Ophtha - Uvea and Sclera

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UVEA AND SCLERA Dr. Marin

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Sclera

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Fibrous outer global covering of the eye Continuous with cornea and optic nerve dural sheath Thin (0.3 m) at rectus muscles insertion (N: 0.1 mm) Around ON, penetrated by long and short post.ciliary arteries and nerves 4 vortex veins: slightly posterior to equator of eye/globe EPISCLERA o Thin layer of fine elastic tissue o Blood vessels LAMINA FUSCA o Inner surface brown pigment layer

SCLERAL DISEASES Blue Sclera -

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Thinned out (collagen) Occurs in several disorders such as osteogenesis imperfecta, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, prolonged steroid use, pseudohypoparathyroidism Marfan’s syndrome o More common are dislocation of vessels Newborn: keratoconus and keratoglobus

Associated with pronounced choroidal atrophy May be congenital, large w/ poor vision or high myopia or normal vision Differentiate with: scleral ectasia in Optic Nerve coloboma

INFLAMMATION Episcleritis More common in superficial and inter-recti areas and limbus and more benign 66% unilateral 30-40y/o; may be up to 60 Recurrence rate: 66% (recurrence is the rule!) More on female Secondary to staphylococcal toxins/TB/other granulomatous type of cells Rarely perforates S/Sx: ciliary injection, diffuse w/ or w/o nodules, usually temporary, mild-mod pain with tenderness, 15% photophobia, rarely affects cornea and iris, self-limiting, no ectasia appears Scleritis Less common, involves deep sclera 2 types o Anterior: diffuse, nodular or necrotizing, w/or w/o inflammation (scleromalacia perforans) o Posterior: usually associated with anterior S/sx o mod to sev pain o usually with photophobia o ciliary injection, scleralizing, occasionally nodular, mod-severe pain at ciliary ganglion th th Peak incidence: anterior, early – midlife; posterior 4 -6 decade High recurrence rate High incidence of perforation

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Scleral Ectasia -

Early childhood: prolonged high IOP, congenital glaucoma Congenital anomaly: optic disk or macula

Staphyloma -

Bulging out of uvea into a stretched and thinned out sclera Localized global segment Anterior: over ciliary body (ciliary staphyloma), equatorial, posterior (high myopia), between ciliary and limpus (interciliary) Usually present in high myopia GABAY MEDISINA 2011-2012 / 3rd Year /

zaborina reyes

Primary tissue involvement Redness Pain Blurry vision Occurrence Laterality Incidence Perforation Nodules

Episcleritis superficial

Scleritis deep

Focal Mild-mod Mild Recurs/MC in women Unilateral 30-40 yo

Diffuse Mod-severe Mod-severe Recurs/MC in women Ant: Bi Post: Uni 40-60 yo

Common

Uncommon

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SCLERITIS Necrotizing Most severe (brawny scleritis) Acute, painful, tender localized congestion and necrosis May remain localized or involve entire globe Scleromalacia and scleral ectasia Inc IOP – exacerbates ectasia Steroids may thin it further 35% of death w/in 5 years Necrotizing Scleromalacia Stereomalacia perforans Assoc with severe RA Scleral thinning, melting and perforation Without historical and clinical evidence of inflammation May be secondary to trauma Prognosis: site dependent but generally poor Steroids are c/i Homologous preserved scleral graft

SCLERAL INJURIES -

Signs: -

Trauma: blunt or perforating, lacerations assoc with internal eye injuries: RD, vitreous hemorrhage Physical damage: irradiation, thermal burns Chemicals: alkali and acid burns Treatment: antibiotics, repair with conjunctiva and Tenon’s capsule approximated

Hypopyon – pus in anterior chamber Ruptured Globe Vitritis Retina: primary target of infection agents Choroid: primary target of granulomatous processes

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Scleral Injuries Trauma Physical damage Chemicals Tx: ABCs, w/ repair of conjunctiva and Tenon’s capsule

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Hyaline degeneration Fairly frequent in sclera, >60 Small, round, translucent gray areas loc ant to the insertion of rectus muscles No sxs or complications

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Middle coat of the eye Made up of o Iris o Ciliary body o Choroid GABAY MEDISINA 2011-2012 / 3rd Year /

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Vascular layer: contributes to blood supply of retina Intimately related to cornea anteriorly and retina posteriorly

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Anterior extension of CB Flat surface with central circular aperture – pupil, inc or dec size by intrinsic muscles: dilator or sphincter pupillae, respectively Divides AC from PC Unfenestrated endothelium of vasculature, does not leak IV fluorescein 2 layers of epithelium: posterior surface heavily pigmented, anterior extension of RPE Blood supply: major iris cycle

Iris

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Ciliary Body Roughly triangular in cross section 2 anterior surface zones: corona ciliaris) corrugated anterior 2mm) and pars plana (smoother and flatter posterior 4mm) 2 layers ciliary epithelium: external pigmented and internal non-pigmented Pigmented epithelium represents forward extension of RPE Ciliary muscles: longitudinal, radial or circular for accommodation Pars plana: consists of thin layer of ciliary muscles and vessels Choroid Most posterior part of uvea, vascular layer Choroidal vessel bound by Bruch’s membrane internally and suprachoroid 3 layers of blood vessels: large (mostly veins draining to 4 vortex veins), medium and small (choriocapillaries) -– supplied outer retina

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Inflammation of uvea Acute non-granulomatous Posterior uveitis o Usually chronic

Onset Pain Photophobia Blurred Vision Circumcorneal flush Keratic Precipitates

Nongranulomatous Acute Marked Marked Mod Marked Fine White

Pupil

Small and irregular

Post synechiae Iris nodules Site Course Recurrence

Sometimes None Ant Acute Common

Granulomatous Insidious None or minimal Slight Marked Slight Large Gray (mutton fat) Small and irregular (variable) Sometimes Sometimes Ant, Post, Diffuse Chronic Sometimes

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Anterior uveitis -

Marie-Strumpell Ankylosing Spondylitis -

Iris irregular Keratic precipitates Posterior synechiae Mutton fats Hypopyon

(+) HLA B27: lumbosacral fusion Male preponderance Eventually leads to permanent damage Inc ESR but non-specific RF test – exc for 5% asx pxs

Fuch’s Heterochromic Uveitis Acute: caused by viruses Granulomatous: granuloma disease (TB, leprosy, histoplasmosis) -

Can develop nodules o Busacca: stroma o Koeppe: margin Anterior Uveitis Slit-beam illumination – shows transudation Early, acute anterior uveitis – can change color of iris Irregular pupil posterior synechia Cardinal Finding: Mutton fat nodules – keratic precipitates if small amounts only, posterior synechiae w/c secludes pupil and cataract Posterior Uveitis No anterior segment signs Contrast CT scan may be helpful Suspect is with pain, proptosis, papilledema and exudative retinal detachment Usu unilateral, severe pain, dec VA, diplopia, EOM limitations Assoc with severe RA Recurrence may lead to severe thinning results in staphyloma Complications: keratitis

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Unknown cause Usually unilateral Iris color different Onset insidious rd th 3 or 4 decade Blurred vision due to cataract; less common, glaucoma Most obvious over iris sphincter Keratic precipitates are small and stellate Good prognosis Tx: surgery

INTERMEDIATE Uveitis Also Pars planitis, Chronio Cyclitis -

Young patient presenting with “floating spots” “snowballs” “snowbanking” nd 2 MC type of intraocular inflammation Hallmark: vitreous inflammation Bilateral; commonly in late teens or early adult MC in men Striking finding: vitritis u/k cause: MS and sarcoidosis in 10-20% cases complications: macular edema, retinal vasculitis, neovascularization of optic disc

UVEITIS SYNDROMES LENS INDUCED

Associated with Joint Disease

Phacogenic Endophthalmitis Phacoanaphylactica (EPA)

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Heterochromic iris 20% deelop bilateral non-granulamatous iridocyclitis Females No correlation between arthritis onset MC: knee Uveitis in 3-10 years

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Autoimmune disease secondary to lens-induced uveitis Lens develops hypermature cataract and lens capsule leaks lens material into posterior and anterior chambers  inflammatory reaction Tx: removal of lens material

Endophthalmitis Phacoanaphylactica

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Juvenile RA

Adult peripheral RA -

Iridocyclitis association coincidental More prone to scleritis and sclera-uveitis Associated with cells and flares in AC aqueous accompanying scleritis misinterpreted as iridocyclitis Occlusion pupillae GABAY MEDISINA 2011-2012 / 3rd Year /

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Severe phacogenic uveitis Post ECCE: lens material sensitization High flares

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Sympathetic Ophthalmia -

Rare but devastating bilateral granulomatous uveitis ff a perforating eye injury 90% occurs 1 year after injury Sympathetic Uveitis o Bilateral granulomatous uveitis o Forms non-caseating tubercles, inflammation from uvea to ON sheaths o Photophobia, redness and BOv o Hypersensitivity to pigment containing cells in the uvea

Tuberculous -

Granulomatous, diagnosed more clinically Characteristically localized: chorioretinitis Caseating necrosis on histopath Uncommon in pxs with active pulmonary TB

Sarcoidosis (Boaek’s Sarcoid) -

Mikulicz’s syndrome – involvement of lacrimal glands Heerfordt’s disease – involvement of parotid glands (uveoparotid fever) 90% lung involvement Uveitis occurs ins 25% of patients Noncaseating cranuloma + biopsy: cutaneous nodule even at tarsal or bulbar…

Toxoplasmosis -

Cat definite host

Histoplasmosis -

Toxocariasis -

Canis: dog Cati: cat Localized choroidal granuloma Oral-fecal route

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Endemic in Ohio and Missisipi River valley Areas Punched-out spots o Small, irregularly round or oval, usu depigmented centrally w/ finely pigmented border Tx: amphotericin

GABAY MEDISINA 2011-2012 / 3rd Year /

zaborina reyes