uvea & sclera
TRANSCRIPT
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