Uvea & sclera

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  • Translucent coz of rearrangement of the superficial scleral lamellae into more parallel rows
  • Uvea & sclera

    1. 1. UVEA & SCLERA JUAN S. LOPEZ, MD Chief, Section of Uveitis Institute of Ophthalmology and Visual Sciences St. Luke’s Medical Center
    2. 2. UVEAL TRACT <ul><li>Middle vascular layer of the eye </li></ul><ul><li>Protected by the cornea and sclera </li></ul><ul><li>Contributes blood </li></ul><ul><li>supply to the retina </li></ul><ul><li>Composed of: </li></ul><ul><ul><li>Choroid </li></ul></ul><ul><ul><li>Ciliary body </li></ul></ul><ul><ul><li>Iris </li></ul></ul>Iris Ciliary Body Choroid
    3. 3. IRIS <ul><li>Anterior extension of the ciliary body </li></ul><ul><li>PUPIL – central round aperture </li></ul><ul><li>Divides the anterior from the posterior chamber </li></ul><ul><li>LAYERS: </li></ul><ul><ul><li>STROMA – anterior portion with chromatophores containing melanin </li></ul></ul><ul><ul><ul><li>- Contains the sphincter and dilator muscles </li></ul></ul></ul><ul><ul><li>PIGMENTED EPITHELIUM – posterior portion </li></ul></ul><ul><ul><li>- anterior extension of the neuroretina and retinal </li></ul></ul><ul><ul><li>pigment epithelium </li></ul></ul>FUNCTION: regulates pupil size
    4. 4. CILIARY BODY <ul><li>From anterior end of the choroid to the root of the iris </li></ul><ul><li>Consist of: </li></ul><ul><ul><li>PARS PLICATA </li></ul></ul><ul><ul><ul><li>Corrugated, anterior </li></ul></ul></ul><ul><ul><ul><li>Where ciliary processes arise </li></ul></ul></ul><ul><ul><li>PARS PLANA </li></ul></ul><ul><ul><ul><li>Flattened, posterior </li></ul></ul></ul>
    5. 5. <ul><li>Composed of capillaries and veins that drain through the vortex veins </li></ul><ul><li>2 layers of ciliary epithelium: </li></ul><ul><ul><li>INTERNAL NONPIGMENTED LAYER </li></ul></ul><ul><ul><li>EXTERNAL PIGMENTED LAYER </li></ul></ul><ul><li>CILIARY MUSCLE </li></ul><ul><ul><li>Longitudinal – inserts into the trabecular meshwork </li></ul></ul><ul><ul><li>Circular contracts and relaxes the zonular fibers </li></ul></ul><ul><ul><li>Radial </li></ul></ul><ul><li>CILIARY PROCESSES responsible for the formation of aqueous </li></ul>FUNCTIONS: 1. Acts in accommodation 2. Secretes aqueous humor
    6. 6. IRIS AND CILIARY BODY <ul><li>Blood supply: </li></ul><ul><ul><li>Major circle of the iris </li></ul></ul><ul><li>Innervation: </li></ul><ul><ul><li>Ciliary nerves </li></ul></ul>
    7. 7. CHOROID <ul><li>Very vascular </li></ul><ul><li>Between the retina and the sclera </li></ul><ul><li>The deeper the vessels, the larger the caliber </li></ul><ul><li>Drains via the vortex veins </li></ul><ul><li>Bounded externally by the sclera </li></ul><ul><li>Bounded internally by Bruch’s membrane </li></ul>
    8. 8. <ul><li>Nourishes the OUTER portion of the retina </li></ul><ul><li>(inner portion supplied by central retinal artery ) </li></ul><ul><li>Layers: </li></ul><ul><ul><li>Haller’s – largest, outermost </li></ul></ul><ul><ul><li>Sattler – middle </li></ul></ul><ul><ul><li>Choriocapillaris – exclusively supplies the fovea </li></ul></ul>UVEAL TRACT FUNCTION: provides nourishment <ul><li>Method of examination: </li></ul><ul><ul><li>ANTERIOR uveal disease </li></ul></ul><ul><ul><ul><li>Gross inspection (flashlight / loupe) </li></ul></ul></ul><ul><ul><ul><li>Slitlamp </li></ul></ul></ul><ul><ul><li>POSTERIOR uveal disease </li></ul></ul><ul><ul><ul><li>Direct / indirect ophthalmoscope </li></ul></ul></ul><ul><ul><ul><li>Slitlamp with special lenses </li></ul></ul></ul>
    9. 9. UVEITIS <ul><li>Inflammation of the uveal tract (1 or 3 parts) </li></ul><ul><li>Usually affects people 20-50 y/o </li></ul><ul><li>Usually unilateral </li></ul><ul><li>Various causes, some are idiopathic </li></ul><ul><li>CLASSIFICATION: </li></ul><ul><ul><li>CLINICAL (anterior/ posterior/ diffuse) </li></ul></ul><ul><ul><li>PATHOLOGICAL (granulomatous/ nongranulomatous) </li></ul></ul>
    10. 10. Clinical <ul><li>ANTERIOR </li></ul><ul><ul><li>Iritis </li></ul></ul><ul><ul><li>Iridocyclitis </li></ul></ul><ul><li>INTERMEDIATE </li></ul><ul><ul><li>Cyclitis </li></ul></ul><ul><ul><li>Pars planitis </li></ul></ul><ul><li>POSTERIOR </li></ul><ul><ul><li>Retinitis </li></ul></ul><ul><ul><li>Retinochoroiditis </li></ul></ul><ul><ul><li>Chorioretinitis </li></ul></ul><ul><li>DIFFUSE (Panuveitis) </li></ul>
    11. 11. ANTERIOR UVEITIS <ul><li>Most common </li></ul><ul><li>Usually unilateral and acute </li></ul><ul><li>CLASSIC TRIAD: </li></ul><ul><ul><li>Pain, photophobia, blurring of vision </li></ul></ul>
    12. 12. ANTERIOR UVEITIS <ul><li>SIGNS: </li></ul><ul><ul><li>Circumcorneal redness </li></ul></ul><ul><ul><li>Miosis </li></ul></ul><ul><ul><li>Irregular pupils (Posterior synechiae) </li></ul></ul><ul><ul><li>Keratic precipitates (large, small, stellate) </li></ul></ul><ul><ul><ul><li>Usually located inferiorly (ARLT’S TRIANGLE) </li></ul></ul></ul><ul><ul><li>Iris nodules </li></ul></ul><ul><ul><li>Hypopyon </li></ul></ul>
    13. 13. CILIARY INJECTION POSTERIOR SYNECHIAE
    14. 14. KERATIC PRECIPITATES Medium-size keratic precipitates Mutton fat keratic precipitates
    15. 15. IRIS NODULES Koeppe nodules Busacca nodule Hypopyon
    16. 16. INTERMEDIATE UVEITIS <ul><li>Second most common </li></ul><ul><li>HALLMARK: </li></ul><ul><ul><li>Vitreous inflammation </li></ul></ul><ul><li>Usually bilateral </li></ul><ul><li>Affects those in their late teens or early adult years </li></ul><ul><li>Men > women </li></ul><ul><li>>50% idiopathic </li></ul>
    17. 17. INTERMEDIATE UVEITIS <ul><li>Symptoms: </li></ul><ul><ul><li>Floaters and blurring of vision </li></ul></ul><ul><ul><li>Pain, photophobia, redness usually absent or minimal </li></ul></ul><ul><li>Most striking finding: VITRITIS </li></ul><ul><li>“ snowballs” / “snowbanking” </li></ul><ul><li>Most common complications: </li></ul><ul><ul><li>Cystoid macular edema, retinal vasculitis </li></ul></ul>
    18. 18. POSTERIOR UVEITIS <ul><li>SYMPTOMS: </li></ul><ul><ul><li>Floaters </li></ul></ul><ul><ul><li>Scotomas </li></ul></ul><ul><ul><li>Decreased vision </li></ul></ul><ul><li>Complication: </li></ul><ul><ul><li>Retinal detachment </li></ul></ul>
    19. 19. POSTERIOR UVEITIS <ul><li>Most common causes of retinitis (immunocompetent patients) : </li></ul><ul><ul><li>Toxoplasmosis </li></ul></ul><ul><ul><li>Syphilis </li></ul></ul><ul><ul><li>Behcet’s disease </li></ul></ul><ul><li>Most common causes of choroiditis (immunocomptent patients) </li></ul><ul><ul><li>Sarcoidosis </li></ul></ul><ul><ul><li>Tuberculosis </li></ul></ul><ul><ul><li>Vogt-Koyanagi-Harada Syndrome </li></ul></ul>
    20. 20. Behcet’s Disease <ul><li>Idiopathic, recurrent, multisystem disease </li></ul><ul><li>Affects young men </li></ul><ul><li>Associated with HLA-B51 </li></ul><ul><li>Recurrent oral aphthous stomatitis, skin lesions, arthritis, epididymitis, intestinal ulceration, vascular problems </li></ul>
    21. 21. Vogt-Koyanagi-Harada Disease <ul><li>Involves the eyes, auditory system, meninges and skin </li></ul><ul><li>Female to male ratio of 2:1 </li></ul><ul><li>3 rd to 5 th decade of life </li></ul><ul><li>Immune reaction to uveal melanin-associated protein, melanocytes or pigment epithelium </li></ul><ul><li>Strongly associated with HLA-DR4 </li></ul>
    22. 22. Pathological General Ophthalmology, 15 th edition, Vaughan, et al Sometimes Common Recurrence Chronic Acute Course Anterior, posterior, diffuse Anterior Site Sometimes None Iris nodules Sometimes Sometimes Posterior synechia Small, irregular Small, irregular Pupil Large gray (“mutton fat”) Fine, white Keratic precipitates Slight Marked Circumcorneal flush Marked Moderate Blurring of vision Slight Marked Photophobia None or minimal Marked Pain Insidious Acute Onset GRANULOMATOUS NONGRANULOMATOUS
    23. 23. Pathological <ul><li>NONGRANULOMATOUS </li></ul><ul><ul><li>Juvenile Rheumatoid Arthritis </li></ul></ul><ul><ul><li>Ankylosing Spondylitis </li></ul></ul><ul><li>GRANULOMATOUS </li></ul><ul><ul><li>Tuberculosis </li></ul></ul><ul><ul><li>Syphilis </li></ul></ul><ul><ul><li>Leprosy </li></ul></ul><ul><ul><li>VKH </li></ul></ul><ul><ul><li>Sarcoidosis </li></ul></ul>
    24. 24. UVEITIS <ul><li>Predisposing factors: </li></ul><ul><ul><li>Viral illness </li></ul></ul><ul><ul><li>Mental depression </li></ul></ul><ul><ul><li>Malnutrition </li></ul></ul><ul><ul><li>Sudden changes in temperature </li></ul></ul><ul><ul><li>Breakdown in immune system </li></ul></ul>Differential Diagnosis <ul><li>Conjunctivitis </li></ul><ul><li>Acute Glaucoma </li></ul><ul><li>Endophthalmitis </li></ul>Very important to know the differences!
    25. 25. Important to know! Posterior synechia (rare) Posterior synechia (hallmark) Hypopyon COMMON Hypopyon RARE Chemosis No swelling Diffuse hyperemia Ciliary injection Purulent Non-purulent ENDOPHTHALMITIS UVEITIS
    26. 26. UVEITIS <ul><li>Common complications: </li></ul><ul><ul><li>Glaucoma </li></ul></ul><ul><ul><li>Cataract </li></ul></ul><ul><ul><li>Band keratopathy </li></ul></ul><ul><ul><li>Cystoid macular edema </li></ul></ul><ul><ul><li>Retinal detachment </li></ul></ul><ul><ul><li>Vitreous opacities </li></ul></ul><ul><ul><li>Occlusio pupillae </li></ul></ul>
    27. 27. Management <ul><li>Laboratory testing not required in the ff: </li></ul><ul><ul><li>Mild uveitis </li></ul></ul><ul><ul><li>1 st episode </li></ul></ul><ul><li>If with recurrent, severe, bilateral, granulomatous, intermediate , posterior, diffuse uveitis or if fails to respond to standard therapy --- INVESTIGATE! </li></ul><ul><li>MAINSTAYS of therapy: </li></ul><ul><ul><li>Corticosteroids (oral/topical) </li></ul></ul><ul><ul><ul><li>To control inflammation </li></ul></ul></ul><ul><ul><li>Cycloplegics </li></ul></ul><ul><ul><ul><li>To prevent synechia formation </li></ul></ul></ul><ul><ul><ul><li>To reduce pain secondary to ciliary spasm </li></ul></ul></ul><ul><ul><li>Chemotherapeutic agents </li></ul></ul>Goals of Treatment: * Control inflammation * Prevent complications
    28. 28. SCLERA <ul><li>Fibrous outer protective coating of the eye </li></ul><ul><li>Collagenous </li></ul><ul><li>Covered anteriorly by episclera (fine elastic tissue with numerous blood vessels) </li></ul><ul><li>Thinnest at the insertion sites of rectus muscles </li></ul>
    29. 29. SCLERA <ul><li>3 Vascular Layers: </li></ul><ul><ul><li>Conjunctival vessels </li></ul></ul><ul><ul><li>Vessels within Tenon’s capsule </li></ul></ul><ul><ul><ul><li>Maximal congestion in episcleritis </li></ul></ul></ul><ul><ul><ul><li>Blanches with topical phenylephrine </li></ul></ul></ul><ul><ul><li>Deep vascular plexus </li></ul></ul><ul><ul><ul><li>Maximal congestion in scleritis </li></ul></ul></ul><ul><ul><ul><li>Phenylephrine has NO EFFECT on these vessels </li></ul></ul></ul>
    30. 30. EPISCLERITIS <ul><li>Common, benign, self-limiting </li></ul><ul><li>Affects young adults </li></ul><ul><li>Unilateral redness with mild discomfort, tenderness and watering </li></ul><ul><li>2 types: </li></ul><ul><ul><li>SIMPLE EPISCLERITIS </li></ul></ul><ul><ul><li>NODULAR EPISCLERITIS </li></ul></ul>
    31. 31. SIMPLE EPISCLERITIS <ul><li>Commonest type </li></ul><ul><li>Usually sectoral but may be diffuse </li></ul><ul><li>Usually resolves spontaneously within 1-2 weeks </li></ul>Simple Sectoral Episcleritis Simple Diffuse Episcleritis
    32. 32. NODULAR EPISCLERITIS <ul><li>Localized, raised, congested nodule </li></ul><ul><li>Longer time to resolve </li></ul><ul><li>With recurrent attacks, sclera may appear more translucent (should not be mistaken for scleral thinning) </li></ul>Scleral translucency
    33. 33. EPISCLERITIS <ul><li>Management: </li></ul><ul><ul><li>Not required </li></ul></ul><ul><ul><li>Simple lubricants / vasoconstrictors </li></ul></ul><ul><ul><li>Topical steroids </li></ul></ul><ul><ul><li>Oral NSAIDS for severe recurrent or prolonged inflammation </li></ul></ul>
    34. 34. SCLERITIS <ul><li>Edema and cellular infiltration of the entire thickness of the sclera </li></ul><ul><li>Systemic associations present in about 50% of patients (Rheumatoid Arthritis is the most common) </li></ul><ul><li>May be surgically induced </li></ul><ul><li>May be infectious caused by spread from a corneal ulcer </li></ul><ul><ul><li>Deeper lesion </li></ul></ul><ul><ul><li>Violaceous vessels </li></ul></ul><ul><ul><li>Unilateral or bilateral </li></ul></ul><ul><ul><li>Hallmark: EYE PAIN </li></ul></ul><ul><ul><li>Associated with connective tissue vascular disease </li></ul></ul>
    35. 35. SCLERITIS <ul><li>Anatomical Classification (based on the primary anatomical site) </li></ul><ul><ul><li>ANTERIOR SCLERITIS (98%) </li></ul></ul><ul><ul><ul><li>NON-NECROTIZING (85%) </li></ul></ul></ul><ul><ul><ul><li>NECROTIZING (13%) </li></ul></ul></ul><ul><ul><li>POSTERIOR SCLERITIS (2%) </li></ul></ul>
    36. 36. ANTERIOR NON-NECROTIZING SCLERITIS <ul><li>Presentation is similar to episcleritis but discomfort may be more severe </li></ul><ul><li>Signs: </li></ul><ul><ul><li>DIFFUSE SCLERITIS </li></ul></ul><ul><ul><ul><li>Widespread inflammation </li></ul></ul></ul><ul><ul><ul><li>Distortion of the normal radial vascular pattern </li></ul></ul></ul><ul><ul><li>NODULAR SCLERITIS </li></ul></ul><ul><ul><ul><li>Resemble nodular episcleritis </li></ul></ul></ul><ul><ul><ul><li>25% visual impairment </li></ul></ul></ul>
    37. 37. <ul><li>DIFFUSE NON-NECROTIZING ANTERIOR SCLERITIS </li></ul><ul><li>NODULAR NON-NECROTIZING ANTERIOR SCLERITIS </li></ul>
    38. 38. ANTERIOR NON-NECROTIZING SCLERITIS <ul><li>Management: </li></ul><ul><ul><li>Oral NSAIDs (initial treatment) </li></ul></ul><ul><ul><li>Oral prednisolone (40-80 mg/day) </li></ul></ul><ul><ul><ul><li>For patients resistant to NSAIDs </li></ul></ul></ul><ul><ul><li>NSAID + Steroids </li></ul></ul><ul><ul><li>Subconjunctival steroid injection with triamcinolone acetonide (40 mg/mL) </li></ul></ul>
    39. 39. ANTERIOR NECROTIZING SCLERITIS WITH INFLAMMATION <ul><li>Most severe and distressing form of scleritis </li></ul><ul><li>Bilateral in 60% of cases </li></ul><ul><li>Most have systemic disease w/ mortality rate of 25% within 5 years of onset </li></ul><ul><li>Pain, redness, responds poorly to analgesia </li></ul><ul><li>Complications: </li></ul><ul><ul><li>Staphyloma formation </li></ul></ul><ul><ul><li>Anterior Uveitis </li></ul></ul>
    40. 40. <ul><li>Management: </li></ul><ul><ul><li>Oral prednisolone (60-120 mg/day x 2-3 days) </li></ul></ul><ul><ul><li>Immunosuppressive agents </li></ul></ul><ul><ul><ul><li>Cyclophosphamide, azathioprine, cyclosporin </li></ul></ul></ul><ul><ul><ul><li>For steroid-resistant patients </li></ul></ul></ul><ul><ul><li>Combined therapy </li></ul></ul><ul><ul><ul><li>Pulsed intravenous methylprednisolone 500-1000 mg and cyclophosphamide 500 mg </li></ul></ul></ul>ANTERIOR NECROTIZING SCLERITIS WITH INFLAMMATION
    41. 41. ANTERIOR NECROTIZING SCLERITIS WITHOUT INFLAMMATION <ul><li>Also known as scleromalacia perforans </li></ul><ul><li>Typically occurs in women w/ long standing rheumatoid arthritis </li></ul><ul><li>Usually bilateral </li></ul><ul><li>Progressive exposure of uvea due to scleral thinning </li></ul><ul><li>TREATMENT IS INEFFECTIVE !!! </li></ul>
    42. 42. POSTERIOR SCLERITIS <ul><li>Uncommon </li></ul><ul><li>Often confused with other inflammatory and neoplastic conditions </li></ul><ul><li>2/3 of cases are unilateral </li></ul><ul><li>Most common symptoms are pain and visual impairment </li></ul><ul><li>Fundus findings: disc swelling, macular edema, choroidal folds, exudative retinal detachment, choroidal detachments </li></ul><ul><li>Management: </li></ul><ul><ul><li>Elderly patients w/ associated systemic disease: </li></ul></ul><ul><ul><ul><li>Treat as anterior necrotizing scleritis </li></ul></ul></ul><ul><ul><li>Young patients without associated systemic disease: </li></ul></ul><ul><ul><ul><li>Non-steroidal anti-inflammatory drugs </li></ul></ul></ul>

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