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The uveal tract Nicolette Sohar Department of Ophthalmology, University of Szeged The uveal tract: Iris Ciliary body Choroid Vascular supply: ophthalmic artery, short posterior ciliary arteries, long posterior ciliary arteries, anterior ciliary arteries, vorticose or vortex veins Sensory supply: long and short ciliary nerves Iris Structure: anterior mesodermal stromal layer posterior ectodermal pigmented epithelial layer Collarette of the iris: pupillary and ciliary part Surface: crypts and trabeculae Color: melanin content of the melanocytes Examination methods: slit lamp (Tyndall effect, cells), gonioscopy, Developmental anomalies Aniridia: bilateral, autosomal dominant or sporadical, traumatic Associated with: foveal hypoplasia, nystagmus, amblyopia, buphthalmos, cataract Coloboma: incomplete fusion of the embryonic optic cup in about the sixth week of pregnancy medially and inferiorly directed iris, ciliary body, zonule fibers, choroid, optic nerve Pigmentation anomalies Iris bicolor: one iris containing varying pigmentation Heterochromia: congenital difference in coloration between the left and right iris Fuchs`heterochromic cyclitis: recurrent iridocyclitis (precipitates, cataract, glaucoma) Sympathetic: unilateral impairment of the sympathetic nerve supply (affected – lighter) Melanosis: dark pigmentation of the iris Albinism: metabolic disease that leads to hypopigmentation of the eye ocular: only the eyes oculocutaneous: eyes, skin and hair iris is light blue choroidal vessels detectable reduction in visual acuity photophobia nystagmus Inflammation 1. Acute iritis and iridocyclitis: Symptoms: dull pain, impaired vision, photophobia, excessive tearing Etiology: immunologic causes, HLA-B27associated, symptom of systemic disease infections HLA-B27 associated:idiopathic ankylosing spondylitis Reiter`s syndrome regional enteritis ulcerative colitis psoriasis Non-HLA-B27 associated: idiopathic viral tuberculosis sarcoidosis syphilis leprosy RA heterochromic iridocyclitis phacogen uveitis trauma Ophthalmic picture: -ciliary injection -combined injection -diffuse structure and reactive miosis -cellular infiltration of the anterior chamber and protein or fibrin accumulation -precipitates -hypopyon -hyphaema Complications: Secondary open angle glaucoma Anterior synechiae Posterior synechiae Differential diagnosis of iritis and acute glaucoma Differential criteria Acute iritis Acute glaucoma Symptoms Dull pain, photophobia Intense pain and vomiting Conjunctiva Combined injection Combined injection Cornea Clear Anterior chamber Pupil Normal depth; cells and fibrin Narrowed Globe Normal pressure Opacified, edematosus Shallow Dilated, not round Rock hard Treatment: topical systemic antibiotic or antiviral due to a pathogen therapeutic mydriasis (atropin,scopolamin cyclopentolate, epinephrine) steroid therapy- topical (prednisolon) subconj.(dexamethason) where no pathogen Secondary open angle glaucoma: beta blockers carbonic anhydrase inhibitors Prognosis: Symptoms usually improve within a few days when proper therapy is initiated. The disorder can progress to a chronic stage. 2. Chronic iritis and iridocyclitis: Epidemiology: one quarter of all iridocyclitis have a chronic clinical course Symptoms: may exhibit minimal symptoms Ophthalmic picture: like acute iridocyclitis Differential diagnosis: acute glaucoma, conjunctivitis, keratitis Complications: Pupillary block- obliteration of the pupil by posterior synechiae Secondary angle closure glaucoma with `iris bombe` Calcific band keratopathy Occlusion of the pupil Fibrous scarring in the pupil posterior subcapsular opacities secondary cataract Treatment: in pupillary block – Nd: YAG laser iridotomy secondary cataract – cataract extraction Prognosis: may progress to blindness from shrinkage of the eyeball Rubeosis iridis Neovascularization in the iris that occurs in various retinal disorders. Etiology: causes - proliferativ diabetic retinopathy - retinal vein occlusion - retinal periphlebitis Signs and symptoms: neovascularisation in the stroma asymptomatic neovascularisation in the angle of the anterior chamber irreversible secondary angle closure glaucoma Symptoms of the secondary angle closure glaucoma: loss of visual acuity intense pain conjunctival and ciliary injection “rock hard” eyeball Differential diagnosis: acute angle closure glaucoma Treatment: -prompt laser treatment of retinal disorders -transscleral freezing of the ciliar body (cyclokryotherapy) to reduce intraocular pressure (sec glaucoma) -when falls, (or the eye shrinks(phtysis)) intense pain enucleation is indicated Prognosis: irreversible blindness loss of an eye Ciliar body Structure: from the root of the iris to the ora serrata anterior - pars plicata posterior- pars plana ciliary processes Function: ciliary muscle – accomodation epithelium covering the ciliary body – produces the aqueous humor Inflammation 1. Intermediate uveitis Symptoms: decreased vision, floaters, photopsia, rarely pain Pathogenesis: immun-mediated or infectious, inflammation of vitreous base Risk factors: Inflammatory – MS, sarcoidosis Infectious – Lyme, syphilis, toxocariasis, tb Genetics – assoc w/ HLA-DR 15 Ophthalmic picture: pars planitis: idiopathic intermed.uveitis associated with snowbank and vitreous snowballs. -inflammation involving the vitreous base and peripheral retina -anterior vitreous inflammation -snowballs, snowbanks, peripheral venous sheating -CME Prognosis: -self-limited or chronic -within first decade of life- more severe inflammation and greater resistance to therapy than in older pts. Complications: -permanent decreased visual acuity -posterior subcapsular cataract -cyclitic membrane -CME -epiretinal membrane -retinal detachment -hypotony -retinal neovascularisation -vitreous haemorrhage -phtisis bulbi Treatment: -infectious- antibiotics -periocular or systemic corticosteroids -immunosuppressants (eg,cyclosporin, azathioprine, mycophenolate mofetil, methotrexate) -vitrectomy – persistent vitreous debris or haemorrhage (limiting visual function) Choroid Structure: middle tunic of the eyeball bounded on the interior by Bruch`s membrane highly vascularised: -vessel layer with large vessels -capillary layer highest blood flow in the entire body Inflammation 1. Choroiditis: Incidence: four cases per 100000 people Symptoms: free of pain, blurred vision, floaters Etiology: toxoplasmosis sarcoidosis syphilis Behcet`s disease histoplasmosis Ophthalmic picture: -isolated or multiple choroiditis foci -acute disease - ill defined white dots -scarring – foci are sharply demarcated with a yellowish-brown color -major choroidal vessels are visible -atrophic scars -no cells in the vitreous bodyprimary choroidal process -cellular infiltration of the vitreous bodyretinochoroiditis Differential diagnosis: retinal inflammations with vitritis - caused by viruses or Toxoplasma gondii Treatment: antibiotics steroids Prognosis: inflammatory foci will heal within two to six weeks localised scotomas chorioretinal scars reduced visual acuity Sympathetic ophthalmia Perforating wound to the eye, or intraocular surgery Chronic irritation of one eye Transferred uveitis in the fellow eye Specific bilateral inflammation of the uveal tract Epidemiology: very rare Etiology: occur in an unaffected eye even years after penetrating injuries or intraocular surgery in the fellow eye. Tissues in the injured eye act as antigens Autoimmune disorder in the unaffected eye Symptoms: -limited range of accomodation -photophobia -diminished visual acuity -pain Ophthalmic picture: -combined injections -cells and protein in the anterior chamber and vitreous body -papillary and retinal edema -granulomatosus inflammation of the choroid Differential diagnosis: -iridocyclitis -choroiditis Treatment:- the injured, usually blind eye - must be enucleated to eliminate the antigen - high dose topical and systemic steroid therapy - concurrent immunosuppressives (cyclophosphamide, azatioprin) Complications: -chronic clinical course -severe complications -secondary glaucoma -secondary cataract -retinal detachment -shrinkage of the eyeball -blindness Tumors 1. Benign choroidal tumors choroidal nevi: 11% of the population secondary neovascularisation with retinal edema (usually asymptomatic) 2. Malignant tumors Uveal melanoma - incidence of one per ten thousand - usually choroidal - unilateral Iris melanomas: often initially asymptomatic melanoma cells in the angle of the anterior chamber Complications: secondary glaucoma Therapy: segmental iridectomy Ciliary body melanomas: changes in accomodation and refraction Therapy: resected en bloc Choroidal melanomas: Symptomatic: - involvement of the macula reduces visual acuity - retinal detachment Diagnosis: transillumination, ultrasound, fluorescein angiography Therapy: radioactive isotopes (brachyterapy) enucleation (>8mm x 5 mm) Uveal metastases: from: breast lung flat with little pigmentation