focal area of retinochoroiditis
• Mild to severe vitritis
• “Headlight in the fog”
– Reactivation- satellite lesion at edge of previous lesions
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– Vasculitis and optic disc involvement
• Endpoint: scarring
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Treatment
• Self-limited; not all needs to be treated
• Indications for treatment:– Peripapillary or posterior pole involvement– Severe vitritis w/ decreased vision
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Treatment Regimen
• Regimen 1– Pyrimethamine
– Sulfadiazine
– Folic Acid
• Regimen 2– Clindamycin
– Sulfadiazine
• Regimen 3– Trimethoprim/
Sulfamethoxazole
• Notes– Prednisone 24 hrs.
after Ab– Never steroids alone– Duration of Tx: 2-8
weeks ( ave: 4 wks)– Endpt: scar formation
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Prognosis
• Untreated: – retinochoroiditis resolves bet 3 wks & 6 mos
( ave: 4.2 mos)
• Treated:– Clindamycin tx results in a 2-6 wk resolution
and an 8% 3-year recurrence rate– Daraprim/sulfa results in 15% recurrence rate
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Toxoplasmosis vs Toxocara
?+++Antiparasites
GranulomaRChoroiditisLesion
UnilateralBilateralEye Involved
NoneInt. HostHumans
DogCatDef. Host
NematodeProtozoaParasite
ToxocaraToxoplasma
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Retinal Vasculitis
• BOV, VF loss, scotomas, floaters from secondary vitritis
• May lead to retinal ischemia, infarction, hemorrhage, neovascularization
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Involvement of arterioles, venules, or bothPerivascular sheathing or cuffing
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Conditions w/ Retinal Vasculitis
• Behcet’s Syndrome• Collagen-vascular dx
– WG,SLE.PAN
• Sarcoidosis• MS• IBD
• Syphilis• TB• Int. Uveitis• Toxoplasmosis• ARN• HSV/HZV/CMV• Birdshot RC• Eales’ Disease
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Behcet’s Disease
• Systemic obliterative vasculitis
• Unknown cause• Common in Japan,
Middle East, Far East, & Mediterranean countries
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Diagnostic Criteria
• Major Criteria– Recurrent oral ulcers
– Skin lesions
– Genital ulcers
– Ocular lesions• Recurrent hypopyon
iritis/iridocyclitis
• Chorioretinitis
• Minor Criteria– Arthritis
– GI lesions
– Epididymitis
– Vascular Lesions
– CNS Involvement• Brainstem Syndrome
• Meningoencephalomyelitic Syndrome
• Confusional Type
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Treatment & Prognosis
• Immunosuppressives (Chlorambucil, AZT)
• Systemic corticosteroids
• 3.4 yrs to bilateral blindness +/- steroids
• 5-10% go blind in spite of all known tx
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Vogt-Koyanagi-Harada’s Disease (VKH)
• Bilateral, diffuse granulomatous panuveitis
• Often recurrent
• Multisystemic inflammatory disease
• Involves melanocytes of uvea, retina, meninges, skin
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Epidemiology
• More common in pigmented races
• More common in females (55-78%)
• Most in 2nd to 5th decade of life
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Criteria for Diagnosis (AUS, 1978)
• No history of previous ocular trauma or surgery
• At least 3 of the ff:
1.Bilateral chronic iridocyclitis
2. Posterior uveitis, incl. SRD, disc hyperemia, subretinal macular edema, “sunset glow” fundus
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3. Neurologic signs of tinnitus, neck stiffness, cranial nerve or CNS problems, CSF pleocytosis
4. Cutaneous findings such as alopecia, poliosis, or vitiligo
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Clinical Phases
1. Prodromal Phase
2. Uveitic Phase
3. Convalescent Phase
4. Chronic Recurrent Phase
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Traditional Treatment
• Early & aggressive use of systemic steroids, followed by slow tapering over 3 to 6 months.
• Immunosuppressive drugs used for cases refractory to steroids or when px cannot tolerate steroids.
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White Dot Syndromes
• Several ocular disorders
• (+) Discrete light-colored lesions in fundus during at least one phase of the disease
• May be interrelated
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White Dot Syndromes
• Multifocal Choroiditis & Panuveitis (MCP)• Multiple Evanescent White Dot Syndrome
(MEWDS)• Acute Retinal Pigment Epithelitis (ARPE)• Acute Posterior Multifocal Placoid Pigment
Epitheliopathy (APMPPE)• Birdshot Retinochoroidopathy (BRC)• Punctate Inner Choroiditis (PIC)
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White Dot Syndromes
- BAcute18-37 FPIC
++ BGradual
40-70 FBCR
+/- BAcute15-30 M/FApmpp
- UAcute15-40 M/F ARPE
- UAcute17-38 FMewds
+ BAcute20-40 FMCP
VitritisLateralBOVAgeSex
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Treatment
None/steroidsPIC
CSABRC
NoneAPMMPE
NoneARPE
NoneMEWDS
Steroids/immunosuppsMCP
Treatment
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Causes of Posterior Uveitis
• Focal Retinitis– Toxoplasmosis– Onchocerciasis– Cysticercosis– Masquerade Syndrome
• Mulitfocal Retinitis– Syphilis– Herpes Simplex virus– CMV– Sarcoidosis– Masquerade– Candidiasis– Meningococcus
• Focal Choroiditis– Toxocariasis– TB– Nocardiosis– Masquerade Syndrome
• Multifocal Choroiditis– Histoplasmosis– SO– VKH– Sarcoidosis– Serpiginous Choroidopathy– Birdshot Choroidopathy– Masquerade Syndrome
(metastatic tumor)
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Masquerade Syndrome
• Group of disorders simulating chronic uveitis
• Often mistaken for chronic idiopathic uveitis
• Many are malignant; prompt dx needed.
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Masquerade Syndromes
• Anterior Segment– RB
– Leukemia
– IOFB
– Malignant Melanoma
– JXG
– Peripheral RD
• Posterior Segment– RP
– Reticulum Cell Sarcoma
– Lymphoma
– Malignant Melanoma
– MS
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Tumors of the Uvea
• Iris Nevus• Choroidal Nevus• Choroidal
Hemangioma
• Iris Melanoma• Ciliary Body
Melanoma• Choroidal Melanoma
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Iris Nevus
• Common, benign• Pigmented, flat or
slightly elevated lesion in superficial layer of iris
• Assoc’n w/ neurofibromatosis I (Lisch nodules)
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Choroidal Melanoma
• Most common primary intraocular tumors in adults
• 6th decade of life, rarely before 30 and after 80
• Mushroom-shaped mass
• Secondary exudative RD
• Enucleation for large tumors
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Choroidal Melanoma
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Vitreous• 4/5 of volume of the globe
• 4.0 g, 4.0 ml
• Gel-like structure – collagen framework + hydrated hyaluronic acid
• 99% H20
• Passageway for metabolites used by lens, ciliary body, and retina
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Functions
• Transparent medium occupying major volume of the globe
• Absorbs and redistributes forces applied to surrounding ocular tissues
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Diseases of the Vitreous
• Vitritis
• Vitreous Hemorrhage
• Posterior Vitreous Detachment
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Vitreous Hemorrhage
• Bleeding inside the vitreous cavity
• Sudden painless LOV or sudden appearance of black spots with flashing lights
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Causes of Vitreous Hemorrhage
• DM Retinopathy• Retinal break• Retinal Detachment• PVD• ARMD
• Retinal Vein Occlusion
• Trauma• Tumor• Sickle-Cell Disease• Subarachnoid or
subdural hemorrhage• Others
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Treatment
• Determine etiology• Bed rest w/ head
elevation for 2-3 days• d/c ASA, NSAIDS
unless necessary
• Surgical removal– VH +RD
– VH > 6 mos
– VH + neovasc of iris
– Hemolytic or ghost cell glaucoma
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Posterior Vitreous Detachment (PVD)
• Posterior vitreous separates from retina and collapses toward vitreous base
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• Floaters and flashing lights– Floaters = vitreous
opacity casting shadow on retina
– Flashing lights = physical stimulation of the retina due to vitreoretinal traction
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Prevalence and Incidence
• 27% bet 60-69 y.o., 63% > 70 y.o.
• 10-15% asymptomatic PVD w/ retinal tear
• 70% PVD + VH w/ retinal tear
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Management
• No Retinal Break– Follow-up closely
initially then every 6 months
– If (+) VH, treat as VH
• (+) Retinal Break– Laser or cryo tx to
prevent RD
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Thank you very much!