UVEA & SCLERAUVEA & SCLERA

JUAN S. LOPEZ, MDJUAN S. LOPEZ, MDChief, Section of UveitisChief, Section of Uveitis

Institute of Ophthalmology and Visual SciencesInstitute of Ophthalmology and Visual SciencesSt. Luke’s Medical CenterSt. Luke’s Medical Center

UVEAL TRACTUVEAL TRACT Middle vascular Middle vascular

layer of the eyelayer of the eye Protected by the Protected by the

cornea and scleracornea and sclera Contributes blood Contributes blood

supply to the supply to the retinaretina

Composed of:Composed of: ChoroidChoroid Ciliary body Ciliary body IrisIris

Iris Ciliary Body

Choroid

IRISIRIS Anterior extension of the Anterior extension of the

ciliary bodyciliary body PUPIL – central round aperturePUPIL – central round aperture Divides the anterior from the Divides the anterior from the

posterior chamber posterior chamber

LAYERS:LAYERS: STROMA – anterior portion with chromatophores STROMA – anterior portion with chromatophores

containing melanincontaining melanin

- Contains the sphincter and dilator muscles- Contains the sphincter and dilator muscles PIGMENTED EPITHELIUM – posterior portion PIGMENTED EPITHELIUM – posterior portion

- anterior extension of the neuroretina and - anterior extension of the neuroretina and retinal retinal

pigment epitheliumpigment epithelium

FUNCTION: regulates pupil size

CILIARY BODYCILIARY BODY From anterior end of From anterior end of

the choroid to the root the choroid to the root of the irisof the iris

Consist of:Consist of: PARS PLICATAPARS PLICATA

Corrugated, Corrugated, anterioranterior

Where ciliary Where ciliary processes ariseprocesses arise

PARS PLANAPARS PLANA Flattened, Flattened,

posteriorposterior

Composed of capillaries and veins that Composed of capillaries and veins that drain through the vortex veinsdrain through the vortex veins

2 layers of ciliary epithelium:2 layers of ciliary epithelium: INTERNAL NONPIGMENTED LAYERINTERNAL NONPIGMENTED LAYER EXTERNAL PIGMENTED LAYEREXTERNAL PIGMENTED LAYER

CILIARY MUSCLECILIARY MUSCLE Longitudinal – inserts into the trabecular Longitudinal – inserts into the trabecular

meshworkmeshwork Circular contracts and relaxes the zonular Circular contracts and relaxes the zonular

fibersfibers Radial Radial

CILIARY PROCESSES responsible for the formation CILIARY PROCESSES responsible for the formation of aqueousof aqueous

FUNCTIONS:1. Acts in accommodation2. Secretes aqueous

humor

IRIS AND CILIARY BODYIRIS AND CILIARY BODY

Blood supply:Blood supply: Major circle of the irisMajor circle of the iris

Innervation:Innervation: Ciliary nervesCiliary nerves

CHOROIDCHOROID Very vascularVery vascular Between the retina Between the retina

and the scleraand the sclera The deeper the The deeper the

vessels, the larger vessels, the larger the caliberthe caliber

Drains via the vortex Drains via the vortex veinsveins

Bounded externally Bounded externally by the scleraby the sclera

Bounded internally Bounded internally by Bruch’s membraneby Bruch’s membrane

Nourishes the Nourishes the OUTEROUTER portion of the retina portion of the retina(inner portion supplied by (inner portion supplied by central retinal central retinal arteryartery))

Layers:Layers: Haller’s – largest, outermostHaller’s – largest, outermost Sattler – middleSattler – middle Choriocapillaris – exclusively supplies the foveaChoriocapillaris – exclusively supplies the fovea

FUNCTION: provides nourishment

UVEAL TRACTUVEAL TRACT

Method of examination:Method of examination: ANTERIOR uveal diseaseANTERIOR uveal disease

Gross inspection (flashlight / loupe)Gross inspection (flashlight / loupe) SlitlampSlitlamp

POSTERIOR uveal diseasePOSTERIOR uveal disease Direct / indirect ophthalmoscopeDirect / indirect ophthalmoscope Slitlamp with special lensesSlitlamp with special lenses

UVEITISUVEITIS Inflammation of the uveal tract (1 or 3 Inflammation of the uveal tract (1 or 3

parts)parts) Usually affects people 20-50 y/oUsually affects people 20-50 y/o Usually unilateralUsually unilateral Various causes, some are idiopathicVarious causes, some are idiopathic CLASSIFICATION:CLASSIFICATION:

CLINICAL CLINICAL (anterior/ posterior/ diffuse)(anterior/ posterior/ diffuse) PATHOLOGICAL PATHOLOGICAL (granulomatous/ (granulomatous/

nongranulomatous)nongranulomatous)

Clinical Clinical ANTERIORANTERIOR IritisIritis IridocyclitisIridocyclitis

INTERMEDIATEINTERMEDIATE CyclitisCyclitis Pars planitisPars planitis

POSTERIORPOSTERIOR RetinitisRetinitis RetinochoroiditisRetinochoroiditis ChorioretinitisChorioretinitis

DIFFUSE DIFFUSE (Panuveitis)(Panuveitis)

ANTERIOR UVEITISANTERIOR UVEITIS Most commonMost common Usually Usually

unilateral and unilateral and acuteacute

CLASSIC TRIAD:CLASSIC TRIAD: Pain, Pain,

photophobia, photophobia, blurring of blurring of visionvision

ANTERIOR UVEITISANTERIOR UVEITIS

SIGNS:SIGNS: Circumcorneal rednessCircumcorneal redness MiosisMiosis Irregular pupils (Posterior synechiae)Irregular pupils (Posterior synechiae) Keratic precipitates (large, small, stellate)Keratic precipitates (large, small, stellate)

Usually located inferiorly (ARLT’S Usually located inferiorly (ARLT’S TRIANGLE)TRIANGLE)

Iris nodulesIris nodules HypopyonHypopyon

CILIARY CILIARY INJECTIONINJECTION

POSTERIOR POSTERIOR SYNECHIAESYNECHIAE

KERATIC KERATIC PRECIPITATESPRECIPITATES

Medium-size keratic precipitates

Mutton fat keratic precipitates

IRIS IRIS NODULESNODULES

Koeppe nodules

Busacca nodule

Hypopyon

INTERMEDIATE UVEITISINTERMEDIATE UVEITIS Second most Second most

commoncommon HALLMARK:HALLMARK:

Vitreous Vitreous inflammationinflammation

Usually bilateralUsually bilateral Affects those in Affects those in

their late teens or their late teens or early adult yearsearly adult years

Men > womenMen > women >50% idiopathic>50% idiopathic

INTERMEDIATE UVEITISINTERMEDIATE UVEITIS Symptoms:Symptoms:

Floaters and blurring of visionFloaters and blurring of vision Pain, photophobia, redness usually Pain, photophobia, redness usually

absent or minimalabsent or minimal Most striking finding: VITRITISMost striking finding: VITRITIS ““snowballs” / “snowbanking”snowballs” / “snowbanking” Most common complications:Most common complications:

Cystoid macular edema, retinal Cystoid macular edema, retinal vasculitisvasculitis

POSTERIOR UVEITISPOSTERIOR UVEITIS SYMPTOMS:SYMPTOMS:

FloatersFloaters ScotomasScotomas Decreased Decreased

visionvision Complication:Complication:

Retinal Retinal detachmentdetachment

POSTERIOR UVEITISPOSTERIOR UVEITIS Most common causes of retinitis Most common causes of retinitis

(immunocompetent patients) :(immunocompetent patients) : ToxoplasmosisToxoplasmosis SyphilisSyphilis Behcet’s diseaseBehcet’s disease

Most common causes of choroiditis Most common causes of choroiditis (immunocomptent patients)(immunocomptent patients) SarcoidosisSarcoidosis TuberculosisTuberculosis Vogt-Koyanagi-Harada SyndromeVogt-Koyanagi-Harada Syndrome

Behcet’s DiseaseBehcet’s Disease Idiopathic, recurrent, Idiopathic, recurrent,

multisystem diseasemultisystem disease Affects young menAffects young men Associated with HLA-Associated with HLA-

B51B51 Recurrent oral Recurrent oral

aphthous stomatitis, aphthous stomatitis, skin lesions, arthritis, skin lesions, arthritis, epididymitis, epididymitis, intestinal ulceration, intestinal ulceration, vascular problemsvascular problems

Vogt-Koyanagi-Harada Vogt-Koyanagi-Harada DiseaseDisease Involves the eyes, Involves the eyes,

auditory system, auditory system, meninges and skinmeninges and skin

Female to male ratio of Female to male ratio of 2:12:1

33rdrd to 5 to 5thth decade of life decade of life Immune reaction to Immune reaction to

uveal melanin-associated uveal melanin-associated protein, melanocytes or protein, melanocytes or pigment epitheliumpigment epithelium

Strongly associated with Strongly associated with HLA-DR4HLA-DR4

PathologicalPathologicalNONGRANULOMATONONGRANULOMATO

USUSGRANULOMATOUSGRANULOMATOUS

OnsetOnset AcuteAcute InsidiousInsidious

PainPain MarkedMarked None or minimalNone or minimal

PhotophobiaPhotophobia MarkedMarked SlightSlight

Blurring of visionBlurring of vision ModerateModerate MarkedMarked

Circumcorneal flushCircumcorneal flush MarkedMarked SlightSlight

Keratic precipitatesKeratic precipitates Fine, whiteFine, white Large gray (“mutton Large gray (“mutton fat”)fat”)

PupilPupil Small, irregularSmall, irregular Small, irregularSmall, irregular

Posterior synechiaPosterior synechia SometimesSometimes SometimesSometimes

Iris nodulesIris nodules NoneNone SometimesSometimes

Site Site AnteriorAnterior Anterior, posterior, Anterior, posterior, diffusediffuse

CourseCourse AcuteAcute ChronicChronic

RecurrenceRecurrence CommonCommon SometimesSometimesGeneral Ophthalmology, 15th edition, Vaughan, et al

PathologicalPathological NONGRANULOMATOUSNONGRANULOMATOUS

Juvenile Rheumatoid ArthritisJuvenile Rheumatoid Arthritis Ankylosing SpondylitisAnkylosing Spondylitis

GRANULOMATOUSGRANULOMATOUS TuberculosisTuberculosis SyphilisSyphilis LeprosyLeprosy VKHVKH SarcoidosisSarcoidosis

UVEITISUVEITIS Predisposing factors:Predisposing factors:

Viral illnessViral illness Mental depressionMental depression MalnutritionMalnutrition Sudden changes in temperatureSudden changes in temperature Breakdown in immune systemBreakdown in immune system

Differential DiagnosisDifferential Diagnosis

ConjunctivitisConjunctivitis Acute GlaucomaAcute Glaucoma EndophthalmitisEndophthalmitis

Very important to know the differences!

Important to know!Important to know!

UVEITISUVEITIS ENDOPHTHALENDOPHTHALMITISMITIS

Non-purulentNon-purulent PurulentPurulent

Ciliary injectionCiliary injection Diffuse Diffuse hyperemiahyperemia

No swellingNo swelling ChemosisChemosis

Hypopyon RAREHypopyon RARE Hypopyon Hypopyon COMMONCOMMON

Posterior Posterior synechiasynechia

(hallmark)(hallmark)

Posterior Posterior synechiasynechia

(rare)(rare)

UVEITISUVEITIS

Common complications:Common complications: GlaucomaGlaucoma CataractCataract Band keratopathyBand keratopathy Cystoid macular edemaCystoid macular edema Retinal detachmentRetinal detachment Vitreous opacitiesVitreous opacities Occlusio pupillaeOcclusio pupillae

ManagementManagement Laboratory testing not required in the ff:Laboratory testing not required in the ff:

Mild uveitisMild uveitis 11stst episode episode

If with recurrent, severe, bilateral, granulomatous, If with recurrent, severe, bilateral, granulomatous, intermediate , posterior, diffuse uveitis or if fails to intermediate , posterior, diffuse uveitis or if fails to respond to standard therapy --- respond to standard therapy --- INVESTIGATE!INVESTIGATE!

MAINSTAYSMAINSTAYS of therapy: of therapy: Corticosteroids (oral/topical)Corticosteroids (oral/topical)

To control inflammationTo control inflammation CycloplegicsCycloplegics

To prevent synechia formationTo prevent synechia formation To reduce pain secondary to ciliary spasmTo reduce pain secondary to ciliary spasm

Chemotherapeutic agentsChemotherapeutic agents

Goals of Treatment:* Control inflammation* Prevent complications

SCLERASCLERA Fibrous outer Fibrous outer

protective coating protective coating of the eyeof the eye

CollagenousCollagenous Covered anteriorly Covered anteriorly

by by episcleraepisclera (fine (fine elastic tissue with elastic tissue with numerous blood numerous blood vessels) vessels)

Thinnest at the Thinnest at the insertion sites of insertion sites of rectus musclesrectus muscles

SCLERASCLERA 3 Vascular Layers:3 Vascular Layers:

Conjunctival vesselsConjunctival vessels Vessels within Tenon’s Vessels within Tenon’s

capsule capsule Maximal congestion in Maximal congestion in

episcleritisepiscleritis Blanches with topical Blanches with topical

phenylephrinephenylephrine Deep vascular plexusDeep vascular plexus

Maximal congestion in Maximal congestion in scleritisscleritis

Phenylephrine has NO Phenylephrine has NO EFFECT on these EFFECT on these vesselsvessels

EPISCLERITISEPISCLERITIS

Common, benign, self-limitingCommon, benign, self-limiting Affects young adultsAffects young adults Unilateral redness with mild Unilateral redness with mild

discomfort, tenderness and wateringdiscomfort, tenderness and watering 2 types:2 types:

SIMPLE EPISCLERITISSIMPLE EPISCLERITIS NODULAR EPISCLERITISNODULAR EPISCLERITIS

SIMPLE EPISCLERITISSIMPLE EPISCLERITIS

Commonest typeCommonest type Usually sectoral Usually sectoral

but may be but may be diffuse diffuse

Usually resolves Usually resolves spontaneously spontaneously within 1-2 weekswithin 1-2 weeks

Simple Sectoral Episcleritis

Simple Diffuse Episcleritis

NODULAR EPISCLERITISNODULAR EPISCLERITIS Localized, raised, Localized, raised,

congested nodulecongested nodule Longer time to Longer time to

resolveresolve With recurrent With recurrent

attacks, sclera may attacks, sclera may appear more appear more translucent (should translucent (should not be mistaken for not be mistaken for scleral thinning)scleral thinning)

Scleral translucency

EPISCLERITISEPISCLERITIS

Management:Management: Not requiredNot required Simple lubricants / Simple lubricants /

vasoconstrictorsvasoconstrictors Topical steroidsTopical steroids Oral NSAIDS for severe recurrent Oral NSAIDS for severe recurrent

or prolonged inflammationor prolonged inflammation

SCLERITISSCLERITIS Edema and cellular infiltration of the Edema and cellular infiltration of the

entire thickness of the scleraentire thickness of the sclera Systemic associations present in about Systemic associations present in about

50% of patients (Rheumatoid Arthritis is 50% of patients (Rheumatoid Arthritis is the most common)the most common)

May be surgically inducedMay be surgically induced May be infectious caused by spread from May be infectious caused by spread from

a corneal ulcera corneal ulcer•Deeper lesionDeeper lesion•Violaceous vesselsViolaceous vessels•Unilateral or bilateralUnilateral or bilateral•Hallmark: EYE PAINHallmark: EYE PAIN•Associated with connective tissue Associated with connective tissue vascular diseasevascular disease

SCLERITISSCLERITIS

Anatomical Classification (based on Anatomical Classification (based on the primary anatomical site)the primary anatomical site) ANTERIOR SCLERITIS (98%)ANTERIOR SCLERITIS (98%)

NON-NECROTIZING (85%)NON-NECROTIZING (85%) NECROTIZING (13%)NECROTIZING (13%)

POSTERIOR SCLERITIS (2%)POSTERIOR SCLERITIS (2%)

ANTERIOR NON-NECROTIZING ANTERIOR NON-NECROTIZING SCLERITISSCLERITIS

Presentation is similar to episcleritis but Presentation is similar to episcleritis but discomfort may be more severediscomfort may be more severe

Signs:Signs: DIFFUSE SCLERITIS DIFFUSE SCLERITIS

Widespread inflammationWidespread inflammation Distortion of the normal radial vascular Distortion of the normal radial vascular patternpattern

NODULAR SCLERITISNODULAR SCLERITISResemble nodular episcleritisResemble nodular episcleritis25% visual impairment25% visual impairment

DIFFUSE NON-DIFFUSE NON-NECROTIZING ANTERIOR NECROTIZING ANTERIOR

SCLERITISSCLERITIS

NODULAR NON-NODULAR NON-NECROTIZING ANTERIOR NECROTIZING ANTERIOR

SCLERITISSCLERITIS

ANTERIOR NON-NECROTIZING ANTERIOR NON-NECROTIZING SCLERITISSCLERITIS

Management:Management: Oral NSAIDs (initial treatment)Oral NSAIDs (initial treatment) Oral prednisolone (40-80 mg/day) Oral prednisolone (40-80 mg/day)

For patients resistant to NSAIDsFor patients resistant to NSAIDs NSAID + SteroidsNSAID + Steroids Subconjunctival steroid injection Subconjunctival steroid injection

with triamcinolone acetonide (40 with triamcinolone acetonide (40 mg/mL)mg/mL)

ANTERIOR NECROTIZING ANTERIOR NECROTIZING SCLERITIS WITH INFLAMMATIONSCLERITIS WITH INFLAMMATION

Most severe and Most severe and distressing form of distressing form of scleritisscleritis

Bilateral in 60% of casesBilateral in 60% of cases Most have systemic Most have systemic

disease w/ mortality rate disease w/ mortality rate of 25% within 5 years of of 25% within 5 years of onsetonset

Pain, redness, responds Pain, redness, responds poorly to analgesiapoorly to analgesia

Complications:Complications: Staphyloma formationStaphyloma formation Anterior UveitisAnterior Uveitis

Management:Management: Oral prednisolone (60-120 mg/day x 2-3 Oral prednisolone (60-120 mg/day x 2-3

days)days) Immunosuppressive agents Immunosuppressive agents

Cyclophosphamide, azathioprine, Cyclophosphamide, azathioprine, cyclosporincyclosporin

For steroid-resistant patientsFor steroid-resistant patients Combined therapy Combined therapy

Pulsed intravenous Pulsed intravenous methylprednisolone 500-1000 mg and methylprednisolone 500-1000 mg and cyclophosphamide 500 mgcyclophosphamide 500 mg

ANTERIOR NECROTIZING ANTERIOR NECROTIZING SCLERITIS WITH INFLAMMATIONSCLERITIS WITH INFLAMMATION

ANTERIOR NECROTIZING ANTERIOR NECROTIZING SCLERITIS WITHOUT SCLERITIS WITHOUT

INFLAMMATIONINFLAMMATION Also known as Also known as scleromalacia scleromalacia perforansperforans

Typically occurs in Typically occurs in women w/ long women w/ long standing rheumatoid standing rheumatoid arthritisarthritis

Usually bilateralUsually bilateral Progressive exposure Progressive exposure

of uvea due to scleral of uvea due to scleral thinningthinning

TREATMENT IS TREATMENT IS INEFFECTIVE !!!INEFFECTIVE !!!

POSTERIOR SCLERITISPOSTERIOR SCLERITIS UncommonUncommon Often confused with other inflammatory and Often confused with other inflammatory and

neoplastic conditionsneoplastic conditions 2/3 of cases are unilateral2/3 of cases are unilateral Most common symptoms are pain and visual Most common symptoms are pain and visual

impairmentimpairment Fundus findings: disc swelling, macular edema, Fundus findings: disc swelling, macular edema,

choroidal folds, exudative retinal detachment, choroidal folds, exudative retinal detachment, choroidal detachmentschoroidal detachments Management:Management: Elderly patients w/ associated systemic disease:Elderly patients w/ associated systemic disease:

Treat as anterior necrotizing scleritisTreat as anterior necrotizing scleritis Young patients without associated systemic Young patients without associated systemic

disease:disease: Non-steroidal anti-inflammatory drugsNon-steroidal anti-inflammatory drugs