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– Anterior

– Intermediate

– Posterior

– Panuveitis

• Clinical– Acute

– Chronic

– Recurrent

• Etiological– Exogenous– Endogenous

• Pathological– Granulomatous– Non-granulomatous

• Pathophysiology– Infectious– Inflammatory

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– 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

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

Iris Noodules• Bussaca

– 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– 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)

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– 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

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

?+++Antiparasites

GranulomaRChoroiditisLesion

UnilateralBilateralEye Involved

NoneInt. HostHumans

DogCatDef. Host

NematodeProtozoaParasite

ToxocaraToxoplasma

Retinal Vasculitis

• BOV, VF loss, scotomas, floaters from secondary vitritis

• May lead to retinal ischemia, infarction, hemorrhage, neovascularization

Involvement of arterioles, venules, or bothPerivascular sheathing or cuffing

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

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

• Meningoencephalomyelitic Syndrome

• Confusional Type

Treatment & Prognosis

• Immunosuppressives (Chlorambucil, AZT)

• Systemic corticosteroids

• 3.4 yrs to bilateral blindness +/- steroids

• 5-10% go blind in 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

- BAcute18-37 FPIC

++ BGradual

40-70 FBCR

+/- BAcute15-30 M/FApmpp

- UAcute15-40 M/F ARPE

- UAcute17-38 FMewds

+ BAcute20-40 FMCP

VitritisLateralBOVAgeSex

Treatment

None/steroidsPIC

CSABRC

NoneAPMMPE

NoneARPE

NoneMEWDS

Steroids/immunosuppsMCP

Treatment

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)

Masquerade Syndrome

• Group of disorders simulating chronic uveitis

• Often mistaken for chronic idiopathic uveitis

• Many are malignant; prompt dx needed.

Masquerade Syndromes

• Anterior Segment– RB

– Leukemia

– IOFB

– Malignant Melanoma

– JXG

– Peripheral RD

• Posterior Segment– RP

– Reticulum Cell Sarcoma

– Lymphoma

– Malignant Melanoma

– MS

Tumors of the Uvea

• Iris Nevus• Choroidal Nevus• Choroidal

Hemangioma

• Iris Melanoma• Ciliary Body

Melanoma• 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 break• Retinal Detachment• PVD• ARMD

• Retinal Vein Occlusion

• Trauma• Tumor• Sickle-Cell Disease• Subarachnoid or

subdural hemorrhage• Others

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

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– Follow-up closely

initially then every 6 months

– If (+) VH, treat as VH

• (+) Retinal Break– Laser or cryo tx to

prevent RD

Thank you very much!