Diseases of the Uvea & Vitreous

Vicente Victor D. Ocampo, Jr., MD, DPBO

Pamantasan Ng Lungsod Ng Maynila
 Objectives
• To discuss and differentiate the various
diseases of the uveal tract
• To discuss and differentiate the various
diseases of the vitreous
 Uveal Tract
• Iris
• Ciliary Body
• Choroid
 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
 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
 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
 Classification of Uveitis
• Anatomical • Etiological
– Anterior – Exogenous
– Intermediate – Endogenous
– Posterior • Pathological
– Panuveitis – Granulomatous
– Non-granulomatous
• Clinical
• Pathophysiology
– Acute
– Infectious
– Chronic
– Inflammatory
– Recurrent
 Onset
• Sudden – pain,redness,photophobia
• Insidious – painless, white eye
 Duration
• Limited – 3 mos or less
• Persistent > 3 mos
 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
 Pathology
• Granulomatous • Non-granulomatous
– chronic,insidious – acute
– large,mutton-fat kp's – smaller kp's
– ++ ps – less likelihood fr ps
– ++ busacca nod – (-) busacca nod
– ++ koeppe nod – +/- koeppe nods
– TB,VKH,SO, sarcoid – most ant uveitides
 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
 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
 Markers of Inflammation
• Cells
• Flare
• Keratic Precipitates
• Iris Nodules
 Cells and Flare

• Cells – sign of active inflammation

• Aqueous flare – proteinaceus materials
leaking from damaged iris blood vessels
 Keratic Precipitates
• cellular aggregates that form on the corneal
endothelium
• Bussaca
Iris Noodules
– in iris stroma
– granulomatous

• Koeppe
– pupil margin
 Acute Anterior Uveitis
• Pain, redness, photophobia
• Several days to weeks
• Acute & unilateral
• Recurrences common
• 50-70% idiopathic
 Signs of Acute Anterior Uveitis
• Non-pigmented KP’s
• AC cells & flare
• Ciliary injection
• Fibrin
• Hypopyon
• Hypotony
 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
 Ankylosing Spondylitis
• 88% HLA-B27
positive
• Chronic backache &
stiffness during 2nd-3rd
decades
• Sacroiliitis, sclerosis,
narrowing of joint
space
 Reiter’s Syndrome
• Non-specific
urethritis,
polyarthritis,
conjunctivitis/iritis
• 85-95% HLA-B27
positive
• Keratoderma
blennorhagicum.
Circinate balanitis
Signs of Chronic Anterior Uveitis
• Band K
• Posterior synechiae
• Pigmented KP’s
• Cataract
• Glaucoma
 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
 High Risk Factors
• Girls > Boys
• Pauciarticular (80-90%)
• Wrist-sparing, affects lower extremity
• ANA(+)
 Intermediate Uveitis
• Intraocular
inflammation
predominantly
involving the
vitreous &
peripheral retina
• Not part of a
specific disease
entity
 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
 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
 Signs & Symptoms

• BOV & floaters

• Rarely w/ pain,
redness &
photophobia
• Bilateral
• Always w/ vitritis
• Snowballs
• Snowbanks
• Minimal AC rxn
 Snowballs
• White & yellow
• Epithelioid cells &
multinucleated
giant cells which
are not found in
the uvea
• Inferior vitreous
 Snowbanks
• Yellowgray
exudates
• Usually in inferior
ora
 Vascular Abnormalities
• Perivasculitis
• Whitish retinal
infiltrates
• Neovasc
• Cyclitic
membrane
 Complications
• CME (30%)
• ODE (50%)
• Vit Hem (3%)
• Glaucoma (8%)
• Cataract (46%)
• RD (5%)
• Synechiae (25%)
 Prognosis
• More vitritis & macular edema w/
snowbanks.
• Severity of disease related to visual
outcome.
• Only 5% remission.
• Burn-out eventually.
 Causes of Posterior Uveitis
• Focal Retinitis • Focal Choroiditis
– Toxoplasmosis – Toxocariasis
– Onchocerciasis – TB
– Cysticercosis – Nocardiosis
– Masquerade Syndrome – Masquerade Syndrome
• Mulitfocal Retinitis • Multifocal Choroiditis
– Syphilis – Histoplasmosis
– Herpes Simplex virus – SO
– CMV – VKH
– Sarcoidosis – Sarcoidosis
– Masquerade – Serpiginous Choroidopathy
– Candidiasis – Birdshot Choroidopathy
– Meningococcus – Masquerade Syndrome
(metastatic tumor)
 Ocular Toxoplasmosis
• 30-50% of posterior uveitis
• Intracellular protozoan Toxoplasma gondii
• Def host: cat; Intermediate host: human
– 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
• Floaters,BOV
• Focal area of retinochoroiditis
• Mild to severe vitritis
• “Headlight in the fog”

– Reactivation-
satellite lesion
at edge of
previous lesions
– Vasculitis and
optic disc
involvement

• Endpoint: scarring
 Treatment
• Self-limited; not all needs to be treated
• Indications for treatment:
– Peripapillary or posterior pole involvement
– Severe vitritis w/ decreased vision
 Treatment Regimen
• Regimen 1 • Regimen 3
– Pyrimethamine – Trimethoprim/Sulfame
thoxazole
– Sulfadiazine
• Notes
– Folic Acid
– Prednisone 24 hrs.
• Regimen 2 after Ab
– Clindamycin – Never steroids alone
– Sulfadiazine – Duration of Tx: 2-8
weeks ( ave: 4 wks)
– Endpt: scar formation
 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
 Toxoplasmosis vs Toxocara
Toxoplasma Toxocara
Parasite Protozoa Nematode
Def. Host Cat Dog
Humans Int. Host None
Eye Involved Bilateral Unilateral
Lesion RChoroiditis Granuloma
Antiparasites +++ ?
 Retinal Vasculitis
• BOV, VF loss,
scotomas, floaters
from secondary vitritis
• May lead to retinal
ischemia, infarction,
hemorrhage,
neovascularization
Involvement of arterioles, venules, or both
Perivascular sheathing or cuffing
Conditions w/ Retinal Vasculitis
• Behcet’s Syndrome • Syphilis
• Collagen-vascular dx • TB
– WG,SLE.PAN • Int. Uveitis
• Sarcoidosis • Toxoplasmosis
• MS • ARN
• IBD • HSV/HZV/CMV
• Birdshot RC
• Eales’ Disease
 Behcet’s Disease
• Systemic
obliterative
vasculitis
• Unknown cause
• Common in Japan,
Middle East, Far
East, &
Mediterranean
countries
 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
• Meningoencephalomyel
itic Syndrome
• Confusional Type
 Treatment & Prognosis
• Immunosuppressives • 3.4 yrs to bilateral
(Chlorambucil, AZT) blindness +/- steroids
• Systemic • 5-10% go blind in
corticosteroids spite of all known tx
Vogt-Koyanagi-Harada’s Disease
(VKH)
• Bilateral, diffuse granulomatous panuveitis
• Often recurrent
• Multisystemic inflammatory disease
• Involves melanocytes of uvea, retina,
meninges, skin
 Epidemiology
• More common in pigmented races
• More common in females (55-78%)
• Most in 2nd to 5th decade of life
 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
3. Neurologic signs of tinnitus, neck
stiffness, cranial nerve or CNS problems,
CSF pleocytosis
4. Cutaneous findings such as alopecia,
poliosis, or vitiligo
 Clinical Phases
1. Prodromal Phase
2. Uveitic Phase
3. Convalescent Phase
4. Chronic Recurrent Phase
 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.
 White Dot Syndromes
• Several ocular
disorders
• (+) Discrete light-
colored lesions in
fundus during at least
one phase of the
disease
• May be interrelated
 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)
 White Dot Syndromes
Sex Age BOV Lateral Vitritis
MCP F 20-40 Acute B +
Mewds F 17-38 Acute U -

ARPE M/F 15-40 Acute U -

Apmpp M/F 15-30 Acute B +/-

BCR F 40-70 Gradu B ++

al
PIC F 18-37 Acute B -
 Treatment
Treatment
MCP Steroids/immunosupps
MEWDS None
ARPE None
APMMPE None
BRC CSA
PIC None/steroids
 Causes of Posterior Uveitis
• Focal Retinitis • Focal Choroiditis
– Toxoplasmosis – Toxocariasis
– Onchocerciasis – TB
– Cysticercosis – Nocardiosis
– Masquerade Syndrome – Masquerade Syndrome
• Mulitfocal Retinitis • Multifocal Choroiditis
– Syphilis – Histoplasmosis
– Herpes Simplex virus – SO
– CMV – VKH
– Sarcoidosis – Sarcoidosis
– Masquerade – Serpiginous Choroidopathy
– Candidiasis – Birdshot Choroidopathy
– Meningococcus – Masquerade Syndrome
(metastatic tumor)
 Masquerade Syndrome
• Group of disorders simulating chronic
uveitis
• Often mistaken for chronic idiopathic
uveitis
• Many are malignant; prompt dx needed.
 Masquerade Syndromes
• Anterior Segment • Posterior Segment
– RB – RP
– Leukemia – Reticulum Cell
– IOFB Sarcoma
– Malignant Melanoma – Lymphoma
– JXG – Malignant Melanoma
– Peripheral RD – MS
 Tumors of the Uvea
• Iris Nevus • Iris Melanoma
• Choroidal Nevus • Ciliary Body
• Choroidal Melanoma
Hemangioma • Choroidal Melanoma
 Iris Nevus
• Common, benign
• Pigmented, flat or
slightly elevated
lesion in superficial
layer of iris
• Assoc’n w/
neurofibromatosis I
(Lisch nodules)
 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
Choroidal Melanoma
 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
 Functions
• Transparent medium
occupying major
volume of the globe
• Absorbs and
redistributes forces
applied to surrounding
ocular tissues
 Diseases of the Vitreous
• Vitritis
• Vitreous Hemorrhage
• Posterior Vitreous Detachment
 Vitreous Hemorrhage
• Bleeding inside the
vitreous cavity
• Sudden painless LOV
or sudden appearance
of black spots with
flashing lights
 Causes of Vitreous Hemorrhage
• DM Retinopathy • Retinal Vein
• Retinal break Occlusion
• Retinal Detachment • Trauma
• PVD • Tumor
• ARMD • Sickle-Cell Disease
• Subarachnoid or
subdural hemorrhage
• Others
 Treatment
• Determine etiology • Surgical removal
• Bed rest w/ head – VH +RD
elevation for 2-3 days – VH > 6 mos
• d/c ASA, NSAIDS – VH + neovasc of iris
unless necessary – Hemolytic or ghost cell
glaucoma
 Posterior Vitreous Detachment
(PVD)
• Posterior vitreous
separates from retina
and collapses toward
vitreous base
• Floaters and flashing
lights
– Floaters = vitreous
opacity casting shadow
on retina
– Flashing lights =
physical stimulation of
the retina due to
vitreoretinal traction
 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
 Management
• No Retinal Break • (+) Retinal Break
– Follow-up closely – Laser or cryo tx to
initially then every 6 prevent RD
months
– If (+) VH, treat as VH
Thank you very much!