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Posterior Uveitis


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Posterior Uveitis approxs about 10-30% of all uveitis cases. It presents in varied clinical entities. The pt should be throughly examined and investigated. Hope this ppt will serve some help in doing so..!

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Posterior Uveitis

  1. 1. POSTERIOR UVEITIS Dr Vijay Joshi GMC Haldwani
  2. 2. POSTERIOR UVEITIS Inflammation of retina or choroid posterior to vitreous base.  Choroiditis  Chorioretinitis  Retinochoroiditis  Retinal vasculitis  Neuroretinitis 
  3. 3. SUN working group classification of uveitis and the primary site of inflammation Type Primary site of inflammation Includes Anterior uveitis Anterior chamber Iritis, iridocyclitis, anterior cyclitis Intermediate uveitis Vitreous Pars planitis, posterior cyclitis, hyalitis Posterior uveitis Retina/choroid Focal, multifocal, diffuse choroiditis, chorioretinitis, retinochoroiditis, retinitis, neuroretinitis Panuveitis Anterior chamber, vitreous and retina or choroid
  4. 4. CAUSES..!
  5. 5.  Non-infective causes Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) Multiple evanescent white dot syndrome (MEWDS) Geographic helicoid peripapillary choroidopathy (GHPC) Multifocal choroiditis (MFC) Punctate inner choroidopathy (PIC) Birdshot choroidopathy Presumed ocular histoplasmosis syndrome (POHS) Subretinal fibrosis and uveitis syndrome (SFU) Diffuse unilateral subacute neuroretinitis (DUSN) Retinal pigment epithelitis (Krill's disease) Sarcoidosis
  6. 6. EPIDEMIOLOGY  Global variation and pattern changes in epidemiology of uveitis SR Rathinam, P Namperumalsamy Aravind Eye Hospital and PG Institute of Ophthalmology, 1, Anna Nagar, Madurai - 625 020, India
  7. 7.  In paediatric uveitis, anterior uveitis accounts for 30-40%, posterior uveitis 40-50%, intermediate uveitis 10-20% and panuveitis 5-10%  Cunningham ET Jr. Uveitis in Children. Ocular Immunology and Inflammation 2000;8:251-61.
  9. 9.    Pattern of uveitis in a referral uveitis clinic in India Debashis Das, Jyotirmay Biswas, Sudha K Ganesh A standard clinical protocol was followed for each case. Of the 465 new uveitic cases, anterior uveitis was most commonly encountered (170 cases, 36.5%), followed by posterior uveitis (132 cases, 28.4%), intermediate uveitis (92 cases, 19.8%) and panuveitis (71 cases, 15.3%).
  10. 10. FINDING THE TRUTH..! Critical questions for diagnosis: 1. Is it posterior uveitis only or is it part of a panuveitis? 2. Is it choroiditis, retinitis, or retinochoroiditis? 3. Is there associated involvement of the optic nerve head and/ or the retinal vessels? 4. Does the clinical feature fit into any known infective or noninfective entity? 5. Is there associated anterior segment inflammation, vitritis, or complications? 6. Is it associated with other systemic features? 7. Is it recurrent? If so, how has it responded to previous therapy? 8. Is it associated with an immunocompromised state? 9. Is it a masquerade syndrome? 
  11. 11. GET IT FROM HISTORY..!!
  13. 13. Evaluate Systemically..!
  14. 14. SLE Diagnostic criteria- malar rash serositis arthritis etc.  Choroidopathy: Fundus examination may reveal multiple serous retinal detachments, retinal pigment epithelium detachments and a central serous chorioretinopathy  Retinopathy: It typically occurs bilaterally but may be unilateral or asymmetric. The most common findings include cotton-wool spots and hemorrhages. Less common findings include hard exudates, retinal edema, visual acuity loss or metamorphopsia, and vascular tortuosity.  Ant uveitis- U/L or B/L  Specific tests- Anti nuclear Ab Assay 
  15. 15. HSV, VZV Immunosuppressed patients  Hutchinson’s sign  Reduced corneal sensations  Sectoral atrophy of iris  Peripheral retinal periarteritis  ARN- retinal necrosis in periphery which rapidly spreads.  PORN- rapidly progressive visual loss  Full thickness necrosis and early macular involvement.  PCR based assay. 
  16. 16.   Acute retinal necrosis (ARN): classical presentation of herpetic viruses. Triad of moderate to severe vitritis, arteritis, and periphlebitis and confluent peripheral retinal necrosis is diagnostic of ARN, which can present as a panuveitis.  Progressive outer retinal necrosis (PORN): Necrotizing retinitis  Confluent areas of outer retinal whitening with minimal vitritis involving the posterior pole and sparing of retinal vessels at the early stage, the typical “cracked mud appearance”is virtually diagnostic of the host.  PORN is frequently bilateral, occurring exclusively in immunocompromised state, such as in patients with HIV infection, and is associated with rapid development of rhegmatogenous retinal detachment or optic atrophy.
  17. 17. SARCOIDOSIS Ant seg- sarcoid granulomas, iris nodules  Post seg- vitritis, periphlebitis, snow balls  Diagnosis- x-ray chest hypercalcemia serum ACE Ga scan Biopsy 
  18. 18. Ocular sarcoidosis (A) Large iris nodules; (B) nodular involvement of the trabecular meshwork; (C) snowballs Periphlebitis in sarcoidosis. (A) Periphlebitis with involvement of the optic nerve head; (B) occlusive periphlebitis and disc oedema; (C) ‘candlewax’ drippings
  19. 19. Choroidal and retinal involvement in sarcoidosis. (A) Small peripheral choroidal granulomas; (B) confluent choroidal infiltrates; (C) multifocal choroiditis; (D) multiple small retinal granulomas
  20. 20. TOXOPLASMOSIS Congenital toxoplasmosis  Acquired toxoplasmosis  Toxoplasmosis in the immunocompromised host  Ocular toxoplasmosis 
  22. 22.  The classic clinical triad of retinochoroiditis, cerebral calcifications, and convulsions defines congenital toxoplasmosis. Congenital toxoplasmosis. (A) Hydrocephalus and right anophthalmos; (B) axial CT shows cerebral calcification; (C) macular scar
  23. 23. OCULAR TOXOPLASMOSIS Ocular Toxoplasmosis is the most common cause of Posterior uveitis, the lesions develop in deep retina, few to no vitreous cells may be present.  It frequently presents as a focal necrotizing retinitis, adjacent to a larger, atrophic chorioretinal scar,often located in macula in congenital cases. 
  24. 24. TOXOPLASMOSIS    The hallmark of the disease is a necrotizing retinochoroiditis, which may be primary or recurrent In primary ocular toxoplasmosis, a unilateral focus of necrotizing retinitis is present at the posterior pole in more than 50% of cases The area of necrosis usually involves the inner layers of the retina and is described as a whitish fluffy lesion surrounded by retinal edema
  25. 25. Active toxoplasma retinitis. (A) Typical ‘satellite’ lesion adjacent to an old scar; (B) two small foci; (C) severe vitreous haze and ‘headlight in the fog’ appearance Progression of toxoplasma retinitis. (A) Mild fluffy haze adjacent to an old scar at presentation; (B) after 2 weeks the area of retinitis is larger and denser; (C) after 7 weeks the retinitis has nearly resolved.
  26. 26. TOXOPLASMOSIS The retina is the primary site for the multiplying parasites, while the choroid and the sclera may be the sites of contiguous inflammation  When the optic nerve becomes involved by toxoplasmosis, the typical manifestation is optic neuritis or papillitis associated with edema, often called Jensen disease. 
  27. 27. TOXOPLASMOSIS  The sheath of the optic nerve may serve as a conduit for the direct spread of Toxoplasma organisms into the optic nerve from an adjacent cerebral infection. This also results in optic neuritis or papillitis
  28. 28. TOXOPLASMOSIS  Posterior vitreous detachment is commonly seen, and patients may develop precipitates of inflammatory cells on the posterior vitreous face, referred to as vitreous precipitates
  29. 29. TOXOPLASMOSIS  The Critical sign include white yellow retinal lesion with hazy Vitreous as a result of presence of Vitreous cells (Headlight in fog).  Old Choroiretinal scar can be seen often adjacent to new white yellow lesion but it is not always present
  30. 30. TOXOPLASMOSIS Toxoplasma antigens are responsible for a hypersensitivity reaction that may result in retinal vasculitis and granulomatous or nongranulomatous anterior uveitis.  Posterior synechiae may complicate the course of anterior uveitis, and keratic precipitates (KP) may be seen  KP may appear in the classic Arlt distribution in milder nongranulomatous configurations and granulomatous morphology. 
  31. 31. TOXOPLASMOSIS Some patients present with the stellate KP pattern, characterized by a diffuse homogeneous distribution pattern and a stellate fibrillar KP morphology.  As the lesion heals, it appears as a punched-out scar, revealing white underlying sclera. This results from extensive retinal and choroidal necrosis surrounded by variable pigment proliferation 
  32. 32. TOXOPLASMOSIS With reactivation of live tissue cysts located at the border of the scars (recurrent ocular toxoplasmosis), the areas of newly active necrotizing retinitis are usually adjacent to old scars (so-called satellite lesions)  In some patients, multiple grayish white dots at the level of the retinal pigment epithelium (RPE) appear. No associated vitreous reaction occurs with this manifestation  As in other inflammatory conditions, macular edema may be seen 
  33. 33. TOXOPLASMOSIS Rarely, ocular inflammation without the necrotizing retinochoroiditis can occur in patients with acquired toxoplasmosis.  These patients present with retinal vasculitis, vitreitis, and anterior uveitis. Later, they may develop retinochoroidal scars that suggest that the inflammatory reaction was secondary to T gondii. 
  34. 34. TOXOPLASMOSIS Rarely, retinal and optic nerve neovascularization may follow.  The neovascularization usually regresses with resolution of the inflammation  The exact etiology of neovascularization of the optic nerve and the retina is not well understood.  Retinal ischemia associated with severe retinal vasculitis may predispose to neovascularization of the retina. 
  35. 35. TOXOPLASMOSIS The areas of retinitis are the result of tissue cysts bursting and releasing bradyzoites that transform into tachyzoites, which in turn invade neighboring cells.  These destructive lesions are usually larger than 1 disc diameter and appear as soft, white, fluffy infiltrates surrounded by retinal edema with subjacent choroiditis 
  36. 36. TOXOPLASMOSIS When the tachyzoites come under increasing attack by the host’s immune response, they gradually transform back into bradyzoites. Inflammatory cells will be found in the vitreous overlying the active lesion. Perivascular inflammatory exudates are frequently present around retinal vessels peripheral to an area of active inflammation.
  37. 37. TOXOPLASMOSIS  Occasionally, patients presents initially with severe unilateral papillitis, macular hard exudates distributed in a star fashion, and vitreal inflammation.  Active toxoplasmosis simultaneously involving both the retina and the optic nerve is unusual.  Associated CME or CNVM is seen on rare occasion or as late sequel.
  38. 38. LABORATORY STUDIES  Serology  The diagnosis is usually based on the clinical appearance of the fundus lesion. Serologic evidence of exposure to Toxoplasma organisms serves as supportive evidence.  Serum antitoxoplasma antibody titers can be determined by several techniques, to include the following: Enzyme-linked immunosorbent assay (ELISA)  Indirect fluorescent antibody test  Indirect hemagglutination test  Complement fixation  Sabin-Feldman dye test 
  39. 39.      Serologic findings are important in determining whether acute or chronic systemic infection is present. Acute systemic toxoplasmosis has traditionally been diagnosed by seroconversion. Anti-Toxoplasma immunoglobulin G (IgG) titers present a 4-fold increase that peak 6-8 weeks following infection, then decline over the next 2 years, but remain detectable for life Anti-Toxoplasma IgM appears in the first week of the infection and then declines in the next few months. The presence of anti-Toxoplasma immunoglobulin A (IgA) has also been shown to be detectable in acute infection; however, since the titers can last for more than 1 year, its value in helping to diagnose an acute phase is limited.
  40. 40. Value of Serological Tests for Toxoplasmosis IgG:  * No previous infection: < 1:16  * Prevalent in general population: 1:16-1:256  * Suggests recent infection: > 1:256  * Suggests active infection: > 1:1024 IgM:  In children: any titer is significant  In adults: > 1:64 indicates active infection
  41. 41.  Invasive techniques are usually reserved for cases such as immunocompromised patients.  Ocular fluids can demonstrate the presence of intraocular antibody production.  Polymerase chain reaction can detect the causative organism.  A fluorescent treponemal antibody absorption (FTAABS) test should be obtained to rule out syphilis.
  42. 42. IMAGING STUDIES  Fluorescein angiography (FA) of active lesions shows hypofluorescence during the early phase of the study, followed by progressive hyperfluorescence secondary to leakage.  Indocyanine green (ICG) of active lesions are mostly hypofluorescent. ICG has imaged hypofluorescent satellite lesions that are not imaged by FA and are not seen during clinical examination. The etiology of such hypofluorescent lesions is unknown but suspected of being a noninfectious, perilesional inflammatory reaction. 
  43. 43. ULTRASOUND  It is indicated in the presence of ocular media opacities, especially vitreous opacities. The most common findings include intravitreal punctiform echoes, thickening of the posterior hyaloid, partial or total posterior vitreous detachment, and focal retinochoroidal thickening.
  44. 44. INDICATIONS FOR TREATMENT Lesions within the vascular arcade,  Lesions near the optic disc (Jensen papillitis),  Lesions in the papillomacular bundle  Large lesions irrespective of location are treated.  Patients with severe debilitating vitritis are also treated aggressively. 
  45. 45. In the case of ocular toxoplasmosis, several therapeutic regimens have been recommended.  Triple drug therapy refers to pyrimethamine, sulfadiazine, and prednisone.  Quadruple therapy refers to pyrimethamine, sulfadiazine, clindamycin, and prednisone. (Pyrimethamine should be combined with folinic acid to avoid hematological complications.)  The duration of treatment varies depending on the patient's response but usually lasts for 4-6 weeks. 
  46. 46. TRIMETHOPRIM AND SULFAMETHOXAZOLE  Exerts bacteriostatic action through competitive antagonism with   para-aminobenzoic acid (PABA). Double strength (DS) tab - 800 mg sulfamethoxazole & 160 mg trimethoprim. Regular strength tab- 400 mg sulfamethoxazole & 80 mg trimethoprim. Adult 1 DS tab bid for first 2 wk; then, 1 regular tab bid for next 3-4 wk  Pediatric <2 months: Not recommended >2 months: 8 mg/kg/d of trimethoprim plus 40 mg/kg/d of sulfamethoxazole divided bid  Pregnancy Safety for use during pregnancy has not been established. 
  47. 47. RX     Pyrimethamine (Daraprim) is administered as a loading dose of 50 mg followed by 25–50 mg daily for 4 weeks in combination with oral folinic acid 5 mg (mixed with orange juice) three times a week to prevent thrombocytopenia, leucopenia and folate deficiency. Weekly blood counts should be performed. In AIDS pyrimethamine is avoided because of possible pre-existing bone marrow suppression and the antagonistic effect of zidovudine when the drugs are combined. Sulfadiazine 1 g q.i.d. for 3–4 weeks is usually given in combination with pyrimethamine. Side-effects of sulphonamides include renal stones, allergic reactions and Stevens–Johnson syndrome. Other systemic options include clindamycin, spiramycin, tetracyclines, atovaquone, azithromycin and clarithromycin. Topical steroids may be given for anterior uveitis but periocular depot injections are contraindicated as they may lead to uncontrolled progression.
  48. 48. SURGICAL CARE  Photocoagulation or cryotherapy  Caution must be exercised if photocoagulation or cryotherapy is being considered in the treatment of intraocular toxoplasmosis.  Intraretinal hemorrhages, vitreous hemorrhage, and retinal detachment have been reported as complications of such treatment. Tissue cysts can exist in a normalappearing retina.  Pars plana vitrectomy may be indicated in cases of retinal detachment secondary to vitreous traction or in cases where vitreous opacities persist.
  49. 49. PREVENTION The principal mode of transmission worldwide is through the ingestion of oocysts shed into the environment in the feces of cats.  These organisms remain viable in the soil for at least a year and become a source of infection for mice, rats, birds, and other prey. 
  50. 50. PROGNOSIS In one series, 40% of patients had a final visual acuity of 20/100 or worse, and 16% of patients had a visual acuity between 20/40 and 20/80.  Toxoplasma retinitis frequently reactivates, and recurrence rates approach 50% at 3 years  Patients with recurrent disease are more likely to have a permanent visual disability. 
  51. 51. COMPLICATIONS           Choroidal neovascular membrane Branch retinal vein occlusion Branch retinal artery occlusion Tractional retinal detachment Cataract Glaucoma  Secondary glaucoma may occur with anterior uveitis that is secondary to the obstruction of the outflow channels by the inflammatory cells..  Destruction of the trabecula by chronic inflammation and anterior synechiae may also create a chronic pharmacologically nonresponsive glaucoma. Posterior synechiae Cystoid macular edema Retinal perivasculitis Optic atrophy
  52. 52. OCULAR TOXOCARIASIS 4 – 8 yr old  H/o contact with dogs/ geophagia  Unilateral  Post pole / peripheral granuloma  c/c endophthalmitis  RD, cyclitic membrane  ELISA, USG, CT scan  Chronic toxocara endophthalmitis may cause a cyclitic membrane and a white pupil. 
  53. 53. OCULAR TOXOCARIASIS Toxocara granuloma. (A) Juxtapapillary granuloma: (B) posterior pole granuloma associated with a localized tractional retinal detachment; (C) peripheral granuloma with a vitreous band extending to the disc
  54. 54. DIFFERENTIATING FEATURES  Marked vitreous inflammation, yellow grey strands extending into vitreous from chorioretinal lesions  CT- no calcification  Positive serological test is supportive but not diagnostic
  55. 55. EALE’S DISEASE  An idiopathic inflammatory venous occlusion that primarily affects the peripheral retina of adults.  Henry Eales, a British ophthalmologist [1880]  It is a diagnosis of exclusion, as many other retinal disorders can mimic Eales disease Retinal changes include perivascular phlebitis, peripheral non perfusion, and neovascularization.   Visual loss is characteristically caused by bilateral recurrent vitreous haemorrhage and its sequelae.
  56. 56. EALE’S DISEASE Rare in developed countries ,more commonly reported from the Indian subcontinent.  Healthy young adults  Male predominance  20 to 30 years  Associated with TB  Symptoms- floaters or decrease of visual acuity.  70 to 80% of patients develop bilateral involvement 
  57. 57. STAGES Charmis in 1965 classified Eales' disease into four stages:  Stage I : Mild periphlebitis of small peripheral retinal capillaries  Stage II : Perivasculitis of the venous capillary system is widespread.  Stage III: New vessel formation with vitreous haemorrhage  Stage IV : Retinitis proliferans and tractional retinal detachment. 
  58. 58. Eales disease. (A) Peripheral vascular sheathing and occlusion in the superotemporal quadrant; (B) peripheral neovascularization; (C) haemorrhage from new vessels
  59. 59. RETINAL PHLEBITIS  In the early stages of the disease - venous dilatation in the periphery with tortuosity and discontinuity of veins.  Vascular sheathing ranges from thin white lines limiting the blood column on both sides to heavy exudative sheathing.
  60. 60. RETINAL PHLEBITIS  Superficial flame-shaped haemorrhages are seen in the areas of sheathed vessels  Later the arteries also attenuate in the periphery.  The involved vessels become obliterated and an avascular area develops in the periphery better visualized by FFA
  61. 61. PERIPHERAL NONPERFUSION • • • Intraretinal haemorrhages , increase in vascular tortuosity , collateral formation around occluded vessels. Junction - sharply demarcated. The vascular abnormalities at the junction include microaneurysms, veno-venous shunts, venous beading and occasionally hard exudates and cotton-wool spots.
  62. 62. NEOVASCULARIZATION  NVE, is more common than NVD and is usually located at the junction between the perfused and nonperfused retina.  Bleeding from neovascularization is common, usually recurrent, and is one of the major causes of visual loss Signs of fibrous organization, retinitis proliferans or even tractional retinal detachment.  Some patients may develop uveitis, complicated cataract, rubeosis iridis, and secondary neovascular glaucoma in the late stage of the disease
  63. 63. FFA • Active vasculitis is characterized by staining of the vessel wall or even frank extravasation • Retinal oedema is characterized by increased permeability and diffuse extravasation of the dye. • The new vessels become distinctly outlined during the AV phase . These soon begin to leak the dye. Monitoring the regression and disappearance of new vessels during treatment and follow-up • •
  64. 64. NATURAL COURSE The diagnostic clinical picture of Eales' disease is the evidence of peripheral venous inflammation, past or present.  Inflammation of the vein causes variable degrees of venous insufficiency.  Retinal ischaemia stimulates neovascular growth from the surrounding normal vasculature  Occasionally with time, there is a tendency for the new vessels to regress. Get replaced by glial tissue.  The glial tissues on contraction cause several retinal complications.  The macula may get distorted or detached.  Rhegmatogenous retinal detachment. 
  65. 65. IMMUNOLOGICAL STUDIES  Type III (immune complex mediated) and/or type IV delayed hypersensitivity reaction to an infectious agent probably leads to retinal changes in Eales' disease.  Both antiretinal autoimmunity and circulating immune complexes may act as immunopathogenic factors
  66. 66. IMMUNOLOGICAL STUDIES  Antiendothelial cell antibodies  Predominant involvement of CD4 positiveT-cells  Indicate that a cell-mediated immune mechanism is involved
  67. 67. BIOCHEMICAL STUDIES  Raised alpha-globulins and reduced albumin levels in the serum  Raised serum alpha-1 acid glycoprotein (an important immunoregulatory protein formed by lymphocytes and epithelial cells)  PDGF, IGF-I, epidermal growth factor (EGF), TGF-a and TGF-(3), play a key role in the process of neovascularization
  68. 68. PATHOLOGY Epiretinal membrane (ERM) in Eales' disease, composed of several neovascular channels with glial cells, macrophages , fibrocytes, retinal pigment epithelial cells ,collagen material and basement membrane.  Significant lymphocytic infiltration. 
  69. 69. PATHOLOGY  Predominance of T-cells in the ERM and SRM indicate a cell-mediated immune reaction involved in the formation of these membranes.
  70. 70. TREATMENT  Corticosteroids, photocoagulation with or without anterior retinal cryoablation (ARC), and vitrectomy
  71. 71. CORTICOSTEROIDS  Active perivasculitis  High doses of oral steroids, e.g. prednisolone 2 mg/kg body wt, gradually tapering off  Periocular depot steroids (hydrocortisone or depomedrol) given as deep posterior sub-Tenon injection in active retinal vasculitis.
  72. 72. ANTITUBERCULAR TREATMENT  Given empirically  The ATT regimen usually includes two drugs (rifampicin 450 mg; isoniazid 300 mg once daily) for nine months.  Usually reserved for cases with acute phlebitis with massive infilteration, nodule formation, and complete obliteration of segments of the vein.
  73. 73. PHOTOCOAGULATION  Xenon arc photocoagulation in the area posterior to the vortex vein and cryotherapy anterior to the vortex vein.  Cryotherapy consisted of 4 to 5 ophthalmoscopically controlled transconjunctival applications in each quadrant involved , not more than two quadrants were treated in one cryositting
  74. 74. PHOTOCOAGULATION  Laser photocoagulation- currently, the most accepted modality of treatment in Eales' retinopathy  Beneficial in stage II and III of Eales' disease.  Focal treatment of flat retinal new vessels, sectoral scatter photocoagulation of areas of capillary dropouts and direct treatment of neovascular frond into the vitreous have been found beneficial in proliferative Eales' retinopathy
  75. 75. VITRECTOMY  Unresolving vitreous haemorrhage  Tractional retinal detachment involving the posterior pole  Multiple vitreous membranes with or without tractional retinal detachment  Combined tractional and rhegmatogenous retinal detachment.
  76. 76. BEHCET’S DISEASE Age- 25- 35 yrs  Diagnostic criteria Major- Recurrent oral aphthae skin lesions genital ulcers eye  Pathergy, HLA B51, ESR, CRP  Bilateral  NG ant uveitis of explosive nature  Mobile hypopyon  Obliterative , necrotizing vasculitis  Both arteries and veins in post pole 
  77. 77. Ocular Behçet syndrome. (A) Hypopyon in a white eye; (B) retinal infiltrates; (C) occlusive vasculitis; (D) end-stage disease
  78. 78. SYMP OPHTHALMIA B/L gran panuveitis  After surgery / penetrating trauma  Dalen Fuchs nodules – multiple, periphery  FFA- multiple hyper spots at RPE level  Nodules may show early blocked fluorescence  B scan – choroidal thickening 
  79. 79. VKH Vogt–Koyanagi–Harada (VKH) syndrome is an idiopathic multisystem autoimmune disease featuring inflammation of melanocyte-containing tissues such as the uvea, ear and meninges.  VKH can be subdivided into Vogt–Koyanagi disease, characterized mainly by skin changes and anterior uveitis, and Harada disease, in which neurological features and exudative retinal detachments predominate.  Possible trigger factors include cutaneous injury or a viral infection which may lead to sensitization of melanocytes 
  80. 80. VKH 1. Prodromal phase: lasting a few days is characterized by neurological and auditory manifestations. Meningitis causing headache and neck stiffness. Encephalopathy is less frequent and may manifest with convulsions, paresis and cranial nerve palsies.Auditory features include tinnitus, vertigo and deafness. 2. Acute uveitic phase: follows soon thereafter and is characterized by bilateral granulomatous anterior or multifocal posterior uveitis and exudative retinal detachments. 3. Convalescent phase: follows several weeks later and is characterized by: Localized alopecia, poliosis and vitiligo. Focal depigmented fundus lesions (sunset glow fundus) and depigmented limbal lesions (Sugiura sign). 4. Chronic-recurrent phase: is characterized by smouldering anterior uveitis with exacerbations
  81. 81. CRITERIA FOR VKH 1. Absence of a history of penetrating ocular trauma 2. Absence of other ocular disease entities 3. Bilateral uveitis 4. Neurological and auditory manifestations 5. Integumentary findings, not preceding onset of central nervous system or ocular disease, such as alopecia, poliosis and vitiligo In complete VKH, criteria 1–5 must be present. In incomplete VKH, criteria 1–3 and either 4 or 5 must be present. In probable VKH (isolated ocular disease), criteria 1–3 must be present.
  82. 82. DISEASE SPECTRUM  Posterior uveitis occurs in patients with Harada disease and is frequently bilateral. In chronological order the findings are as follows:  Diffuse choroidal infiltration and papillitis.    Multifocal detachments of the sensory retina and disc oedema. The chronic phase shows diffuse RPE atrophy (sunset glow fundus) which may be associated with small peripheral atrophic spots . Complications include CNV and subretinal fibrosis.  FA of the acute phase shows multifocal hyperfluorescent dots at the level of the RPE and then accumulation of dye in the subretinal space. The chronic phase shows areas of hyperfluorescence due to RPE window defects.  Treatment involves high-dose oral prednisolone (60–100 mg/day) that may be augmented with 3-day intravenous pulse therapy with methylprednisolone (500–1000 mg/day). Steroid-resistant patients may require ciclosporin.
  83. 83. Sunset glow fundus Active Harada disease. (A) Multifocal serous retinal detachments; (B) FA venous shows multiple hyperfluorescent spots; (C) late phase shows extensive areas of hyperfluorescence due to pooling of dye under the serous detachments
  84. 84. TB Ant uveitis- granulomatous, KPs iris nodules  h/o fever, sweats, weight loss, headache, localised back ache, abd pain.  Choroiditis is caused by direct infection.  Unilateral focal or (less frequently) multifocal.  Extensive diffuse choroiditis may occur in patients with AIDS .  Large solitary choroidal granulomas are uncommon.  Choroiditis may occasionally resemble serpiginous choroidopathy. 
  85. 85.      Periphlebitis is often bilateral and represents a manifestation of hypersensitivity to the bacillus. It may be mild and innocuous or occlusive resulting in severe retinal ischaemia and secondary retinal neovascularization. In TB granuloma, OCT reveals an area of localized adhesion between the choriocapillaris–RPE layer and overlying the neurosensory retina (“contact” sign), possibly due to inß ammatory adhesions overlying the granuloma that cause the neurosensory retina to stick to the RPE at that point. Treatment is initially with at least three drugs (isoniazid, rifampicin and pyrazinamide) and then with isoniazid and rifampicin. Quadruple therapy with the addition of ethambutol is necessary in pulmonary TB and in resistant cases. Concomitant systemic steroid therapy is also frequently necessary. The steroid dose needs to be adjusted when given with rifampicin.
  86. 86. Tuberculous choroiditis (A) Diffuse involvement in a patient with AIDS; (B) choroidal granuloma (A) Tuberculous choroiditis resembling serpiginous choroidopathy; (B) FA shows corresponding areas of hyper- and hypofluorescence
  87. 87. CMV AIDS  Indolent retinitis  Fulminant retinitis  Cytomegalovirus retinitis. (A) Indolent retinitis; (B) fulminating disease; (C) advanced disease involving the optic nerve head; (D) large posterior retinal tear with shallow localized detachment
  88. 88. PRESUMED OCULAR HISTOPLASMOSIS SYNDROME Histoplasmosis is caused by Histoplasma capsulatum acquired by inhalation of infective mycelia fragments and/or spores with dust particles.  The organisms then pass via the bloodstream to the spleen, liver and, on occasion, the choroid, setting up multiple foci of granulomatous inflammation. 
  89. 89. GENERAL SIGNS Absence of intraocular inflammation.  The acute stage may manifest with localized swelling of the choroid which may also lead to changes in the overlying RPE.  Atrophic ‘histo’ spots consist of roundish, slightly irregular, yellowish-white lesions about 200 µm in diameter, often associated with pigment clumps within or at the margins of the scars. The lesions are scattered in the mid-retinal periphery and posterior fundus.  Peripapillary atrophy may be diffuse or focal, or a combination of both.  Linear streaks in the midperiphery are found in 5% of cases. 
  90. 90. Presumed ocular histoplasmosis syndrome; (A) Peripheral ‘histo’ spots; (B) circumferential peripapillary atrophy and histo spots; (C) linear streaks
  91. 91. MASQUERADE NHL of the eye and CNS ( Intraocular CNS lymphoma)  50 to 60 yrs  Immunosuppression  B/L  Diagnosis- CNS evaluation  MRI  Lumbar puncture  Diagnostic vitrectomy 
  92. 92. IDIOPATHIC INFLAMMATORY WHITE DOT SYNDROMES 1. Multiple evanescent white dot syndrome (MEWDS) 2. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) 3. Punctate inner choroidopathy (PIC) 4. Birdshot retinochoroidopathy 5. Multifocal choroiditis with panuveitis 6. Serpiginous choroidopathy
  93. 93. • • • • • MEWDS Young adults (F > M) Unilateral Small, subtle, deep grey-white dots Orange macular granularity Mild vitritis Posterior pole Mid-periphery
  94. 94. FA of MEWDS Many hyperfluorescent spots at posterior pole Increase in hyperfluorescence and late leakage from disc
  95. 95. APMPPE • Young adults (F=M) • Associated with HLA-B7 and DR2 • Bilateral and symmetrical • Large, deep, grey-white, placoid lesions • Mainly at posterior pole • Mild vitritis
  96. 96. FA of APMPPE Early dense hypofluorescence Late staining
  97. 97. APMPEE • • • • Treatment - nil Course - 4 weeks Complications - nil Prognosis - good Residual RPE changes
  98. 98. Punctate Inner Choroidopathy ( PIC ) Young, myopic females Eventually bilateral but asymmetrical • • • • • Deep, small, indistinct yellow spots at posterior pole All same age No vitritis
  99. 99. FA of punctate inner choroidopathy Focal hyperfluorescent spots No increase in size
  100. 100. • • • • Punctate inner choroidopathy Treatment - not beneficial Course - several weeks Complications - occasional choroidal neovascularization (CNV ) Prognosis - guarded Residual dumb-bell shaped scars
  101. 101. FA of CNV in punctate inner choroidopa Early hyperfluorescence Progressive leakage
  102. 102. Birdshot retinochoroidopathy • • • • • Middle-aged (F > M) HLA - A29 Deep, oval, creamy, indistinct spots Radiate from disc towards equator Moderate vitritis
  103. 103. FA of birdshot retinochoroidopathy • Venous hyperfluorescence • Extensive late intraretinal and disc leakage
  104. 104. Birdshot retinochoroidopathy • • • • Treatment - steroids and immunosuppressive agent Course - chronic-remittent Complications - CMO Prognosis - guarded Residual punched-out, non-pigmented scars
  105. 105. Multifocal choroiditis with panuveitis • • • • • • Adults (20-50) ( F > M ) Bilateral but asymmetrical Deep,discrete, grey-yellow spots Mixed fresh and old Mid-periphery and fewer at posterior pole Moderate to severe panuveitis
  106. 106. Multifocal choroiditis with panuveitis • • • • Treatment - steroids Course - chronic-remittent Complications - CMO and occasional subretinal fibrosis Prognosis - guarded Residual pigmented scars
  107. 107. Serpiginous choroidopathy • • Middle age (F = M) Eventually bilateral but asymmetrical • • • Deep, grey-white lesions with hazy borders Initially peripapillary and at posterior pole then outward spread Mild vitritis
  108. 108. Serpiginous choroidopathy Treatment - steroids and immunosuppressive agents • Course - chronic-remittent • Complications - macular scarring • Prognosis - poor • Residual scalloped, punched-out areas
  109. 109. APPROACH DUSN:diffuse u/l subacute neuroretinitis
  110. 110. RECENT ADVANCES..! Auto fluorescence  Many substances fluorescence spontaneously (emit light of a particular wavelength) when illuminated by light of a different wavelength.  This fluorescence emission, arising from endogenous fluorophores, is an intrinsic property of cells and is called auto-fluorescence .  FAF : excitation 488nm,emission >500nm  Utilizes fluorescent properties of lipofuscin to study the health and viability of the retinal pigment epithelium/photoreceptor complex.
  111. 111. Studying Lipofuscin through FAF Lipofuscin (LF) is a mixture of autofluorescent pigments that accumulate in post-mitotic cells throughout life.  In the RPE, LF granules accumulate in the lysosomal compartment mainly as a by-product of constant phagocytosis of shed PR outer segments.  Accumulation of lipofuscin and its major flurophore A2E [formed out of residues of all-trans retinal ] can be visualized with FAF.  A2E can disable the function of RPE cell  Increase or decrease of FAF intensity is associated with different disease stages. 
  112. 112. AUTOFLUORESCENCE IN POSTERIOR UVEITIS      (APMPPE): In the acute phases of APMPPE, AF imaging shows more lesions as compared to FFA or ophthalmoscopy. This indicates that RPE damage occurs secondary to choroidal changes. During the follow up, many lesions developed increased pigmentation centrally, with a depigmented halo clinically. The central portions of the lesions appeared to have increased pigment and showed hyperautofluorescence. The depigmented halo appeared to show decreased, almost absent AF, suggesting atrophy or absence of functional RPE cells.
  113. 113. MEWDS): Acute phase of MEWDS, AF photography shows less numerous, but increased AF areas, corresponding to the focal hypocyanescent spots seen on indocyanine green angiography (ICG), probably due to the excitation of the photoreceptorretinal pigment epithelium complex. Following resolution of the lesions, AF and ICG return to a normal pattern.    Multifocal choroiditis and panuveitis (MCP): In MCP, AF shows numerous hypo autofluorescent spots corresponding to the clinically visible chorioretinal scars, but more in number. This indicates more extensive RPE damage compared to the clinically visible damage.