GOOD EVENING
The Uveal TractCongenital Anomalies of Uveal Tract
1. Coloboma: failure of mesodermal tissue of the eye to fuse tissue deficiency of iris. It may extend posteriorly (Ciliary Body, Choroid)…Extensive choroidal coloboma: will affect retinal growth
a. Scotoma (blind area).
b. White reflex on direct ophthalmoscopy..
2. Corectopia: ectopic pupil.
3. Polycoria: multiple pupils.
4. Persistent Pupillary Membrane
5. Heterochromia Iridis:
right iris color is different from the left iris.6. Aniridia: total or subtotal absence of the iris
Poor vision
Nystagmus
It may be associated with cataract or glaucoma.
It is either:
a. Sporadic (associated with Wilms tumour US of kidney)
b. Autosomal dominant
Coloboma
Corectopia
Polycoria
Persistent Pupillary Membrane
Persistent Pupillary Membrane
Heterochromia Iridis
Posterior Synechia
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Keratic precipitates (KPs)
UveitisThe uvea is the intermediate vascular layer of the eye which comprise the iris, ciliary body and choroid .
Uveitis: inflammation of uveal tract ,retina and blood vessels.
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Anterior uveitis: subdivided into iritis & iridocyclitis.
Intermediate uveitis: inflammation predominantly involving the vitreous.
Posterior uveitis: involve funds posterior to the vitreous base.
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Panuveitis: entire uveal tract inflammation.
Panophthalmitis: involve entire globe with orbital extention.Uveitis may be acute,incidoius, limited or persistant.
The coarse may be acute ,recurrent or chronic if it is more than 3 months.
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History
Age of presentation is very important, e.g. uveitis associated with juvenile idiopathic arthritis (JIA) is typically affects children.HLA B27 associated uveitis and Behcet syndrome usually affect young adults.
Sepiginous choroditis affect 5th-7th decade of life.
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Toxoplasmosis may affect any age group.
Hygiene and dietary habits: e.g. toxoplasmosis ( uncooked meat).History of sexual practice: in AIDS & syphilis.
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Past ocular history e.g. previous ocular trauma would point to sympathetic ophthalmia.
Past medical history: exposure to infectious agent e.g. Tuberculosis and syphilis.History of raring pits: in toxoplasmosis.
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Investigations
Generally not necessary in single attack if:
Mild unilateral acute anterior uveitis without suggestion of possible underlying cause.
Specific Uveitis entities .g. sympathetic ophthalmia.
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Indications for investigation
• Recurrent granulomatous anterior uveitis.• Bilateral case
• Systemic manifestations without specific diagnosis.
• Confirmation of suspected ocular picture.
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Investigations
Skin test Tuberculin skin testPathargy test
Serology Non-Trepanemal test
Trepanemal antibody test
Toxoplasmosis Dye test
Immunofluorescent antibody test
ELISA
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Retinal & choroidal biopsy
Radiology: chest X-ray, T.B and sarcoidosis
Sacroiliac joint,spondyloarthropathy
CT & MRI of brain and thorax.
Antinuclear antibody (ANA)for JIA.
Enzyme assay Angiotensin converting enzyme(ACE),for sarcoidosis,T.B & leprosy.lysosomes for sarcoidosis
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HLA tissue typing
Imaging Flurescein AngiographyIndocyanin Green
Ultrasonography
Biopsy conjunctival & lacrimal gland for Sarcoidosis
Aqueous sample with PCR for viral retinitis
Vitreous biopsy for endophthalmitis & intraocular Lymphoma
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Acute Anterior uveitis
Anterior uveitis is the most common form of uveitis. Acute anterior uveitis(AAU) is the most common form of anterior uveitis ,accounting for three-quarters of cases. It is easily recognized due to severity of symptoms, which force the patient to seek medical attention.
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Clinical features:
Symptoms:Presentation is typically with sudden onset of unilateral pain, photophobia and redness, which may be associated with lacrimation.
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Signs;
Visual acuity: is usually good at presentation, except in very sever cases with hypopyon.Ciliary ( circumcorneal) injection has a violaceous hue.
Miosis due to sphincter spasm may predispose to formation of posterior synechiae unless the pupil is dilated.
Aqueous cells indicates disease activity and their number reflects disease severity.
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Anterior vitreous cells indicate iridocyclitis.
Aqueous flare reflects the presence of protein due to break down of blood aqueous barrier.Endothelial dusting may be myriads of cells is present early and gives rise to a “dirty” appearance and true keratic precipitates usually appears only after a few days and are usually non-granulomatous.
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Hypopyon is a feature of intense inflammation in which the cells settle in the inferior part of anterior chamber and form a horizontal level.
Dilated iris vessels .
Posterior synechiae may develop quite quickly and must be broken down before they become permanent.
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Low IOP is the rule as a result of reduced secretion of aqueous by ciliary epithelium .Occasionally ,the IOP may be elevated (hypertensive uveitis) in herpetic uveitis and toxoplasma retinitis.
In AAU associated with HLA-B27,the hypopyon has a high fibrin content which makes it dense, immobile and slow to adsorb.
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In patients with Behcet syndrome, the hypopyon has minimal fibrin content and there fore shifts according to patient head position and may disappear quickly.
Hypopyon associated with blood occurs in herpetic infection and in eyes with associated rebuosis iridis.
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Treatment:
Mydriatics ( short and long acting)To promote comfort .
To break down recently formed synechiae .
To prevent formation of posterior synechiae.
Topical steroid;
Intensive therapy, once inflammation controlled, gradual therapy.
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Steroids
Systemic steroidPeriocular steroids
Intraocular steroid
Slow release implant
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Antimetabolites:
Steroid spearing therapy and for sight threatening uveitis.Azathioprine , Methotroxate
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Biological agents
Tumour necrosis factor alpha therapy; infliximab.Immune modulator therapy: cyclosporine , tacrolimus.
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Granulomatous uveitis,Mutton fat KPs
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Fine ,diffuse KPs
Complications:Raised IOP
Cataract
Corneal complications: HSK
Cystiod macular edema
Retinal detachment
Hypotony
Vitreous opacification and vitritis
Retinal and choroidal neovascularization
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