Uvea sclera 990829

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Uvea sclera 990829

  1. 1. 三軍總醫院 蔡明霖醫師 M.D. Ph.D. Uveitis and Sclera
  2. 2. Anatomy of eyeball Uvea: vascular layer of the eye ( Ocular branch of Ophthalmologic a.) iris, ciliary body, choroid. uvea sclera Eyeball: outer coat (cornea, sclera), middle vascular layer (uveal tract) inner sensory layer ( retina)
  3. 3. Origin of uveal vessel Ocular branch 1. Central retinal artery 2. Anterior ciliary artery 3. long posterior ciliary artery 4. short posterior ciliary artery 5. Muscle Br Internal carotid a. =>Ophthalmologic a. =>Ocular branch 4 3 5 2
  4. 4. Iris anterior part of uveal tract Iris 以 collarette 為界 ; 分成 pupilary and ciliary 兩部份
  5. 5. Function of iris <ul><li>pupil open </li></ul><ul><li>Iris dilator muscle </li></ul><ul><li>cervical sympathetic trunk </li></ul><ul><li>pupil close </li></ul><ul><li>Iris sphincter muscle </li></ul><ul><li>short ciliary nerve </li></ul><ul><li>(brainstem: E-W Nu ) </li></ul>
  6. 6. 散瞳劑 副交感神經阻斷劑 : Atropine(1%) :2 weeks 。 Scopolamine:1-3 days Homatropine:1-3 days cyclopentolate(0.5%):24 hours tropicamide (0.5%): 6 hours 。
  7. 7. Ciliary body <ul><li>middle part of uvea </li></ul>Function: aqueous production accommodation
  8. 8. Histology <ul><li>Stroma : ciliary muscle </li></ul><ul><li>(accommodation ) </li></ul><ul><li>circular muscle=>near </li></ul><ul><li>( lens thickening) </li></ul><ul><li>radial muscle=>far </li></ul><ul><li>(lens thinning) </li></ul>Epithelium: pigmented epithelium non-pigmented epithelium (aqueous formation)
  9. 9. Accommodation <ul><li>near </li></ul><ul><li>lens thickening </li></ul><ul><li>parasympathetic fiber </li></ul><ul><li>ciliary circular M. contraction </li></ul><ul><li>zonular relax </li></ul><ul><li>Far </li></ul><ul><li>lens thinning </li></ul><ul><li>sympathetic fiber </li></ul><ul><li>ciliary radial M. contraction </li></ul><ul><li>zonular contraction </li></ul>
  10. 10. 睫狀肌鬆弛劑 <ul><li>副交感神經阻斷劑 : </li></ul><ul><li>Atropine(1%) :2 weeks 。 </li></ul><ul><li>Scopolamine:1-3 days </li></ul><ul><li>Homatropine:1-3 days cyclopentolate(0.5%):24 hours </li></ul><ul><li>tropicamide (0.5%):6 hours 。 </li></ul>
  11. 11. Choroidal function <ul><li>Retina : blood supply </li></ul><ul><li>(Inner 1/3 retina vessel; outer 2/3 choroid) </li></ul><ul><li>light-absorbing layer </li></ul>
  12. 12. Uvea and scleral disease <ul><li>Uveitis </li></ul><ul><li>Uveal tumor </li></ul><ul><li>Scleral diseased </li></ul>
  13. 13. I Disease of Uvea Tract <ul><li>Uvea inflammation: </li></ul><ul><li>Incidence 1/1000 case </li></ul><ul><li>Etiology: infection, neoplasm, </li></ul><ul><li>immunology, unknown </li></ul><ul><li>anterior uveitis 75%; post uveitis 25% </li></ul><ul><li>Classification: </li></ul><ul><li>location, duration, inflammation </li></ul>
  14. 14. Uveitis: Classification <ul><li>Location: iritis or anterior uveitis, </li></ul><ul><li>cyclitis or intermediate uveitis </li></ul><ul><li>choroiditis or posterior uveitis. </li></ul><ul><li>Duration: acute-within 3 months </li></ul><ul><li>chronic- exceed 3 months </li></ul><ul><li>Inflammation type </li></ul><ul><li>: non-granulomatous </li></ul><ul><li>: granulomatous (nodule>1mm) </li></ul><ul><li>(Koppe`s nodule papillary margin </li></ul><ul><li>Busacca`s nodule: iris surface) </li></ul>
  15. 15. Uveitis: anterior segment sign hypopyron synethiae Flares,cells Keratic precepitation Hypopyon Synechiae KP
  16. 16. Uveitis: posterior segment sign hypopyron synethiae Intermediate uveitis: snowballs, string of pearls) Retina: Retinal vasculitis Choroid: Choroiditis, granuloma snowballs vasculitis Choroiditis granuloma
  17. 17. Synptom/Sign of uveitis Sign/Symptom Description Pathogenic Mechanisms Flare Milkiness of aqueous humor Protein is transudate from uveal vessels Cells Inflammatory and pigmented cells in AC White blood cells released from uveal vessels Fibrin (cyclytic membrane) Coagulation of exudates in AC (only seen in severe cases) Extreme inflammation with accumulation of fibrin  Iris nodules: Koeppe and Busacca Fluffy white nodular precipitates on inner surface of pupillary margin (Koeppe) or on surface of iris (Busacca) (seen in some systemic based diseases) Inflammatory cellular infiltration into iris stroma Keratic precipitates (KP's) Deposits of inflammatory cells on endothelium. inferiorly; fine white (non-granulomatous) to giant waxy mutton fat (granulomatous); may be pigmented Inflammatory cells from iris and uveal vessels which stick to corneal endothelium Hypopyon Purulent exudate in lower AC (seen in severe cases) Purulent Exudation from inflamed uveal vessels
  18. 18. Grading Cells and Flare Dense Haze: With Obvious Fibrin Collecting on Iris 4 Dense Cells in Beam, More Than You Can Count 4 Marked: Iris Details Becoming Hazy 3 Cells Scattered Through Out Beam 20 or More 3 Moderate: But Iris Detail Still Clear 2 5-10 Cell Seen at Once 2 Faint: Haze or Not Equal Bilaterally 1 2-5 Cells Seen in 45 Seconds or One Minute 1 Optically Empty Compared Bilaterally 0 None 0 Flare Grade Aqueous Cells Grade
  19. 19. Synptom/Sign of uveitis IOP variation Low IOP is characteristic; glaucoma may develop as complication Low IOP from decreased aqueous production; high IOP is seen if TM becomes clogged with inflammatory debris Synechiae Adhesions of the iris to the lens in the pupillary zone (posterior synechiae) or of iris to angle structures (peripheral anterior synechiae) -- these develop during acute phase and persist indefinitely if not broken heavy exudation of protein (posterior); shallowing of AC from pupillary block, organization of exudates in angle, or from swelling of iris root (PAS) Conjunctival and perilimbal injection Pink to violet circumlimbal episcleral vessels (ciliary flush) Inflammatory vasodilation of radial episcleral vessels Cataract Opacification of crystalline lens From nutritional deprivation of lens fibers; or as a toxic response to inflammatory cell breakdown; or from topical or systemic steroid use Small pupils Not always present; miosis relative to other pupil Vasodilation of iris vessels; prostaglandin release
  20. 20. Diagnosis of uveitis <ul><li>Find curable uveitis: </li></ul><ul><li>Trauma; Infection; Neoplasm </li></ul><ul><li>Find controllable disease </li></ul><ul><li>Systemic associated ocular inflammation: </li></ul><ul><li>rheumatoid arthritis; lupus </li></ul><ul><li>Specific ocular inflammation </li></ul>
  21. 21. Diagnosis of uveitis Find curable uveitis <ul><li>Trauma : Hx </li></ul><ul><li>Infection: </li></ul><ul><li>Bacteria (complete blood count; culture) </li></ul><ul><li>Fungus (complete blood count; culture, culture) </li></ul><ul><li>Parasite (complete blood count; culture, eosinophil) </li></ul><ul><li>Virus: PCR; Serology: VDRL, HIV, Torch marker </li></ul><ul><li>Neoplasm: </li></ul><ul><li>CBC, image study( PET) </li></ul>
  22. 22. Diagnosis of uveitis Find controllable disease <ul><li>Systemic associated ocular inflammation: </li></ul><ul><li>ankylosing spondylitis( image study, HLA-B27) </li></ul><ul><li>Rheumatoid arthritis ( image study; CRP) </li></ul><ul><li>lupus( CBC; anti-ds DNA) </li></ul><ul><li>sarcoidosis( CxR; biopsy) </li></ul><ul><li>Behcet disease (oral ulcer) </li></ul><ul><li>Specific ocular inflammation </li></ul><ul><li>Vogt-Koyanagi-Harada's Disease </li></ul><ul><li>Sympathetic ophthalmia </li></ul>
  23. 23. Uveitis treatment <ul><li>Mandatory : </li></ul><ul><li>1. Treatment of underlying disease, if known ( trauma, infection, n eoplasm) </li></ul><ul><li>2. Cycloplegic agent </li></ul><ul><li>3. topical steroid </li></ul><ul><li>Optional : </li></ul><ul><li>1. Periocular or systemic steroids: </li></ul><ul><li>unresponsive to topical steroid </li></ul><ul><li>2. Immunosuppressors, anti-TNF therapy </li></ul><ul><li>sight-threatening inflammation </li></ul><ul><li>which has been unresponsive to steroids </li></ul><ul><li>3. control elevated intraocular pressure </li></ul>
  24. 24. Specific ocular uveitis <ul><li>Anterior uveitis </li></ul><ul><ul><li>HLA-B27 associated uveitis(20-40%) </li></ul></ul><ul><ul><li>Sarcoidosis (15%) </li></ul></ul><ul><ul><li>Posner-Schlossman Syndrome (1-5%) </li></ul></ul><ul><li>Posterior uveitis </li></ul><ul><ul><li>Ocular toxoplasmosis(8-40%) </li></ul></ul><ul><ul><li>Behcet disease (5%) </li></ul></ul><ul><ul><li>Vogt-Koyanagi-Harada's Disease </li></ul></ul><ul><ul><li>Sympathetic ophthalmia </li></ul></ul>
  25. 25. HLA-B27 associated uveitis <ul><li>S/S: flares, cells, fibrin, seldom KP </li></ul><ul><li>Dx: 1. clinical picture </li></ul><ul><li>2. HLA-B27 positive. </li></ul><ul><li>3. associated systemic disease </li></ul>Treatment: 1. Cycloplegic agent 2. Steroid 3. NSAID 4. anti-TNF therapy
  26. 26. HLA-B27 associated system disease <ul><li>Treatment: control uveitis, consult: CV, RIA , REH </li></ul><ul><li>AS :systemic anti-inflammatory agent </li></ul><ul><li>CV consult(heart block, aortic insufficiency) </li></ul><ul><li>Reiter syndrome: systemic antibiotic </li></ul><ul><li>Psoriasis: analgesics, immunosuppressive </li></ul>AS (55-90%) Psoriasis 3-4% Reiter syndrome (8-21% )
  27. 27. sarcoisosis <ul><li>S/S </li></ul><ul><li>Hilar enlaragement </li></ul><ul><li>Granulomatous uveitis </li></ul><ul><li>Mutton fat KP </li></ul><ul><li>Dx : biopsy, non-caseous granuloma </li></ul><ul><li>Treatment </li></ul><ul><li>Cycloplegic agent </li></ul><ul><li>Steroid </li></ul><ul><li>immunosuppressor </li></ul>
  28. 28. Posner –Schlossman syndromn (Glaucomatocyclitic crisis) <ul><ul><li>S/S: KP, IOP elevation </li></ul></ul><ul><ul><li>Eti: prostaglandin induced trabeculitis </li></ul></ul><ul><ul><li>Dx: clinical criteria </li></ul></ul><ul><ul><li>1. Fine KP 2. trabeculitis </li></ul></ul><ul><ul><li>3. IOP elevation </li></ul></ul><ul><ul><li>TX: 1. Steroid </li></ul></ul><ul><ul><li>2 atropine </li></ul></ul><ul><ul><li>3 control IOP </li></ul></ul><ul><ul><li>4 avoid miotic </li></ul></ul><ul><ul><li>5 avoid prostagradin </li></ul></ul>
  29. 29. Ocular toxoplasmosis <ul><ul><li>S/S: central chorioretinal scar , vitritis </li></ul></ul><ul><ul><li>Eti: Toxoplasma gondii </li></ul></ul><ul><ul><li>raw meat (cyst); contaminated vegetable (oocyst) </li></ul></ul><ul><ul><li>Dx: serum anti-toxoplasma gondii antibody </li></ul></ul><ul><ul><li>PCR ; biopsy - trophozoite, cyst </li></ul></ul><ul><ul><li>Tx: 1. Sulfadiazine 2 clindamycin </li></ul></ul><ul><ul><li>3. Prednisolone 4. pyrimethamine (folic acid) </li></ul></ul>
  30. 30. Toxoplasmosis
  31. 31. Behcet’s disease <ul><ul><li>S/S: systemic vasculitis, young male, HLA B51 </li></ul></ul><ul><ul><li>Dx: clinical picture </li></ul></ul><ul><ul><li>1. Oral ulcer 2. Skin Lesions </li></ul></ul><ul><ul><li>3. Genital ulcer 4. Ocular inflammation </li></ul></ul><ul><ul><li>Eti: unknown. </li></ul></ul><ul><ul><li>infection-induced autoimmune process to vessel </li></ul></ul><ul><ul><li>endothelium is the most likely mechanism. </li></ul></ul><ul><ul><li>TX: 1. Steroid 2 immunosuppressor </li></ul></ul>
  32. 32. Diagnosis MAJOR CRITERIA MINOR CRITERIA Recurrent Oral Aphthous Ulcers (pain) Arthritis (heat, effuse, limitation) Skin Lesions: **Erythema nodosum-like lesions **Folliculitis Epididymitis Genital Ulcers( Gastrointestinal Involvement Ocular Disease : **Iridocyclitis with hypopyon **Posterior Uveitis with retinal vasculitis Vascular Involevement: **Thrombophlebitis Neurologic Symptoms Complete   Presence of all major criteria Incomplete 3 major criteria 2 major + 2 minor criteria ocular disease + 1 major criterion ocular disease + 2 minor criteria Suspect 2 major criteria Possible 1 major criterion
  33. 34. Vogt-Koyanagi-Harada's Disease (Uveomenigitic Syndrome) <ul><li>S/S: bilateral, painful visual loss associated with posterior or panuveitis. </li></ul><ul><li>pathogenesis: unknown. </li></ul><ul><li>An autoimmune process to melanocyte is the most likely mechanism . </li></ul><ul><li>S/S : </li></ul><ul><ul><li>Prodromal phase meningeal phase, patients have headache, fever, and meningitis. </li></ul></ul><ul><ul><li>Uveitic phase The uveitic phase, which lasts for several weeks </li></ul></ul><ul><ul><li>Convalescent phase This phase is characterized by dermatologic changes </li></ul></ul><ul><ul><li>Chronic phase The chronic phase consists of smoldering panuveitis with acute episodes of anterior uveitis. </li></ul></ul><ul><li>Treatment: steroid or immunosuppressor for 6 months </li></ul>
  34. 35. Vogt-Koyanagi-Harada's Disease <ul><li>Diagnostic criteria ( American Uveitis Society, 1978) </li></ul><ul><li>No history of ocular trauma or surgery </li></ul><ul><li>At least 3 out of the following 4 criteria: </li></ul>Bilateral chronic iridocyclitis Bilateral posterior uveitis <ul><li>Cutaneous findings: alopecia, </li></ul><ul><li>poliosis, or vitiligo (late) </li></ul><ul><li>Neurological signs: tinnitus, neck stiffness, cranial nerve or central nervous system dysfunction, or cerebrospinal fluid pleocytosis </li></ul>
  35. 36. Sympathetic ophthalmia <ul><li>S/S: injury in one eye , granulomatous inflammation developed in following eye </li></ul><ul><li>Pathogenesis: </li></ul><ul><li>granulomatous inflammation </li></ul><ul><li>to to melanocyte </li></ul><ul><li>Dx: Dallen-Fuch nodule </li></ul><ul><li>Treatment : repair would </li></ul><ul><li>steroid or immunosuppressor for 6 months </li></ul>
  36. 37. Uveal melanoma <ul><li>most common primary intraocular malignancy </li></ul><ul><li>How to DD from benign tumor: MSOFT </li></ul>1. Ill-defined margin 2. symptoms (Rapid growth) 3. orange pigmentation 4. fluid 5.thinkness>2mm
  37. 38. II Uveal tumor <ul><li>Uveal melanoma 最常見的原發性眼內惡性腫瘤 </li></ul><ul><li>眼內最常見的腫瘤為 metastatic uveal tumor </li></ul><ul><li>女性最常見的的轉移性腫瘤 , 來源為乳癌 </li></ul><ul><li>男性最常見的的轉移性腫瘤 , 來源為肺癌 </li></ul>
  38. 39. Uveal melanoma <ul><li>Pathology:spindle cell A, spindle cell B, mixed type </li></ul><ul><li>Treatment </li></ul><ul><li>Photocoagulation, brachytherapy, enucleation, cyberknife </li></ul>
  39. 40. III Sclera and scleral disease Sclera histology : two layer Episclera, Scleral stroma( 越後面越厚 ) <ul><li>Sclera: </li></ul><ul><li>white outer covering of the eye </li></ul><ul><li>Muscle insertion : </li></ul><ul><li>danger site of eyeball rupture </li></ul><ul><li>MR 5.5; IR 6.5; LR 7.0; SR 7.8mm </li></ul>
  40. 41. Episcleritis: episcleral tissue inflammation S/S: red eye episcleral vessel engorgement (between the conjunctiva and the sclera) Eti: allergy, unknown,… DD: epinephrine => bleach Tx: steroid
  41. 42. Scleritis Scleritis: an inflammation of the sclera stroma S/S: red eye Eti: unknown, 50% associated with immune disease such as : rheumatoid arthritis, Crohn’s disease, Wegener’s granulomatosis, metabolic disorders, infections and chemical or physical injuries. DD: epinephrine => scleral vessel bleach do not bleach Tx: 1. Steroid 2 immunosuppressor 3 scleral graft
  42. 43. Video for uveitis Biopsy
  43. 44. THANKS
  44. 45. Find systemic disease <ul><li>Diagnostic imaging </li></ul><ul><li>Chest x-ray (TB, sarcoidosis, histoplasmosis, tumor) </li></ul><ul><li>Sacroiliac films (HLA-B27, Reiter, ankylosing spondylitis) </li></ul><ul><li>Orbital image (tumor, foreign body, thyroid, scleritis) </li></ul><ul><li>Skull films (congenital toxoplasmosis) </li></ul><ul><li>Joint films (rheumatoid, HLA-B27, JRA, lupus, gonorrhea) </li></ul><ul><li>Gallium scan (sarcoidosis, infection, metastasis) </li></ul>
  45. 46. HLA B27
  46. 47. VKH
  47. 48. VKH <ul><li>minimum of 6 months, has been shown to improve the prognosis by reducing the length of disease, increasing the incidence of a convalescent phase, and decreasing the extraocular manifestations. </li></ul>
  48. 49. SO: 須找出傷口
  49. 54. Sarcoidosis <ul><li>The initial dose is 30 to 60 mg of prednisone daily for 8 to 12 weeks, followed by slow tapering over 6 or 12 weeks to establish the minimal effective dose.5,6 Pulsed intravenous methylprednisolone might be necessary in some cases. A maintenance dose of oral corticosteroid at 10 to 15 mg per day in alternate days should be given at least for 3 months. </li></ul>
  50. 55. HLA-B27 associated system disease <ul><li>Treatment: control uveitis, consult: CV, RIA , REH </li></ul><ul><li>AS :systemic anti-inflammatory agent </li></ul><ul><li>CV consult(heart block, aortic insufficiency) </li></ul><ul><li>Reiter syndrome: systemic antibiotic </li></ul><ul><li>Psoriasis: analgesics, immunosuppressive </li></ul>AS (55-90%) Psoriasis 3-4% Reiter syndrome (8-21% )
  51. 56. Find systemic disease <ul><li>Diagnostic imaging </li></ul><ul><li>Chest x-ray (TB, sarcoidosis, histoplasmosis, tumor) </li></ul><ul><li>Sacroiliac films (HLA-B27, Reiter, ankylosing spondylitis) </li></ul><ul><li>Orbital image (tumor, foreign body, thyroid, scleritis) </li></ul><ul><li>Skull films (congenital toxoplasmosis) </li></ul><ul><li>Joint films (rheumatoid, HLA-B27, JRA, lupus, gonorrhea) </li></ul><ul><li>Gallium scan (sarcoidosis, infection, metastasis) </li></ul>

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