Post Uveitis PDF

Traumatic Injury Fibrin Membrane Retinal Dialysis Presumed Ocular Histoplasmosis Syndrome (POHS)  Key Characteristics — Histoplasma capsulatum –fungus, inhalation of spores — Prior exposure to chickens or pigeons — Histo Belt: Ohio & Mississippi River valleys — Maculopat...

Traumatic Injury Fibrin Membrane Retinal Dialysis Presumed Ocular Histoplasmosis Syndrome (POHS)  Key Characteristics — Histoplasma capsulatum –fungus, inhalation of spores — Prior exposure to chickens or pigeons — Histo Belt: Ohio & Mississippi River valleys — Maculopathy - 6 x’s more frequent in whites than blacks — Macular involvement usually between 20-50 years of age (pts are asymptomatic unless maculopathy) — Classic triad- 2 of the 3 — Peripheral atrophic histo spots (may have linear streaks of C/R atrophy in periphery)- asymptomatic — Peripapillary atrophy — Maculopathy Symptoms —Blurred vision/painless —Metamorphopsia/micropsia —Asymptomatic Peripapillary Atrophy — Characterized by a diffuse circumferential, choroidal atrophy — May extend up to half a disc diameter beyond the disc margin — Rarely focal peripapillary atrophy — Has accompanying RPE hyperplasia — Up to 5-10% of POHS patients develop CNVM  Histo Spots — Atrophic spots consist of roundish, slightly irregular, yellowish-white, punched out lesions measuring between.2 and.7 DD in size — Pigment clumps in and at margin — May number 1 to 70, bilateral 60% — Linear streaks of chorioretinal atrophy in periphery noted in less than 5% of patients with POHS  Maculopathy Fluffy yellow-white focus of active Stage I choroiditis near macula Stage II Pigment (green/black ring) nodule with overlying serous detachment Stage III- Exudative detachment--SRNVM, severe V reduction in vision Subretinal Heme (red) or subretinal pigment & hemorrhages (grey/green) from net Hard exudates Disciform scar/fibrotic scar  Choroidal Neovascularization (CNVM) — Late manifestation of POHS between the ages 20-45 yrs — Bilateral macula involvement more common in men — May be triggered by stress — Most often associated with an old macular scar (histo spot in disc/macula area, rarely absence of scar) — if one eye has a net/scar, the fellow eye has a 9- 22% chance of development within 6-7 yrs. if presence of histo spot in disc macula area. — May develop within the peripapillary atrophy CNVM — Hyperpigmentation (round or oval ring within light gray area) indicates new growth of vessels WITHIN subretinal space rather than sub-RPE space — Some cases develop large grayish green membrane or mound indicating reactive proliferation of RPE attempting to surround CNVM  Diagnosis — A clinical diagnosis based on typical clinical signs — Lab testing of no value except in atypical cases — Skin testing may reactivate ocular condition — Linked to HLA-B7 and HLA-DRw2 — Suggests genetic predisposition — ?autoimmune/allergy response to antigen vs infection — IVFA (early hyperfluorescence and late leakage) and OCT  Management — Patient education-at risk — Routine exam and monitoring of vision, esp. if histo spots near macula — IVFA & OCT if suspicious active maculopathy — Antifungals are not beneficial — ? value of oral steroids in Stage I  Signs of Recurrence of Net — New areas of hypopigmentation near edge of scar — New areas of subretinal fluid or blood  Clinical Course — May have metamorphopsia, blurring of central vision, and scotoma with subtle serous elevation & focal yellow-white or gray lesion, may progress or resolve — A dark green- black ring and subretinal & intraretinal blood — High risk of maculopathy in good eye if atrophic scar in good eye & maculopathy in other eye: 9 to 22% chance — Must check vision with Amsler grid Differential Diagnosis — ARMD — Degenerative myopia — Angioid streaks — Choroidal ruptures — Idiopathetic net Toxoplasma gondii  Toxoplasmosis — One of the most common causes of posterior uveitis in the USA- 30-50% of all posterior uveitis in USA — Systemic Et.- obligate intracellular protozoan parasite- toxo gondii — Cat- final host, other animals as well as human are intermediate hosts: sporozoite, tachyzoite & bradyzoite — The organism has a predilection for nerve tissue and can persist encysted in retinal tissue for years — Systemic features- usually asymptomatic, AIDS (severe disease) — Congenital or acquired toxo — Recurrent active ocular toxo  Ways Humans Infected — Ingestion of tissue cyst in undercooked meat — Ingestion of sporocysts present in contaminated soil, food (dairy products) or water — Inhalation of spores from soil — Contaminated blood transfusions — Transplacental infection of the fetus from non- immunized infected mother Diagnostic Tests — Serum antitoxoplasma antibody titers — Indirect immunofluorescent antibody tests (IFA) — Haemagglutination tests — Enzyme linked immunosorbent assay (ELISA) — Sabin- Feldman dye test — PCR of ocular fluids  Congenital Toxoplasmosis — Primary maternal infection occurs during pregnancy from exposure to cat feces or raw meat — 60-70% of females at risk during child bearing yrs. — Infection during early pregnancy may result in stillbirth — Rate of fetal infection increases during pregancy:15% during first tri (severity of disease greater), 30% 2nd tri, & 60% during 3rd tri. — Infection in pregnancy may cause convulsions, intracranial calcifications, retinochoroiditis, paralysis, hydrocephalus, visceral involvement, intellectual impairment, & epilepsy — Most cases are subclinical with bilateral healed chorioretinal scars that maybe discovered later in life either by chance or a child is found to have defective vision (85% with ocular lesions without treatment)  Toxoplasma Retinitis — Most common cause of retinitis in otherwise a healthy individual — Recurrence usually takes place between the ages of 10- 35 when the cysts rupture from an old congenital reactive toxo scar or acquired toxoplasmosis — Compromised immune status of patient — Clinical features — Iridocyclitis- non or granulomatous- red eye — Focal (Y/W), fluffy, white, retinitis adjacent to an old scar (satellite lesion), overlying vitritis (headlights in fog), may be focal — Papillitis  Toxo in HIV patients — Multifocal deep retina — Occur without vitritis — High incidence of toxo encephalitis  Key Symptoms — Hazy vision — Floaters — Field loss — Red eye — Pain is absent  Key Signs — Anterior uveitis — Vitritis — Detached posterior face with inflammatory vitreous precipitates — Retinitis — Arteritis of neighboring vessels — Optic nerve inflammation Clinical Course — In uncompromised host, the retinitis heals within 1-4 months — Vitreous haze gradually clears, the inflammatory focus is replaced by a sharply demarcated atrophic scar surrounded by hyperpigmented borders Eyes With Toxo Lose Vision by — Direct involvement of the fovea, papillomacular bundle, optic nerve head, or major blood vessels — Indirect involvement may result in CME & macular pucker — Tractional retinal detachment by severe vitritis — Visual field defects by lesions Indications for Treatment — Not all lesions need to be treated esp. small peripheral lesions — A lesion threatening or involving the macula, papllomacular bundle, ONH, or major blood vessel — A very severe vitritis- if 2 line reduction — All AIDS pts  Treatment — Enlist infectious disease specialists — Systemic (Prednisone) steroids; never without anti- toxo meds, never subtenon injections — Clindamycin — Sulphonamides (Sulfadiazine) — Pyrimethamine plus folinic acid to minimize bone marrow toxicity — Co-trimoxazole — Azithromycin — Atovaquone  Management — Attempt to educate the community regarding expectant mother avoidance of cat litter and undercooked meat — Serological monitoring during pregnancy — Educate patient regarding inactive scars and follow at routine intervals Old quiet scar Nerve fiber defect Active lesion Healing lesion  Note NF defect Inactive lesion Recurrent Active Toxo Fresh active lesion without scarring Ocular Toxocariasis Canis — Intestinal parasite (nematode) Toxocara canis — Young children — Symptoms of unilateral floaters, blurred vision and photophobia  Toxocariasis — Caused by infection with a common intestinal ascarid (roundworm) of dogs called Toxocara canis, especially puppies — Human infection by accidental ingestion of soil or food contaminated with ova shed in dogs feces — Young children (2-4yrs) who eat dirt (pica/ geophagia) or are in close contact with puppies are at particular risk, ova can exist for long periods in the soil — Visceral larva migrans is the systemic form of acute toxocariasis  Ocular Toxocariasis Canis Ocular Signs — Unilateral chronic endophthalmitis: Uveitis, vitritis, and large elevated white granulomatous retinal lesion in either posterior pole or a — White, raised, granulomatous mass in periphery- 1DD in size- most common — Retinal/fibrous tractional folds radiating from lesion, deviated blood vessels towards mass and optic nerve dragging — VA will be extremely decreased (20/200 or worse) if lesion affected the macula  Diagnostic Tests — Enzyme – linked immunosorbent assay (ELISA)- the test of choice — Ultrasonography  Ocular Toxocariasis — Chronic endophthalmitis — Posterior pole granuloma — Peripheral granuloma Chronic Endophthalmitis — Presentation between the ages of 2 & 9 with leukocoria, strabismus, or unilateral visual loss — Signs: uveitis, vitritis, posterior or peripheral granuloma — Poor visual prognosis  Posterior Pole Granuloma — Presentation is typically with unilateral visual loss between the ages of 6 & 14 — Signs: anterior uveitis & vitritis are absent: round, y-w , solid granuloma (1-2 DD in size), usually located at the macula or between the macula & ONH, retinal stress lines  Peripheral Granuloma — Presentation is usually during adolescence or adult as a result of visual impairment from distortion of macula or retinal detachment — Signs: anterior uveitis & vitritis are absent, a white hemispherical granuloma located at equator, vitreous bands, dragged disc Treatment — Important to rule out retinoblastoma in young children — No real treatment except steroids to suppress the inflammation — In some cases, vitreoretinal surgery Visual Loss — Heterotropia of the macula — Retinal detachment Congenital Rubella Syndrome — First 8 weeks — Triad of hearing defects (100%), heart defects (70%), and ocular defects (30-60%) Congenital Rubella-Systemic Features — Results from transplacental transmission of virus to the fetus from infected mother usually during the first trimester of pregnancy — May lead to serious fetal infections and malformations — Spontaneous abortion, stillbirths, heart malformations, deafness plus others Ocular Signs — Pigmentary retinopathy- atypical RP or salt/pepper fundus — Bilateral nuclear cataracts — Microphthalmos — Glaucoma Ocular Rubella  Sarcoidosis — Class: granulomatous, inflammatory — System: Multisystem, usually pulmonary — Pathology: granulomatous — Sex: F>M, most common in 20-50 — Etiology: Possible allergy to airborne antigen, unknown — Race: B>W 10x in the USA — Symptom: Coughing and breathing difficulty: pt feels sick, fever, arthralgias — Systemic treatment: Steroids, Antimetabolites — Uveitis: granulomatous and chronic — 25-50% of all patients get U- maybe the presenting sign — Ask all U pts if they have a dry cough or chest pain — Associated with disc edema  Sarcoidosis — Idiopathic multisystem granulomatous infection: Skin, lungs, lymph, liver, spleen, and eyes — African descent, southern, eastern, and gulf states; Scandinavian countries — Females > males — Uveitis occurs independently of the activity or severity of systemic disease  Ocular Features — Anterior segment (conj, episclera, sclera & lacrimal)- granulomas — Keratoconjunctivitis sicca — Anterior uveitis: acute/chronic; granulomatous, mutton-fat KPs, severe posterior synechiae, iris nodules: older pts with chronic granulomatous uveitis- secondary glaucoma — Vitreous- Intermediate uveitis (R/O sarcoidosis), vitritis with snowballs — Periphlebitis- perivenous candle-wax drippings — Retinal granulomata — Choroidal granulomata (maybe mistaken for amelanotic melanoma) — Acute retinopathy — Peripheral retinal neovascularization — Optic nerve lesions also seventh nerve palsy  Management — Systemic steroids for 1st line therapy — Other treatments- oral NSAIDs, immunosuppressive agents — Ocular — Topical steroids & cycloplegic- anterior uveitis — Sub-Tenon & oral steroids for intermediate and posterior uveitis Candle wax drippings of ONH  Tuberculosis — Class: chronic, granulomatous, infectious disease — System: Multisystem; usually pulmonary — Etiology: Mycobacterium tuberculin bacteria; contagious by inhalation of infected respiratory particles in lungs — Symptoms: cough, fever, & malaise — Treatment: Antibiotics (Isoniazid- INH) — Any type of patient- intercity — Uveitis: chronic, granulomatous  Ocular Features — Phlyctenular keratoconjunctivitis — Chronic iridocyclitis — Koeppe & Buscaca nodules — Bilateral Choroiditis Tubercles — Diffuse multi-focal y/w nodular lesions (miliary) — Retinal vasculitis (periphlebitis): R/O Eales’ disease may progress to neovascularization — Optic neuritis — May resemble ocular sarcoidosis  Treatment of TB “one of the few uveitis that can be cured by antibiotic treatment” — Isoniazid (INH) Trial for 3 weeks — Pyrazinamide — Pyridoxine (Vit B6) — Rifampin — Ethambutol — Streptomycin  Syphilis — Bacterial infection– multisystem — Etiology: sexually- transmitted spirochete Treponema pallidum — Symptoms: Chancre, fever, & malaise; joint pain; lymphadenopathy, rash on palms or soles — Primary, secondary & tertiary stages — Ask all U pts if they have a rash of palm, sole of feet — 1% of all U pts have syphilis — “The Great Imitator” — Lab testing - FTA-ABS - MHA-TP - VDRL - RPR - PCR  Ocular Features Congenital — Salt & Pepper fundus: asymmetric VFs, may resemble RP — Pigment scarring with areas of depigmentation and pigmentation — Bilateral Interstitial keratitis: between ages of 5 & 25 — External features: widely spaced peg teeth (incisors) (Hutchinson’s), depressed nasal bridge, deafness, mental retardation & frontal bossing — Hutchison’s triad: IK, Deafness & teeth changes — Iridocyclitis — Multifocal chorioretinitis — Unifocal choroiditis — Neuroretinitis  Neuro-ophthalmic Features — Argyll Robertson pupils — Optic Nerve Atrophy — Ocular motor palsies (3rd & 6th nerve) — Visual field defects Management — Lumbar puncture — IV/ IM penicillin — Alternative to Penicillin-Tetracycline & erythromycin C/R atrophy in congenital syphilis Case Review — Patient Referred for Glaucoma Eval — Decreased Mental Capacity — Hx of Blepharospasm — BCVA: 20/20 OD, OS — Pupils = 1mm OD, OS — IOP: 13, 14 mmHg — Pachs: 511 and 502 Case Review — Gonio: Moderate Depth — Lens: 1-2+ NSC — Difficult View Secondary to Small Pupils Post Dilation KB Photos OCT VF VF Case Review — Small Pupils — Thin Pachs — IOP max 14 — Chronic Blepharospasm — Pale ONH’s — Generalized Loss on OCT — Enlarged Blind Spot with Generalized Constriction — What’s the Next Step? — Treat or Monitor? Conclusion — Multiple Aspects to Consider in a Glaucoma Eval — Beware of Mimicking Disorders — Multiple Visits are Valuable Pieces of the Puzzle Acute Retinal Necrosis or Progressive Outer Retinal Necrosis — Necrotizing herpetic retinopathies represent a spectrum of diseases induced by viruses of the herpes family — Patients with intact immunity tend to develop acute retinal necrosis and patients with severely impaired immunity with progressive outer retinal necrosis — Tests: PCR- very high sensitivity and specificity: aqueous & vitreous biopsy  Acute Retinal Necrosis Syndrome — Rare, but devastating necrotizing retinitis, in typically healthy individuals 20-50, males greater, tends to be caused by herpes simplex in younger patients and herpes zoster in older — Blurred vision (floaters), ocular pain, photophobia — The classic triad: arteritis & periphlebitis, confluent necrotizing retinitis of peripheral retina, moderate to severe vitritis, anterior uveitis, episcleritis/ scleritis, also disc edema — Clinical course: resolution of retinitis in 4-12 weeks with transparent & necrotic retina, with second eye involved in 2 mos. in 30-50% of pts — Cause of visual loss: retinal detachments & ischemic optic neuropathy, poor prognosis — Treatment: refer ASAP to retinal specialist, IV & systemic Acyclovir, systemic steroids, aspirin, laser, surgery Barricade laser Idiopathic Multifocal White-Dot Syndromes Rare, inflammatory disorders affecting retina, RPE, & choroid White Dot Syndromes — AMPPE — Serpiginous Choroiditis — MEWDS — Multifocal Choroiditis with Panuveitis — PIC — Birdshot Retinochoridopathy  Multiple Evanescent White- Dot Syndrome (MEWDS) — Rare condition with no treatment but good prognosis — One eye of young, healthy female with an acute onset of unilateral vision impairment, photopsia — Paracentral scotomas occurring in approximately 50% of patients soon after a flu-like illness — Active lesions consist of numerous, very small white dots at the level of the RPE at posterior pole — Associated features include mild vitritis and optic nerve swelling with enlarged blind spot — Vision recovery to 20/20 to 20/30 in 6-12 weeks Acute Multifocal Placoid Pigment Epitheliopathy (AMPPE) — Rare, bilateral disorder of the RPE in young adults with flu-like prodromal syndrome, visual prognosis is good — Presenting symptoms: subacute unilateral impairment of VA & paracentral scotomas, followed by other eye in a few days — Mild vitritis and multiple, chorioretinal lesions of a deep placoid cream-colored appearance confined to the posterior pole; ½ to 1 DD — IVFA is critical in diagnosis  Management — Up to 80% recover vision to 20/40 or better untreated — Fundus lesions resolve over 9- 14 days leaving RPE mottling — No specific treatment  Serpiginous or Geographic Choroidopathy — Uncommon, idiopathic, chronic progressive, bilateral disease in pts between the ages of 4-6 decades, poor prognosis — Chorioretinal lesions (deep, cream-colored opacities with hazy borders), jigsaw-puzzle-shaped lesions, starting around the optic nerve head and spreading out in all directions in propeller like fashion; bilateral but asymmetric: old atrophic scars — Associated features: mild anterior uveitis & vitritis — CNVMs in 25% of cases — Treatment: systemic & intravitreal steroids & cyclosporin Birdshot Retinochoroidopathy — Uncommon, bilateral, chronic condition in healthy middle-aged white women — Presenting symptoms: floaters, decreased central vision — Acute lesions: bilateral, flat, creamy- yellow, deep, ovoid spots with distinct margins — Midperiphery to posterior pole Birdshot Retinochoroidopathy — Bilateral and symmetrical — Middle aged — Vitritis, chorioretinal lesions with shotgun (birdshot) appearance < ½ DD — Strong relationship to HLA-A29 — Treatment: poor prognosis without treatment, oral or local steroids, immunosuppressive agents  Multifocal Choroiditis with Panuveitis Syndrome — Typically affects middle- aged females with recent flu like syndrome, fair prognosis — Anterior uveitis- may have severe uveitis with hypopyon: typically bilateral — Vitreous inflammation — Yellow-gray multifocal cream colored choroidal lesions scattered throughout the posterior pole — Optic disc edema — Pseudo POHS-later punched out scars: CNVMs — Treatment with periocular and oral steroids, antiVEGF Punctate Inner Choroidopathy (PIC) — Young, healthy patients ages 16-40 — 90% of patients with PIC are women — Most are moderately myopic — No history of preceding illness PIC – Signs andSymptoms — Blurred vision — Central or paracentral scotoma — Photopsia — Bilateral — Clustering of yellow spots with indistinct borders — 100-300 microns in size — Level of the RPE and Choroid — May be associated with overlying serous detachment — Posterior pole and mid-periphery PIC — May have Amsler Defect — Can have enlarged blind spot — Lesions resolve into punched out, pigmented scars (grow as become scarred) PIC Prognosis — Typically good prognosis if do not develop CNV — 75% of eyes retain 20/25 or better vision PIC with CNVM Others to Know — Systemic Lupus Erythematosus — Cutaneous disease — Secondary Sjogren’s syndrome — Retinal vascular lesions — Most common ophthalmic manifestation — Cotton-wool spots with or without intraretinal hemorrhages — Neuroophthalmic lesions — Scleroderma — Relapsing Polychondritis — Diffuse Unilateral Subacute Neuroretinitis (DUSN)