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Peripheral corneal diseases

Peripheral corneal diseases can be congenital, inflammatory, degenerative or neoplastic. Rheumatoid arthritis commonly causes peripheral keratitis like marginal keratitis and phlyctenular keratoconjunctivitis due to rheumatoid vasculitis affecting peripheral cornea. Wegener's granulomatosis can also involve peripheral cornea with necrotizing scleritis and peripheral keratitis. Treatment involves controlling the underlying systemic disease with immunosuppressants and managing ocular inflammation with corticosteroids.

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PERIPHERAL CORNEAL DISEASES P R E S E N T E R – D R S A D H W I N I M H M O D E R ATO R – D R S H I VAY O G I R K U S A G U R
PERIPHERAL CORNEA • Peripheral cornea - that portion located between the central 50% of the cornea and the limbus • Thickest region (upto 0.7mm)of the cornea • Avascular, a vascular arcade originating from the anterior ciliary arteries extends approx. 0.5 mm into clear cornea • Presence of more Langerhans’ cells • Higher concentrations of IgM and C1 (important in the activation of the classic complement pathway) • Its proximity to the limbus and conjunctiva results in a unique collection of abnormalities. • Thus, vascular inflammatory disorders, limbal infections, collagen vascular disorders, neoplastic disease, and local degenerations may affect the peripheral cornea in a distinctive way.
PERIPHERAL CORNEAL DISEASES CONGENITAL /DEVELOPMENTAL /INHERITED Lattice dystrophy type II Wilson’s disease Conea plana Sclerocornea Posterior embryotoxon Axenfeld-Rieger anomaly INFLAMMATORY /AUTOIMMUNE Rheumatoid Arthritis Polyarteritis Nodosa Wegener’s Granulomatosis Marginal keratitis Phlyctenular keratoconjunctivitis Mooren’s ulcer Superior limbic keratoconjunctivitis Contact lens induced DEGENERATIVE DISORDERS WITHOUT CORNEAL THINNING Corneal Arcus Lipid keratopathy Band keratopathy Spheroidal degeneration Limbal Girdle of VOGT Hassall Henle bodies DEGENERATIVE DISRDERS WITH CORNEAL THINNING Furrow degeneration Terrien’s Marginal degeneration Pellucid Marginal degeneration Dellen NEOPLASTIC Dermoid Benign squamous neoplasms Conjunctival Intraepithelial Neoplasia Invasive Squamous call carcinoma Conjunctival Melanoma
C O N G E N I TA L / D E V E L O P M E N TA L / I N H E R I T E D C O N D I T I O N S
LATTICE DYSTROPHY TYPE II • Meretoja’s syndrome or Familial Amyloid Polyneuropathy type IV • Associated with Systemic amyloidosis • Not a true corneal dystrophy • Corneal changes are later in onset • Favourable visual outcome • Mutations in the Gelsolin (GSN) gene, on Chr 9 (9q34) • OCULAR – CORNEA – fewer lattice line, radially oriented and involve primarily the peripheral cornea, with relative central sparing. – Reduced or absent corneal sensitivity. – Glaucoma and pseudoexfoliation with or without glaucoma are common
• Systemic manifestations – Systemic amyloidosis – dermatochalasis, facial drooping along with protruding lips – typical facies. – Cranial and peripheral neuropathies such as facial paresis – Orthostatic hypotension, dysfunction of perspiration – Cardiac conduction abnormalities, carpal tunnel syndrome – Histopathology - mutated gelsolin deposited in arteries, skin, peripheral nerves, sclera, etc. A regular amyloid layer lies beneath a normal-appearing Bowman’s membrane. • Confocal microscopy – Stromal haze with highly reflective deposits - amyloid. – Pleomorphic basal epithelial cells, deposits seen just posterior to these cells.
• MANAGEMENT • Depends on the patient’s symptoms • Recurrent erosions - patching, hypertonic agents, artificial tears, or a therapeutic contact lens • Excimer laser phototherapeutic keratectomy - optional treatment for recurrent erosions and superficial opacities
WILSONS DISEASE • AR – Ch 13q14.3 • Accumulation of copper in most body tissues • Kayser-Fleischer ring is present in approximately 95% of patients – Yellow-brown or green ring located at the level of Descemet’s membrane in the peripheral cornea with no clear interval • Begins peripherally at Schwalbe’s line and progresses centrally. • Gonioscopy - to locate the ring in its early stage • Wilson’s disease - systemic D-penicillamine - prevents disease progression and reduces the amount of copper already deposited in tissues.
CORNEA PLANA • AR or AD. Gene locus – KERA gene (12q22) • Cornea is flat - curvature <43D, typically 30-35D • Alteration in tertiary structure of keratan sulfate proteoglycans • Developmental arrest of corneal curvature relative to sclera • Associated with sclerocornea or microcornea • Angle closure glaucoma - 20% • Other ocular - Arcus juvenilis, nonspecific corneal opacities, anterior segment dysgenesis, uveal & retinal coloboma, aniridia, congenital cataract, ectopia lentis • Systemic – osteogenesis imperfecta, Ehler Danlos syndrome type IV • Management – correction of refractive error, monitor for associated findings
SCLEROCORNEA • 50% -sporadic mutation, 50% - AD/AR/XLD • During embryonic development from 2nd mesenchymal wave forming tissue that resembles sclera instead of clear corneal stroma • May occur in peripheral cornea of one or both eyes, may be complete with an absence of clear cornea. • Commonly associated with cornea plana (80%) • Other ocular – Angle closure glaucoma, high refractive errors, blue sclera, Peter’s anomaly, uveal & retinal coloboma, cataract and microphthalmos. • Systemic – anomalies of skull & facial bones, external ear deformities, polydactyly, testicular abnormalities, osteogenesis imperfecta. • Management – few therapeutic options available – limited to control raised IOP, subsequent keratopalsty.
POSTERIOR EMBRYTOXON • AD • Clear to white line in peripheral cornea – thickened & centrally displaced anterior border ring of Schwalbe • seen with gonioscopy as a fine ground-glass-like membrane • When posterior embryotoxon exists without additional pathology it does not require treatment
AXENFELD – RIEGER ANOMALY • AXENFELD’S ANOMALY - AD – Corneal clouding, posterior embryotoxon, prominent iris processes (iridocorneal adhesions to or beyond schwalbe’s line) – Glaucoma - 50% – Axenfeld’s syndrome – when associated with systemic anomalies – hypertelorism, facial asymmetry, hypoplastic shoulder • RIEGER’S ANOMALY – AD/sporadic – Atrophy of iris stroma and corneal abnormalities – PAS, corectopia, ectropion uveae, pseudopolycoria – Glaucoma - >50% – Rieger’s syndrome – when associated with skeletal, cranial & dental abnormalities
• AXENFELD-RIEGER’S ANOMALY & SYNDROME • ANOMALY – Findings of both conditions – spectrum of anterior segment disorders • SYNDROME – When associated with systemic developmental defects • M Bruce Shields – primordial endothelium is retained over parts of iris and angle late in gestation. These undifferentiated cells contract – iris stromal thinning & hole formation. • MANAGEMENT – aqueous suppressants, goniotomy, trabeculectomy with antimetabolites
I N F L A M M AT O R Y / A U T O I M M U N E C O N D I T I O N S
RHEUMATOID ARTHRITIS • A chronic inflammatory disease of unknown etiology, affecting primarily synovial joints, and less frequently extra-articular tissues, such as the eye, pleura, pericardium, and nerves. • F>M • Between 4th and 6th decades • Presents as a symmetrical polyarthritis, involving small joints of the hands and feet, as well as larger joints • Presentation with ophthalmic disease - with severe, long-standing RA and high titers RF • Ocular manifestations – keratoconjunctivitis sicca, scleritis, sclerosing keratitis, keratolysis
• Pathogenesis • The etiopathogenesis of RA is unknown Immunoglob ulin & Complement deposition within blood vessel wall Focal areas of wall necrosis Rheumat oid Vasculitis Increase in permea bility causing leakage Chemotactic factors, Inflammatory cytokines (IL- 1, TNFα) Neutrophils recruited at peripheral cornea liberate proteolytic and collagenolytic enzymes Corneal destruction Immune mediated Hypersensit ivity Cell mediated Hypersensi tivity scleritis • High titers of rheumatoid factor - higher incidence of extra articular manifestations, more severe joint destruction, and worse prognosis
• KERATOCONJUNCTIVITIS SICCA • most common associated (15–25% of RA patients) • gritty or sandy FB sensation, burning, irritation, mucus discharge, and photophobia • diminished tear meniscus, Schirmer test - decreased tear production • Punctate epithelial keratopathy, mucus stranding, and filamentary keratitis • SCLERITIS • 20% of RA patients, isolated episcleritis in up to 11% • severe boring ocular pain and photophobia • Anterior scleritis - diffuse, nodular, necrotizing scleritis with inflammation and necrotizing scleritis without inflammation, and posterior scleritis. • exhibit zones of intense scleral inflammation, edema, and necrosis with overlying epithelial defects • Also referred to as scleromalacia perforans - Severe scleral thinning diffuse anterior scleritis
necrotizing scleritis post cataract suregery filamentary keratitis diffuse scleromalacia perforans in a 70-year-old patient with long-standing RA acute marginal stromal infiltrates PUK with scleritis, epithelial defect limbal guttering, absence of stromal infiltrate PUK
• KERATITIS • PUK – crescent shaped peripheral corneal thinning, a leading edge of stromal WBC infiltration, epithelial defect overlying the ulceration, with scleral inflammation • SCLEROSING KERATITIS – adjacent to an area of active scleritis. • Peripheral cornea becomes thickened and opacified and may become secondarily vascularized • ACUTE STROMAL - as stromal opacities with edema. The opacities may coalesce if left untreated. Overlying epithelium may break down – PUK • LIMBAL GUTTERING - epithelium remains intact and the extent of peripheral thinning may be variable • KERATOLYSIS • an acute severe melting of the corneal stroma which can proceed to perforation. • most commonly seen in patients with rheumatoid-associated necrotizing scleritis • Loss of stroma occurs in regions of clear cornea and may progress to descemetocele formation. keratolysis
• MANAGEMENT : therapy directed toward control of the systemic vasculitis • System disease : NSAIDs, corticosteroids and DMARDs • KCS : ocular surface lubrication – Moderate doses of topical steroids - underlying inflammatory component. – Topical ciclosporin A drops BD for management of dry eye have – Nutritional supplementation such as omega-3, omega-6, linoleic acid, linolenic acid, and other essential fatty – Punctal occlusion, Medial canthoplasty and/or lateral tarsorrhaphy can be performed • KERATITIS : Oral corticosteroid and topical ciclosporin A play a major role – Topical steroids - with great caution in furrowing with epithelial defects and sterile keratolysis – suture or chemical tarsorrhaphy - persistent epithelial defects – Keratolysis - a patch graft • NON NECROTIZING SCLERITIS : mild to moderate - include oral NSAIDs – Indometacin (25 mg) or ibuprofen (600 mg) TID or naproxen (500 mg) BD – Topical steroids can be used initially in combination with oral NSAID – Anterior scleritis - subconjunctival corticosteroid injections
• NECROTIZING SCLERITIS : Infectious etiologies must be initially ruled out – Topical ciclosporin A, Protease inhibitors such as tetracyclines may be considered – Acute inflammation - oral prednisolone at an initial dose of 1 mg/kg/day, with a subsequent slower tapering – High-dose IV pulse methylprednisolone - 1 g per day for 3 consecutive days followed by oral therapy – Scleromalacia perforans - observation, eye protection, and therapeutic modulation of systemic disease • IMMUNOSUPPRESSIVES : in recalcitrant cases of scleritis or keratitis, including PUK, and keratolysis – Immunomodulators - antimetabolites, alkylating agents, T-cell inhibitors, and biologic agents. • SURGICAL MANAGEMENT : – Cyanoacrylate glue application with oversized BCL, donor scleral grafting, AMT, lamellar or penetrating keratoplasty, aggressive lubrication and tarsorrhaphy may enhance surgical success.
POLYARTERITIS NODOSA • Periarteritis Nodosa - vasculitis of small and medium-sized arteries leading to multiple organ system disease • middle age, M>F • etiology is unknown • Ocular (10– 20%) - secondary to HTN or vasculitis of retinal vessels. – Direct nerve involvement - EOM palsies and optic nerve infarction – Orbital involvement - pseudotumor-like lesion – Conjunctival nodules - edema and necrosis of conjunctival vessels & uveitis • Anterior segment - scleritis, sclerokeratitis, and PUK weig ht loss ≥4 kg testic ular pain livedo reticul aris Myalg ia PMNs in artery walls in biopsy Mon/p olyneur opathy High BUN or Creatini ne HTN with DBP>90 mmHg HBSAg or Ab in serum • Treatment - systemic disease • Corticosteroids, Immunosuppressives, which have markedly improved the outcome ANY 3 OF,
WEGENER’S GRANULOMATOSIS • Systemic vasculitis characterized by necrotizing and granulomatous inflammation of the vessels of the upper and lower respiratory tract, glomerulonephritis, and other organ involvement with small vessel inflammation. • Etiology is unknown • Mid-40s, M:F=2:1 • Can present in children, with ocular involvement as the initial manifestation • Ocular (58%) : – Proptosis with orbital pseudotumor or orbital inflammation, nasolacrimal duct obstruction – Retinal vasculitis and ischemic optic neuropathy Upper airway symptoms of sinusitis, Pulmonary infiltrates on CXR normocytic normochrom ic anemia Elevated ESR, positive RF Circulating immune complexes, ANCA Non specific complaint s Any 2 of 4
Focal necrotizing scleritis with peripheral keratitis Residual grainy anterior stromal opacities Peripheral anterior stromal and subepithelial infiltrates necrotizing scleritis
• Anterior segment - conjunctivitis, episcleritis, scleritis, keratitis, and uveitis, dry eye, • Focal necrotizing scleritis with peripheral keratitis, Peripheral corneal subepithelial infiltrates may be associated and may develop into grainy anterior stromal opacities • corneal guttering may occur • MANAGEMENT • Systemic - oral Prednisone (1 mg/kg) and cyclophosphamide (2 mg/kg) orally are used for initial therapy, with the prednisone tapered after 2 to 4 weeks but continued over many months while the cyclophosphamide is continued for at least 1year • Alternatively, ‘pulsed’ IV methylprednisolone (500 mg to 1 g) may be used • Artificial tears for dry eyes and topical corticosteroids for mild conjunctivitis and episcleritis
MARGINAL KERATITIS • Catarrhal infiltrates or ulcers • Peripheral corneal disorder characterized by inflammatory infiltration that may lead to ulceration • Represent an antibody response to toxins rather than direct bacterial invasion • Associated with Staphylococcal infection, Wegener’s granulomatosis, soft contact lens, phthiriasis, topical antimicrobials, herpes simplex keratitis, Moraxella, Haemophilus, and other streptococcal species • Gram and Giemsa staining of corneal scrapings show neutrophils but no organisms • Pain, photophobia, FB sensation,
• Localized peripheral stromal infiltrates, (2, 4, 8, 10 o’clock positions) • Parallel to the limbus and separated from the limbus by 1–2 mm of clear cornea Infiltrates can coalesce Broader lesions Epithelial break down Ulceration • Spontaneous resolution in 2 to 3 weeks • Recurrences are common • MANAGEMENT • Topical corticosteroids (i.e. fluorometholone, loteprednol, and prednisolone acetate) are the mainstay in acute cases. • Ulceration - a broad-spectrum topical antibiotic should be used briefly prior to starting topical corticosteroids • Treatment of blepharitis, if present
HSV Marginal Keratitis STAPH Marginal Keratitis Etiology Infective Immunologic Epithelial defect Present Generally absent Progression Centrally Circumferentially Limbal Injection More Minimal Lucid interval Absent Present Neovascularization Present Absent Blepharitis Absent Present Location Any meridian 2, 4, 8, 10 O’clock
PHLYCTENULAR KERATOCONJUNCTIVITIS • Represent a bacterial allergy to a number of antigens - tuberculoprotein and staphylococcal. • Greek word phlyctena, which means ‘blister’ • 85% - positive to the tuberculin skin test • Other - Chlamydia, Coccidioides, Candida, HSV and parasites • In teenage years with a preference towards females • Type IV hypersensitivity reaction to a specific antigen and thus requires, – sensitization of the cornea or conjunctiva to antigen – repeated exposure • In TB-induced PKC, sensitization – bacteremia with a past pulmonary infection. Attack of PKC is - when TB antigens are presented to eye either from an endogenous/exogenous source • In Staph-induced PKC, sensitization - via many routes, the same bacterial antigens will be lingering locally to trigger a hypersensitivity attack.
• Subsequent corneal phlyctenules can arise at the central edge of a pannus from prior attacks. Multiple times - phlyctenule can appear to ‘wander’ across the cornea. • Multiple small phlyctenular lesions may be distributed diffusely over the entire corneal surface (i.e. miliary phlyctenulosis). • Histological - monocyte-derived cells with a moderate amount of T lymphocytes CONJUNCTIVAL LESION CORNEAL LESION Symptoms Tearing, FB sensation Same symptoms but with more intensity and photophobia Phlycten near the limbus, as pink fleshy nodule, 1-2mm diameter with conjunctival injection at the limbus, small white nodule with conjunctival injection Course of phylcten Central, superficial portion turns yellow or gray & soft, sloughs off, creating an ulcer, which then reepithelializes undergo necrosis, forming a marginal ulcer Resolution 1-2 weeks Triangular shaped anterior stromal scarring with broad pannus
• MANAGEMENT • Prednisolone acetate 1% - six times daily can be used as a starting dose. Tapering based on the clinical response. • A topical antibiotic is recommended - if a corneal lesion has an overlying epithelial defect • Conjunctival lesions can be treated right away with corticosteroids. • TB-induced PKC - treatment of the primary infection. • Minimizing Staphy colonization of lid margins - removal of bacteria with lid scrubs and the use of topical antibiotic ointments, Oral tetracyclines
MOOREN’S ULCER • A painful, progressive, chronic ulcerative keratitis that begins peripherally and progresses circumferentially and centrally. • An idiopathic disease occurring in complete absence of any diagnosable systemic disorder that could be responsible for the progressive destruction of the cornea. • The disease is strictly a peripheral ulcerative keratitis (PUK), with no associated scleritis • Between 40 and 70 years, M>F • Unilateral involvement - more common overall • Interpalpabral limbus – inferior - superior limbus • Association with hepatitis c, hookworm infestation Wood and Kaufman LIMITED/BENIGN/TYPICAL ATYPICAL/MALIGNANT Unilateral, Older patients Bilateral, Younger patients Mild – moderate symptoms Intense symptoms Responds well to medical and surgical treatment Poor response to therapy Progresses leading to perforation
Autoantigen - ‘cornea- associated antigen’, Trauma/infection may alter normal corneal antigens Taken up by macrophages move through the conjunctival vessels to the peripheral LNs T-cell activation, differentiation, and proliferation lymphocytes return to vessels and cause ocular inflammation corneal edema allows easier diffusion of immune components into pericentral from limbal vessels cornea is damaged, liberating more altered corneal antigens Aggravate the process until corneal stroma is completely destroyed PATHOGENESIS
• Cellular population found in the conjunctiva adjacent to the ulcer is primarily plasma cells, lymphocytes, and macrophages • Upregulation of various adhesion and co-stimulatory molecules in epithelial cells in the conjunctiva • Systemically, Mooren’s ulcer patients have a decrease in suppressor T cells relative to number of helper T cells, elevated IgA levels, and circulating IgG antibodies and immune complexes. • Molecular mimicry - infecting agent stimulating an autoimmune response to corneal antigens through cross-reacting epitopes • SYMPTOMS • Redness, tearing, photophobia • Pain – incapacitating, out of proportion to the inflammation. • Decreased VA, which may be secondary to associated iritis, central corneal involvement, or irregular astigmatism
• SIGNS • Concentric to limbus • Leading edges – undermined and deepithelialized • Conjunctiva, sclera - inflamed & hyperemic. • Spreads in 3 directions: peripherally, centrally, and rarely into sclera • Behind the advancing edge, healing may take place in the form of corneal epithelialization and vascularization • Associated with this is scarring and thinning • Advanced case - most of the cornea is lost, leaving behind a central island surrounded by area of grossly thinned, scarred, and vascularized tissue. Crescent-shaped graywhite infiltrate in peripheral cornea Epithelial breakdown & stromal melting Characteristic chronic crescent- shaped peripheral ulcer Progresses circumferentially & centrally, creating an overhanging edge at its central border Over time it may involve the limbus
• It is a diagnosis of exclusion • CBC, Platelet counts, ESR, RF, ANA, ANCA, CXR, LFT and fluorescent treponemal antibody absorption test. • TREATMENT : • Aim : Stop the ulcerative process and allow re-epithelialization of cornea • Topical corticosteroids - Aggressively on an hourly basis • Topical prophylactic antibiotics • Cycloplegic medications • Signs of reepithelialization - steroid therapy is tapered gradually over months. • Systemic Immunosuppression • Oral Prednisolone is 1–1.5 mg/kg body weight/day, adjusted according to the severity • Cyclophosphamide (2 mg/kg/ day), Methotrexate (7.5–15 mg once weekly), and Azathioprine (2 mg/kg/day), Ciclosporin A (3–4 mg/kg/day) • Topical Ciclosporin 0.5%, 4 to 6 times daily - penetrates corneal epithelium to reach the stroma
• Treating Chronic Hepatitis C patients - subconjunctival injections of interferon alpha- 2b over a 6-month period • SURGICAL PROCEDURES : • Conjunctival resection - Removes involved conjunctiva, adjacent to ulcer, up to 2 clock hours on either side and extends 3–4 mm from the limbus. • Keratoepithelioplasty : Ulcerated corneal tissue and adjacent conjunctiva are removed and donor corneal lenticules with intact epithelium are sutured onto bare sclera • Lamellar keratectomy : 4/5th of the corneal thickness is excised. Controls the inflammatory process by removal of the corneal antigenic stimulus • Lamellar keratoplasty - removal of necrotic ulcerative cornea and reconstruction of anatomical structure using lamellar donor lenticule • Tissue adhesive and BCL : In cases of perforation or impending perforation • Tectonic grafts (patch graft or PK) : Large perforations.
PUK MOOREN’S ULCER Pain Usually absent Intense Pain Scleral inflammation Present Absent Defect Leading edge with stromal WBC infiltration Undermined & overhanging edges Associated with systemic conditions Diagnosis of exclusion Clear zone Exists Doesn’t exist
SUPERIOR LIMBIC KERATOCONJUNCTIVITIS • An external ocular disorder characterized by a well- defined pattern of superior bulbar conjunctival injection • Between 20 and 60 years, Bilateral. • M:F=2:1 • Associated with thyroid dysfunction • The pathogenesis of SLK is unknown • Abnormal friction between the upper eyelid and the superior bulbar conjunctiva, combined with redundancy of the superior conjunctiva • Abnormal eyelid pressure on the globe, abnormal eyelid closure • SYMPTOMS - burning sensation, photophobia and FB sensation, feeling of ocular dryness and some will complain of redness
• SIGNS - Injection of the superior bulbar conjunctiva contiguous to the limbus, injection of palpebral conjunctiva • Fine punctate epitheliopathy – over superior 20% of cornea and bulbar conjunctiva, stains with fluorescein, rose bengal or lissamine green. • Filaments • Bulbar conjunctiva - folds, excessive movement & conjunctivochalasis • Micropannus involving the superior limbus, in conjunction with the ‘proliferation’ or ridge • Papillae, follicles, or scarring of the upper palpebral conjunctiva • Tests for tear dysfunction - evidence of lacrimal insufficiency • Histopathology – shows keratinization of epithelium with acanthosis, dyskeratosis, leukocytic infiltration.
• MANAGEMENT : • Swabbing with silver nitrate, Therapeutic contact lenses • Topical Cyclosporine 0.5% and 0.05%, Autologous serum, Vitamin A, Cromolyn sodium, lodoxamide tromethamine and Acetyl cysteine - beneficial. • Supratarsal Triamcinolone injection • Liquid nitrogen and Thermal cautery have been applied to both bulbar and palpebral conjunctiva • Simple scraping of palpebral conjunctiva was just as effective • Conjunctival resection, with and without AMT, Conjunctival advancement and Conjunctival fixation sutures – reduce redundancy and mobility of conjunctiva
CONTACT LENS INDUCED PERIPHERAL CORNEAL ULCER • RISK FACTORS : – Lens usage – overnight use – Acanthamoeba, Pseudomonas, Fusarium – Lens solution – Thiomersal – topping off of old solutions in the case – infrequent replacement of the storage case – failure to wash hands before handling lenses – exposure of the lens or lens case to tap water, including swimming or showering while wearing it – extended wear of any soft contact lens or a tight lens – acute or chronic hypoxia • SYMPTOMS : – moderate to severe pain – Sterile corneal infiltrates - mild discomfort – Active infection - Increasing pain • SIGNS : – Distinguishing noninfectious infiltrates from microbial keratitis – AC reaction, mucous discharge, an overlying epithelial defect - microbial
CORNEAL INFILTRATES INFECTIVE STERILE Size Larger Smaller Progression Rapid Slow Epithelial defect Very common – larger Less common – small if present Pain Moderate to severe Mild Discharge Purulent Mucopurulent Number of lesions Single Multiple AC Reaction Severe Mild Location Central Typically peripheral
• Acute hypoxia - Stromal & epithelial edema, no epithelial defect, mild AC reaction, with or without a hypopyon • Chronic hypoxia - conjunctival injection, corneal neovascularization, scarring, lipid keratopathy • Multiple peripheral subepithelial sterile corneal infiltrates, chronic or recurrent follicular conjunctivitis, superficial scarring • DIAGNOSIS – smears and cultures to rule infectious etiology • TREATMENT • Discontinuing lens wear until symptoms and signs resolve, Replacing the contact lenses, and switching to a hydrogen peroxide disinfection system • Refitting tight lenses and avoiding extended-wear lenses - if hypoxia • Microbial keratitis - intensive broad-spectrum antibiotic therapy - fluoroquinolones, topical fortified tobramycin and cefazolin or vancomycin • Sterile infiltrates - Low-dose antibiotics & steroids.
DEGENERATIVE DISORDERS WITHOUT CORNEAL THINNING
CORNEAL ARCUS • A degenerative change involving lipid deposition in peripheral cornea • ARCUS SENILIS : • Bilaterally symmetric, progressive • Prevalence increase with age • M>F, Black males • Gray to yellow arc, first in the inferior then the superior cornea • As the deposition progresses, the arcs meet - complete ring • Peripheral border - Sharp, Central edge - more diffuse • A lucent zone (lucid interval of Vogt) to the limbus, due to vessel’s ability to reabsorb the lipid in this area • Lipid is first deposited at Descemet’s membrane and subsequently at Bowman’s layer. • Gerontoxon • Aged population Arcus Senilis • Anterior Embryotoxon • Young population Arcus Juvenilis
• Histopathologically – an hourglass appearance as the opacity extends into the corneal stroma • Contains – cholesterol, cholesterol esters, phospholipids, and neutral glycerides • LDL cross the capillary wall endothelium of blood vessels, in the presence of elevated circulating LDL • Corneal arcus has no visual significance, and thus no necessary treatment • ARCUS JUVENILIS : • Premature corneal arcus formation • Hyperlipoproteinemia types IIa and IIb • Primary or secondary • Xanthelasma may be present • Nephrotic syndrome, hypothyroidism, increased cholesterol intake, obstructive jaundice, and diabetic ketoacidosis
LIPID KERATOPATHY • Primary form (rare) and secondary form (common) • F : M = 70 : 30 • Secondary from occurs in vascularized cornea - associated with interstitial keratitis, trauma, corneal hydrops, corneal ulceration • Primary form - no prior history of - trauma, family history of similar conditions, corneal vascularization, and no known disorders of lipid metabolism • Either central or peripheral • Yellow or cream deposit with intermittent crystals, in superficial and deep stroma • Fan out in association with the blood vessels • Contains - Cholesterol, neutral fats, phospholipids, free fatty acids, fibrin, and glycoprotein • MANAGEMENT : • Superficial argon laser • Lipid will resolve with subconjunctival Bevacizumab. • Progresses - penetrating keratoplasty
BAND KERATOPATHY • Term band keratopathy - Calcific form of keratopathy • Occurs in calcific and noncalcific forms • A corneal degeneration with deposition fine basophilic calcium hydroxyapatite or urate at the level of Bowman’s layer • Calcium deposits are white, while Urate deposits are brown. • Alteration of corneal metabolism - increased tissue pH and precipitation of calcium • Evaporation of the tears because of exposure - calcium precipitation • Early stages - asymptomatic. Later stages - decreasing vision, FB sensation, tearing • Begins at the periphery (3 and 9 o’clock) • Centrally in cases of chronic ocular inflammation.
• Peripheral form - sharp peripheral edge separated from limbus by a lucent zone • Central edge - fades into normal cornea • Gray opacity – white and chalky with Lucent holes • A complete band from limbus to limbus may form in later stages. • A medical work-up should include serum calcium, phosphorus, uric acid, and RFT • MANAGEMENT • Symptomatic - Application of Ethylene Diamine Tetraacetic acid (EDTA 0.05 molar concentration) • Amniotic membrane has been used after the primary surgical removal of band keratopathy Topical Anesthetic Epithelium removed EDTA applied to calcific areas Diamond burr or No. 15 blade is used to remove any residual calcium
SPHEROIDAL DEGENERATION • may occur in the cornea or the conjunctiva • Climatic droplet keratopathy, Hyaline degeneration, Labrador keratopathy • Primary corneal form •Secondary corneal form • Conjunctival form • Increases with age and varies with geographic location –high sunlight exposure and high winds. • M:F=2:1 • Drying of the cornea and repeated corneal trauma are also risk factors • Secondary forms - corneal scars, lattice dystrophy and glaucoma • Shows fine droplets, yellow or golden in color, beneath the epithelium • Droplets appear oily, although they are not of lipid origin
• Protein material, of unknown source - action of ultraviolet light on proteins from limbal vessels • Patients with visual loss may - superficial keratectomy • Lamellar or penetrating keratoplasty have also been described PRIMARY FORM GRADE 1 GRADE II GRADE III Fine shiny droplets Large corneal nodules Only peripherally Central cornea No symptoms vision ≥ 20/100 (6/30) vision ≤ 20/200 (6/60)
LIMBAL GIRDLE OF VOGT • A crescentic yellow-to-white band found in the interpalpebral limbus • Usually symmetric, occurring in nasal limbus more frequently • Incidence increases with age • Asymptomatic - No treatment is required. WHITE LIMBAL GIRDLE OF VOGT TYPE I – b/l WHITE LIMBAL GIRDLE OF VOGT TYPE II Narrow band of chalkywhite opacities and clear areas (Swisscheese) White fleck deposits - nasally and temporally Central border is relatively sharp Centrally - irregular linear extensions Separated from the limbus by a narrow lucent area No clear space Calcium deposits, superficially below epithelium In the limbal area peripheral to Bowman’s layer
HASSALL HENLE BODIES • excrescences of Descemet’s membrane found in peripheral cornea • Aging changes • Due to age related endothelial cell loss • Nodular areas of thickening of DM in the periphery extend into the anterior chamber • Best seen on specular reflection • Dark, round holes in the specular reflection • They are clinically insignificant – no treatment
DEGENERATIVE DISORDERS WITH CORNEAL THINNING
FURROW DEGENERATION • Rare degeneration • Elderly patients • Corneal thinning (shallow) within the clear band of cornea between the limbus and the corneal arcus • In Peripheral cornea under intact epithelium. • Not associated with vascularization, inflammation, progression or perforation • The thinning may be an illusion caused by the arcus senilis. True thinning also can occur. • Rarely, there is an astigmatic effect on vision that can be treated with refraction.
TERRIEN’S MARGINAL DEGENERATION • A rare peripheral corneal inflammatory condition with stromal thinning with a steep central wall and peripheral sloping • Unknown etiology • At any age, commonly between 20 and 40 years • M:F = 3 : 1 • Bilateral and symmetric • Begins superonasally with fine punctate opacities in anterior stroma • Superficial vascularization from the limbal arcades leading to lesion • Lucent area to the limbus • A gutter then forms between the opacity and limbus • Peripheral edge of the gutter gently slopes, whereas the central edge is often steeper • A yellow-white zone of lipid- at the advancing edge of the
• Gutter - vascularized and wider • Extend circumferentially or centrally • Quiescent type - older patients, asymptomatic for a long time • Inflammatory - younger age groups, recurrent episodes of inflammation, episcleritis, or scleritis • Astigmatism may be produced • Histopath - fibrillar degeneration of collagen. Bowman’s layer is fragmented or absent. Breaks in Descemet’s membrane may be seen in thinned areas • Perforation - spontaneously or secondary to minor trauma • MANAGEMENT • Astigmatism may be corrected with refraction. • Lamellar or eccentric penetrating grafts in cases of large astigmatism, impending perforation, or perforation
PELLUCID MARGINAL DEGENERATION • Peripheral corneal ectatic disorder characterized by a band of thinning 1– 2 mm in width, typically in the inferior cornea, extending from the 4 to 8 o’clock position • Atypical cases - >4 clock hours, superior location • 2nd – 5th decade, bilateral, M=F • Association with VKC, atopy, and frequent rubbing • Associated with keratoconus in the same or alternate eye. • Maximal corneal protrusion typically occurs just superior to the area of thinning - ‘beer belly’ contour • Shift in the axis of astigmatism from against-the rule, superiorly, to with- the-rule, near the point of maximal protrusion • Clear and avascular cornea, with no infiltrate or deposition • Stromal scars - at the level of Descemet’s extending into mid stroma
• Acute hydrops can occur - edema, scarring, and vascularization • Topography - classic crab-claw or C configuration - flattening of the central cornea with inferior peripheral steeping • Videokeratograph - superior flattening with against-the-rule astigmatism superiorly and with-the-rule astigmatism inferiorly • MANAGEMENT • Large diameter, rigid gas-permeable contact lenses can be tried • Hybrid lenses, such as the SoftPerm lens, have been used successfully • Poor candidates for surgery • Large-diameter or eccentric PK - to encompass the area of peripheral thinning • 2-stage procedure - large-diameter lamellar keratoplasty is followed by a smaller central PK • Alternative - Thermokeratoplasty, crescentic lamellar keratoplasty, and crescentic or wedge excision
DELLEN • Fuchs’ dimples • Age-related change or secondary to other ocular abnormalities • May last only 24 to 48 hours • Commonly in temporal periphery • Tissue thinning due to inadequate tear coverage & drying adjacent to a paralimbal elevation • Saucer-like areas of thinning or excavation in the corneal surface • Epithelium is usually intact, base can be gray or hazy • If Epithelium – not intact - infection and perforation are critical concerns. • Histopathologically - thinning of the epithelium, Bowman’s layer, and anterior stroma • Treatment with ocular lubricants or pressure patching will accelerate the healing process • After cataract extraction • Strabismus surgery • Glaucoma filtering surgey, filtering blebs • Episcleritis • Conjunctivitis • Pterygium/pingecula • Dermoids • Anesthestics - Cocaine • Lagophthalmos
NEOPLASTIC
DERMOID • Solid benign congenital tumor that frequently arise at the inferotemporal corneoscleral junction. • They are classified as choristomas • Contain ectodermal derivatives, such as hair follicles, sebaceous and sweat glands embedded in connective tissue and covered by squamous epithelium • Commonly as single lesions but may be multiple • Unilateral or bilateral • Yellowish-white, 2 to 15 mm in diameter, solid, vascularized, elevated nodules at the corneal limbus • Extend into deeper stroma without affecting Descemet’s & endothelium
• MANAGEMENT : • usually a cosmetic rather than a visual problem • Astigmatism - correction of refractive errors with spectacles • Drying of surrounding cornea by lifting of the lid during blinking - tumor should be cut flush with the corneal surface • Central cornea involved - penetrating and lamellar keratoplasty
BENIGN SQUAMOUS NEOPLASMS • Nonkeratinized stratified squamous epithelia of the cornea and conjunctiva are contiguous; therefore tumors arising within them are pathologically inseparable, but a transition occurs at limbus • Most conjunctival squamous epithelial tumors arise from limbus & spread to adjacent conjunctival and cornea • BENIGN HEREDITARY INTRAEPITHELIAL DYSKERATOSIS • AD, bilateral, first decade of life • Duplication of a gene on Chr 4 • V-shaped, hyperplastic, translucent, elevations that arise at limbus. Dilated vessels may be present • Biopsy - epithelial acanthosis, parakeratosis, and dyskeratosis, inflammatory cells in stroma. • Complete excision is the treatment of choice
• PSEUDOEPITHELIOMATOUS HYPERPLASIA • Benign, rapidly growing proliferation of conjunctival or corneal epithelium • In response to some preexisting inflammation such as a pterygium • A white elevated mass with hyperkeratotic surface. • A central umbilication may be present • Lateral margins of the lesion show a smooth transition into surrounding normal epithelium • Can mimic ocular surface neoplasia • Histopathologically – acanthosis which may be pushed downward into substantia propria, forming lobules of squamous cells with keratin whorls. • Precarcinomatous • Simple excision is usually curative
CONJUNCTIVAL INTRAEPITHELIAL NEOPLASIA • Neoplastic proliferation of dysplastic squamous epithelium that is noninvasive • Etiology - multifactorial – UV exposure, heavy smoking, light skin pigmentation, immunosuppression, HIV, HPV (6, 8,11- benign, 16,18-malignant), xeroderma pigmentosum • Mutation or deletions of the tumor suppressor gene p53 • 95% - at the limbus, nasal or temporal interpalpebral zone. • Slow-growing • 10% of lesions are leukoplakic • Most are translucent or gelatinous thickenings of the conjunctiva • Fine vascular pattern that has a hairpin configuration • Spreads to involve the adjacent corneal surface, anterior to Bowman’s layer.
• The abnormal epithelium has a frosted appearance with a fimbriated margin, supported by pannus • Primary corneal dysplasia & primary corneal epithelial dysmaturation - to describe cases in which corneal involvement is disproportionate • Histologically - partial- to full-thickness conjunctival epithelial dysplasia. BM is not penetrated • MANAGEMENT • Newly diagnosed lesions - excised with 1 to 2 mm of surrounding clinically uninvolved margins. The sclera is left bare. Corneal debridement, cryotherapy should be applied • Recurrent lesions - wide local excision but more aggressive cryotherapy • Deep tissue invasion - Cutaneous cryogun - full-thickness transscleral ice ball • Topical mitomycin-C - incompletely excised or recurrent lesion • Interferon-alfa, 5FU
INVASIVE SQUAMOUS CELL CARCINOMA • CIN – precursor • Occurs when dysplastic epithelial cells penetrate the underlying basement membrane & extend into subconjunctival space. • HPV is thought to play a role in pathogenesis • Lesions resemble those of CIN but are more elevated • Frequently at limbus - gelatinous, translucent, leukoplakic, or papilliform • Engorged feeder vessels - firmly fixed to underlying episcleral or scleral tissues • A frosted corneal epithelium and an extensive leukoplakic limbal component • Histopathologically – similar to severe CIN. BM - evidence of penetration with subepithelial neoplastic cells.
• MANAGEMENT : • Minimally invasive - wide local excision and aggressive cryotherapy. Often, superficial lamellar sclerectomy or keratectomy is required • Preop and postop adjunctive topical mitomycin-C • Large lesions – excision with the adjunctive use of AMT • Alcohol or cocaine - complete removal of the affected corneal epithelium • Intraocular invasion - enucleation, orbital invasion - exenteration
CONJUNCTIVAL MELANOMA • May arise from PAM, from pre-existing nevi, or de novo • Dark pigmented individuals • Atypical melanocytes invade from the epithelium into the substantia propria • Limbal/ Bulbar/ forniceal/ palpebral • Thickened, nodularity, dilator feeder vessels • May extend to cornea, lids, orbits • Cytologically - spindle-type cells or epithelioid-type cells • Spindle-shaped melanoma cells - amelanotic, highly invasive, with involvement of nerves and extension posteriorly into the orbit • MANAGEMENT : • Excision with clinically free margins & edges treated with double freeze thaw cryotherapy • Scleral adhesion – partial thickness sclerectomy • Corneal extension – removed by absolute alcohol keratoepitheliectomy • Intraocular – enucleation • Orbit - exenteration
REFERENCES • CORNEA – Fundamentals, Diagnosis, Management – Krachmer, Manns, Holland – 3rd edition • Copeland & Afshari’s Principles and Practice of Cornea • OPHTHALMOLOGY – Yanoff and Duker, 5th edition • Posr Graduate Ophthalmology – Zia Chaudhuri • Kanski’s Clinical Ophthalmology – 8th edition

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Peripheral corneal diseases

  • 1.
    PERIPHERAL CORNEAL DISEASES P R ES E N T E R – D R S A D H W I N I M H M O D E R ATO R – D R S H I VAY O G I R K U S A G U R
  • 2.
    PERIPHERAL CORNEA • Peripheralcornea - that portion located between the central 50% of the cornea and the limbus • Thickest region (upto 0.7mm)of the cornea • Avascular, a vascular arcade originating from the anterior ciliary arteries extends approx. 0.5 mm into clear cornea • Presence of more Langerhans’ cells • Higher concentrations of IgM and C1 (important in the activation of the classic complement pathway) • Its proximity to the limbus and conjunctiva results in a unique collection of abnormalities. • Thus, vascular inflammatory disorders, limbal infections, collagen vascular disorders, neoplastic disease, and local degenerations may affect the peripheral cornea in a distinctive way.
  • 3.
    PERIPHERAL CORNEAL DISEASES CONGENITAL /DEVELOPMENTAL /INHERITED Latticedystrophy type II Wilson’s disease Conea plana Sclerocornea Posterior embryotoxon Axenfeld-Rieger anomaly INFLAMMATORY /AUTOIMMUNE Rheumatoid Arthritis Polyarteritis Nodosa Wegener’s Granulomatosis Marginal keratitis Phlyctenular keratoconjunctivitis Mooren’s ulcer Superior limbic keratoconjunctivitis Contact lens induced DEGENERATIVE DISORDERS WITHOUT CORNEAL THINNING Corneal Arcus Lipid keratopathy Band keratopathy Spheroidal degeneration Limbal Girdle of VOGT Hassall Henle bodies DEGENERATIVE DISRDERS WITH CORNEAL THINNING Furrow degeneration Terrien’s Marginal degeneration Pellucid Marginal degeneration Dellen NEOPLASTIC Dermoid Benign squamous neoplasms Conjunctival Intraepithelial Neoplasia Invasive Squamous call carcinoma Conjunctival Melanoma
  • 4.
    C O NG E N I TA L / D E V E L O P M E N TA L / I N H E R I T E D C O N D I T I O N S
  • 5.
    LATTICE DYSTROPHY TYPEII • Meretoja’s syndrome or Familial Amyloid Polyneuropathy type IV • Associated with Systemic amyloidosis • Not a true corneal dystrophy • Corneal changes are later in onset • Favourable visual outcome • Mutations in the Gelsolin (GSN) gene, on Chr 9 (9q34) • OCULAR – CORNEA – fewer lattice line, radially oriented and involve primarily the peripheral cornea, with relative central sparing. – Reduced or absent corneal sensitivity. – Glaucoma and pseudoexfoliation with or without glaucoma are common
  • 6.
    • Systemic manifestations –Systemic amyloidosis – dermatochalasis, facial drooping along with protruding lips – typical facies. – Cranial and peripheral neuropathies such as facial paresis – Orthostatic hypotension, dysfunction of perspiration – Cardiac conduction abnormalities, carpal tunnel syndrome – Histopathology - mutated gelsolin deposited in arteries, skin, peripheral nerves, sclera, etc. A regular amyloid layer lies beneath a normal-appearing Bowman’s membrane. • Confocal microscopy – Stromal haze with highly reflective deposits - amyloid. – Pleomorphic basal epithelial cells, deposits seen just posterior to these cells.
  • 7.
    • MANAGEMENT • Dependson the patient’s symptoms • Recurrent erosions - patching, hypertonic agents, artificial tears, or a therapeutic contact lens • Excimer laser phototherapeutic keratectomy - optional treatment for recurrent erosions and superficial opacities
  • 8.
    WILSONS DISEASE • AR– Ch 13q14.3 • Accumulation of copper in most body tissues • Kayser-Fleischer ring is present in approximately 95% of patients – Yellow-brown or green ring located at the level of Descemet’s membrane in the peripheral cornea with no clear interval • Begins peripherally at Schwalbe’s line and progresses centrally. • Gonioscopy - to locate the ring in its early stage • Wilson’s disease - systemic D-penicillamine - prevents disease progression and reduces the amount of copper already deposited in tissues.
  • 9.
    CORNEA PLANA • ARor AD. Gene locus – KERA gene (12q22) • Cornea is flat - curvature <43D, typically 30-35D • Alteration in tertiary structure of keratan sulfate proteoglycans • Developmental arrest of corneal curvature relative to sclera • Associated with sclerocornea or microcornea • Angle closure glaucoma - 20% • Other ocular - Arcus juvenilis, nonspecific corneal opacities, anterior segment dysgenesis, uveal & retinal coloboma, aniridia, congenital cataract, ectopia lentis • Systemic – osteogenesis imperfecta, Ehler Danlos syndrome type IV • Management – correction of refractive error, monitor for associated findings
  • 10.
    SCLEROCORNEA • 50% -sporadicmutation, 50% - AD/AR/XLD • During embryonic development from 2nd mesenchymal wave forming tissue that resembles sclera instead of clear corneal stroma • May occur in peripheral cornea of one or both eyes, may be complete with an absence of clear cornea. • Commonly associated with cornea plana (80%) • Other ocular – Angle closure glaucoma, high refractive errors, blue sclera, Peter’s anomaly, uveal & retinal coloboma, cataract and microphthalmos. • Systemic – anomalies of skull & facial bones, external ear deformities, polydactyly, testicular abnormalities, osteogenesis imperfecta. • Management – few therapeutic options available – limited to control raised IOP, subsequent keratopalsty.
  • 11.
    POSTERIOR EMBRYTOXON • AD •Clear to white line in peripheral cornea – thickened & centrally displaced anterior border ring of Schwalbe • seen with gonioscopy as a fine ground-glass-like membrane • When posterior embryotoxon exists without additional pathology it does not require treatment
  • 12.
    AXENFELD – RIEGERANOMALY • AXENFELD’S ANOMALY - AD – Corneal clouding, posterior embryotoxon, prominent iris processes (iridocorneal adhesions to or beyond schwalbe’s line) – Glaucoma - 50% – Axenfeld’s syndrome – when associated with systemic anomalies – hypertelorism, facial asymmetry, hypoplastic shoulder • RIEGER’S ANOMALY – AD/sporadic – Atrophy of iris stroma and corneal abnormalities – PAS, corectopia, ectropion uveae, pseudopolycoria – Glaucoma - >50% – Rieger’s syndrome – when associated with skeletal, cranial & dental abnormalities
  • 13.
    • AXENFELD-RIEGER’S ANOMALY& SYNDROME • ANOMALY – Findings of both conditions – spectrum of anterior segment disorders • SYNDROME – When associated with systemic developmental defects • M Bruce Shields – primordial endothelium is retained over parts of iris and angle late in gestation. These undifferentiated cells contract – iris stromal thinning & hole formation. • MANAGEMENT – aqueous suppressants, goniotomy, trabeculectomy with antimetabolites
  • 14.
    I N FL A M M AT O R Y / A U T O I M M U N E C O N D I T I O N S
  • 15.
    RHEUMATOID ARTHRITIS • Achronic inflammatory disease of unknown etiology, affecting primarily synovial joints, and less frequently extra-articular tissues, such as the eye, pleura, pericardium, and nerves. • F>M • Between 4th and 6th decades • Presents as a symmetrical polyarthritis, involving small joints of the hands and feet, as well as larger joints • Presentation with ophthalmic disease - with severe, long-standing RA and high titers RF • Ocular manifestations – keratoconjunctivitis sicca, scleritis, sclerosing keratitis, keratolysis
  • 16.
    • Pathogenesis • Theetiopathogenesis of RA is unknown Immunoglob ulin & Complement deposition within blood vessel wall Focal areas of wall necrosis Rheumat oid Vasculitis Increase in permea bility causing leakage Chemotactic factors, Inflammatory cytokines (IL- 1, TNFα) Neutrophils recruited at peripheral cornea liberate proteolytic and collagenolytic enzymes Corneal destruction Immune mediated Hypersensit ivity Cell mediated Hypersensi tivity scleritis • High titers of rheumatoid factor - higher incidence of extra articular manifestations, more severe joint destruction, and worse prognosis
  • 17.
    • KERATOCONJUNCTIVITIS SICCA •most common associated (15–25% of RA patients) • gritty or sandy FB sensation, burning, irritation, mucus discharge, and photophobia • diminished tear meniscus, Schirmer test - decreased tear production • Punctate epithelial keratopathy, mucus stranding, and filamentary keratitis • SCLERITIS • 20% of RA patients, isolated episcleritis in up to 11% • severe boring ocular pain and photophobia • Anterior scleritis - diffuse, nodular, necrotizing scleritis with inflammation and necrotizing scleritis without inflammation, and posterior scleritis. • exhibit zones of intense scleral inflammation, edema, and necrosis with overlying epithelial defects • Also referred to as scleromalacia perforans - Severe scleral thinning diffuse anterior scleritis
  • 18.
    necrotizing scleritis postcataract suregery filamentary keratitis diffuse scleromalacia perforans in a 70-year-old patient with long-standing RA acute marginal stromal infiltrates PUK with scleritis, epithelial defect limbal guttering, absence of stromal infiltrate PUK
  • 19.
    • KERATITIS • PUK– crescent shaped peripheral corneal thinning, a leading edge of stromal WBC infiltration, epithelial defect overlying the ulceration, with scleral inflammation • SCLEROSING KERATITIS – adjacent to an area of active scleritis. • Peripheral cornea becomes thickened and opacified and may become secondarily vascularized • ACUTE STROMAL - as stromal opacities with edema. The opacities may coalesce if left untreated. Overlying epithelium may break down – PUK • LIMBAL GUTTERING - epithelium remains intact and the extent of peripheral thinning may be variable • KERATOLYSIS • an acute severe melting of the corneal stroma which can proceed to perforation. • most commonly seen in patients with rheumatoid-associated necrotizing scleritis • Loss of stroma occurs in regions of clear cornea and may progress to descemetocele formation. keratolysis
  • 20.
    • MANAGEMENT :therapy directed toward control of the systemic vasculitis • System disease : NSAIDs, corticosteroids and DMARDs • KCS : ocular surface lubrication – Moderate doses of topical steroids - underlying inflammatory component. – Topical ciclosporin A drops BD for management of dry eye have – Nutritional supplementation such as omega-3, omega-6, linoleic acid, linolenic acid, and other essential fatty – Punctal occlusion, Medial canthoplasty and/or lateral tarsorrhaphy can be performed • KERATITIS : Oral corticosteroid and topical ciclosporin A play a major role – Topical steroids - with great caution in furrowing with epithelial defects and sterile keratolysis – suture or chemical tarsorrhaphy - persistent epithelial defects – Keratolysis - a patch graft • NON NECROTIZING SCLERITIS : mild to moderate - include oral NSAIDs – Indometacin (25 mg) or ibuprofen (600 mg) TID or naproxen (500 mg) BD – Topical steroids can be used initially in combination with oral NSAID – Anterior scleritis - subconjunctival corticosteroid injections
  • 22.
    • NECROTIZING SCLERITIS: Infectious etiologies must be initially ruled out – Topical ciclosporin A, Protease inhibitors such as tetracyclines may be considered – Acute inflammation - oral prednisolone at an initial dose of 1 mg/kg/day, with a subsequent slower tapering – High-dose IV pulse methylprednisolone - 1 g per day for 3 consecutive days followed by oral therapy – Scleromalacia perforans - observation, eye protection, and therapeutic modulation of systemic disease • IMMUNOSUPPRESSIVES : in recalcitrant cases of scleritis or keratitis, including PUK, and keratolysis – Immunomodulators - antimetabolites, alkylating agents, T-cell inhibitors, and biologic agents. • SURGICAL MANAGEMENT : – Cyanoacrylate glue application with oversized BCL, donor scleral grafting, AMT, lamellar or penetrating keratoplasty, aggressive lubrication and tarsorrhaphy may enhance surgical success.
  • 24.
    POLYARTERITIS NODOSA • PeriarteritisNodosa - vasculitis of small and medium-sized arteries leading to multiple organ system disease • middle age, M>F • etiology is unknown • Ocular (10– 20%) - secondary to HTN or vasculitis of retinal vessels. – Direct nerve involvement - EOM palsies and optic nerve infarction – Orbital involvement - pseudotumor-like lesion – Conjunctival nodules - edema and necrosis of conjunctival vessels & uveitis • Anterior segment - scleritis, sclerokeratitis, and PUK weig ht loss ≥4 kg testic ular pain livedo reticul aris Myalg ia PMNs in artery walls in biopsy Mon/p olyneur opathy High BUN or Creatini ne HTN with DBP>90 mmHg HBSAg or Ab in serum • Treatment - systemic disease • Corticosteroids, Immunosuppressives, which have markedly improved the outcome ANY 3 OF,
  • 25.
    WEGENER’S GRANULOMATOSIS • Systemicvasculitis characterized by necrotizing and granulomatous inflammation of the vessels of the upper and lower respiratory tract, glomerulonephritis, and other organ involvement with small vessel inflammation. • Etiology is unknown • Mid-40s, M:F=2:1 • Can present in children, with ocular involvement as the initial manifestation • Ocular (58%) : – Proptosis with orbital pseudotumor or orbital inflammation, nasolacrimal duct obstruction – Retinal vasculitis and ischemic optic neuropathy Upper airway symptoms of sinusitis, Pulmonary infiltrates on CXR normocytic normochrom ic anemia Elevated ESR, positive RF Circulating immune complexes, ANCA Non specific complaint s Any 2 of 4
  • 26.
    Focal necrotizing scleritiswith peripheral keratitis Residual grainy anterior stromal opacities Peripheral anterior stromal and subepithelial infiltrates necrotizing scleritis
  • 27.
    • Anterior segment- conjunctivitis, episcleritis, scleritis, keratitis, and uveitis, dry eye, • Focal necrotizing scleritis with peripheral keratitis, Peripheral corneal subepithelial infiltrates may be associated and may develop into grainy anterior stromal opacities • corneal guttering may occur • MANAGEMENT • Systemic - oral Prednisone (1 mg/kg) and cyclophosphamide (2 mg/kg) orally are used for initial therapy, with the prednisone tapered after 2 to 4 weeks but continued over many months while the cyclophosphamide is continued for at least 1year • Alternatively, ‘pulsed’ IV methylprednisolone (500 mg to 1 g) may be used • Artificial tears for dry eyes and topical corticosteroids for mild conjunctivitis and episcleritis
  • 28.
    MARGINAL KERATITIS • Catarrhalinfiltrates or ulcers • Peripheral corneal disorder characterized by inflammatory infiltration that may lead to ulceration • Represent an antibody response to toxins rather than direct bacterial invasion • Associated with Staphylococcal infection, Wegener’s granulomatosis, soft contact lens, phthiriasis, topical antimicrobials, herpes simplex keratitis, Moraxella, Haemophilus, and other streptococcal species • Gram and Giemsa staining of corneal scrapings show neutrophils but no organisms • Pain, photophobia, FB sensation,
  • 29.
    • Localized peripheralstromal infiltrates, (2, 4, 8, 10 o’clock positions) • Parallel to the limbus and separated from the limbus by 1–2 mm of clear cornea Infiltrates can coalesce Broader lesions Epithelial break down Ulceration • Spontaneous resolution in 2 to 3 weeks • Recurrences are common • MANAGEMENT • Topical corticosteroids (i.e. fluorometholone, loteprednol, and prednisolone acetate) are the mainstay in acute cases. • Ulceration - a broad-spectrum topical antibiotic should be used briefly prior to starting topical corticosteroids • Treatment of blepharitis, if present
  • 30.
    HSV Marginal KeratitisSTAPH Marginal Keratitis Etiology Infective Immunologic Epithelial defect Present Generally absent Progression Centrally Circumferentially Limbal Injection More Minimal Lucid interval Absent Present Neovascularization Present Absent Blepharitis Absent Present Location Any meridian 2, 4, 8, 10 O’clock
  • 31.
    PHLYCTENULAR KERATOCONJUNCTIVITIS • Representa bacterial allergy to a number of antigens - tuberculoprotein and staphylococcal. • Greek word phlyctena, which means ‘blister’ • 85% - positive to the tuberculin skin test • Other - Chlamydia, Coccidioides, Candida, HSV and parasites • In teenage years with a preference towards females • Type IV hypersensitivity reaction to a specific antigen and thus requires, – sensitization of the cornea or conjunctiva to antigen – repeated exposure • In TB-induced PKC, sensitization – bacteremia with a past pulmonary infection. Attack of PKC is - when TB antigens are presented to eye either from an endogenous/exogenous source • In Staph-induced PKC, sensitization - via many routes, the same bacterial antigens will be lingering locally to trigger a hypersensitivity attack.
  • 32.
    • Subsequent cornealphlyctenules can arise at the central edge of a pannus from prior attacks. Multiple times - phlyctenule can appear to ‘wander’ across the cornea. • Multiple small phlyctenular lesions may be distributed diffusely over the entire corneal surface (i.e. miliary phlyctenulosis). • Histological - monocyte-derived cells with a moderate amount of T lymphocytes CONJUNCTIVAL LESION CORNEAL LESION Symptoms Tearing, FB sensation Same symptoms but with more intensity and photophobia Phlycten near the limbus, as pink fleshy nodule, 1-2mm diameter with conjunctival injection at the limbus, small white nodule with conjunctival injection Course of phylcten Central, superficial portion turns yellow or gray & soft, sloughs off, creating an ulcer, which then reepithelializes undergo necrosis, forming a marginal ulcer Resolution 1-2 weeks Triangular shaped anterior stromal scarring with broad pannus
  • 33.
    • MANAGEMENT • Prednisoloneacetate 1% - six times daily can be used as a starting dose. Tapering based on the clinical response. • A topical antibiotic is recommended - if a corneal lesion has an overlying epithelial defect • Conjunctival lesions can be treated right away with corticosteroids. • TB-induced PKC - treatment of the primary infection. • Minimizing Staphy colonization of lid margins - removal of bacteria with lid scrubs and the use of topical antibiotic ointments, Oral tetracyclines
  • 34.
    MOOREN’S ULCER • Apainful, progressive, chronic ulcerative keratitis that begins peripherally and progresses circumferentially and centrally. • An idiopathic disease occurring in complete absence of any diagnosable systemic disorder that could be responsible for the progressive destruction of the cornea. • The disease is strictly a peripheral ulcerative keratitis (PUK), with no associated scleritis • Between 40 and 70 years, M>F • Unilateral involvement - more common overall • Interpalpabral limbus – inferior - superior limbus • Association with hepatitis c, hookworm infestation Wood and Kaufman LIMITED/BENIGN/TYPICAL ATYPICAL/MALIGNANT Unilateral, Older patients Bilateral, Younger patients Mild – moderate symptoms Intense symptoms Responds well to medical and surgical treatment Poor response to therapy Progresses leading to perforation
  • 35.
    Autoantigen - ‘cornea- associatedantigen’, Trauma/infection may alter normal corneal antigens Taken up by macrophages move through the conjunctival vessels to the peripheral LNs T-cell activation, differentiation, and proliferation lymphocytes return to vessels and cause ocular inflammation corneal edema allows easier diffusion of immune components into pericentral from limbal vessels cornea is damaged, liberating more altered corneal antigens Aggravate the process until corneal stroma is completely destroyed PATHOGENESIS
  • 36.
    • Cellular populationfound in the conjunctiva adjacent to the ulcer is primarily plasma cells, lymphocytes, and macrophages • Upregulation of various adhesion and co-stimulatory molecules in epithelial cells in the conjunctiva • Systemically, Mooren’s ulcer patients have a decrease in suppressor T cells relative to number of helper T cells, elevated IgA levels, and circulating IgG antibodies and immune complexes. • Molecular mimicry - infecting agent stimulating an autoimmune response to corneal antigens through cross-reacting epitopes • SYMPTOMS • Redness, tearing, photophobia • Pain – incapacitating, out of proportion to the inflammation. • Decreased VA, which may be secondary to associated iritis, central corneal involvement, or irregular astigmatism
  • 37.
    • SIGNS • Concentricto limbus • Leading edges – undermined and deepithelialized • Conjunctiva, sclera - inflamed & hyperemic. • Spreads in 3 directions: peripherally, centrally, and rarely into sclera • Behind the advancing edge, healing may take place in the form of corneal epithelialization and vascularization • Associated with this is scarring and thinning • Advanced case - most of the cornea is lost, leaving behind a central island surrounded by area of grossly thinned, scarred, and vascularized tissue. Crescent-shaped graywhite infiltrate in peripheral cornea Epithelial breakdown & stromal melting Characteristic chronic crescent- shaped peripheral ulcer Progresses circumferentially & centrally, creating an overhanging edge at its central border Over time it may involve the limbus
  • 38.
    • It isa diagnosis of exclusion • CBC, Platelet counts, ESR, RF, ANA, ANCA, CXR, LFT and fluorescent treponemal antibody absorption test. • TREATMENT : • Aim : Stop the ulcerative process and allow re-epithelialization of cornea • Topical corticosteroids - Aggressively on an hourly basis • Topical prophylactic antibiotics • Cycloplegic medications • Signs of reepithelialization - steroid therapy is tapered gradually over months. • Systemic Immunosuppression • Oral Prednisolone is 1–1.5 mg/kg body weight/day, adjusted according to the severity • Cyclophosphamide (2 mg/kg/ day), Methotrexate (7.5–15 mg once weekly), and Azathioprine (2 mg/kg/day), Ciclosporin A (3–4 mg/kg/day) • Topical Ciclosporin 0.5%, 4 to 6 times daily - penetrates corneal epithelium to reach the stroma
  • 39.
    • Treating ChronicHepatitis C patients - subconjunctival injections of interferon alpha- 2b over a 6-month period • SURGICAL PROCEDURES : • Conjunctival resection - Removes involved conjunctiva, adjacent to ulcer, up to 2 clock hours on either side and extends 3–4 mm from the limbus. • Keratoepithelioplasty : Ulcerated corneal tissue and adjacent conjunctiva are removed and donor corneal lenticules with intact epithelium are sutured onto bare sclera • Lamellar keratectomy : 4/5th of the corneal thickness is excised. Controls the inflammatory process by removal of the corneal antigenic stimulus • Lamellar keratoplasty - removal of necrotic ulcerative cornea and reconstruction of anatomical structure using lamellar donor lenticule • Tissue adhesive and BCL : In cases of perforation or impending perforation • Tectonic grafts (patch graft or PK) : Large perforations.
  • 40.
    PUK MOOREN’S ULCER PainUsually absent Intense Pain Scleral inflammation Present Absent Defect Leading edge with stromal WBC infiltration Undermined & overhanging edges Associated with systemic conditions Diagnosis of exclusion Clear zone Exists Doesn’t exist
  • 41.
    SUPERIOR LIMBIC KERATOCONJUNCTIVITIS •An external ocular disorder characterized by a well- defined pattern of superior bulbar conjunctival injection • Between 20 and 60 years, Bilateral. • M:F=2:1 • Associated with thyroid dysfunction • The pathogenesis of SLK is unknown • Abnormal friction between the upper eyelid and the superior bulbar conjunctiva, combined with redundancy of the superior conjunctiva • Abnormal eyelid pressure on the globe, abnormal eyelid closure • SYMPTOMS - burning sensation, photophobia and FB sensation, feeling of ocular dryness and some will complain of redness
  • 42.
    • SIGNS -Injection of the superior bulbar conjunctiva contiguous to the limbus, injection of palpebral conjunctiva • Fine punctate epitheliopathy – over superior 20% of cornea and bulbar conjunctiva, stains with fluorescein, rose bengal or lissamine green. • Filaments • Bulbar conjunctiva - folds, excessive movement & conjunctivochalasis • Micropannus involving the superior limbus, in conjunction with the ‘proliferation’ or ridge • Papillae, follicles, or scarring of the upper palpebral conjunctiva • Tests for tear dysfunction - evidence of lacrimal insufficiency • Histopathology – shows keratinization of epithelium with acanthosis, dyskeratosis, leukocytic infiltration.
  • 43.
    • MANAGEMENT : •Swabbing with silver nitrate, Therapeutic contact lenses • Topical Cyclosporine 0.5% and 0.05%, Autologous serum, Vitamin A, Cromolyn sodium, lodoxamide tromethamine and Acetyl cysteine - beneficial. • Supratarsal Triamcinolone injection • Liquid nitrogen and Thermal cautery have been applied to both bulbar and palpebral conjunctiva • Simple scraping of palpebral conjunctiva was just as effective • Conjunctival resection, with and without AMT, Conjunctival advancement and Conjunctival fixation sutures – reduce redundancy and mobility of conjunctiva
  • 44.
    CONTACT LENS INDUCEDPERIPHERAL CORNEAL ULCER • RISK FACTORS : – Lens usage – overnight use – Acanthamoeba, Pseudomonas, Fusarium – Lens solution – Thiomersal – topping off of old solutions in the case – infrequent replacement of the storage case – failure to wash hands before handling lenses – exposure of the lens or lens case to tap water, including swimming or showering while wearing it – extended wear of any soft contact lens or a tight lens – acute or chronic hypoxia • SYMPTOMS : – moderate to severe pain – Sterile corneal infiltrates - mild discomfort – Active infection - Increasing pain • SIGNS : – Distinguishing noninfectious infiltrates from microbial keratitis – AC reaction, mucous discharge, an overlying epithelial defect - microbial
  • 45.
    CORNEAL INFILTRATES INFECTIVE STERILE Size LargerSmaller Progression Rapid Slow Epithelial defect Very common – larger Less common – small if present Pain Moderate to severe Mild Discharge Purulent Mucopurulent Number of lesions Single Multiple AC Reaction Severe Mild Location Central Typically peripheral
  • 46.
    • Acute hypoxia- Stromal & epithelial edema, no epithelial defect, mild AC reaction, with or without a hypopyon • Chronic hypoxia - conjunctival injection, corneal neovascularization, scarring, lipid keratopathy • Multiple peripheral subepithelial sterile corneal infiltrates, chronic or recurrent follicular conjunctivitis, superficial scarring • DIAGNOSIS – smears and cultures to rule infectious etiology • TREATMENT • Discontinuing lens wear until symptoms and signs resolve, Replacing the contact lenses, and switching to a hydrogen peroxide disinfection system • Refitting tight lenses and avoiding extended-wear lenses - if hypoxia • Microbial keratitis - intensive broad-spectrum antibiotic therapy - fluoroquinolones, topical fortified tobramycin and cefazolin or vancomycin • Sterile infiltrates - Low-dose antibiotics & steroids.
  • 47.
  • 48.
    CORNEAL ARCUS • Adegenerative change involving lipid deposition in peripheral cornea • ARCUS SENILIS : • Bilaterally symmetric, progressive • Prevalence increase with age • M>F, Black males • Gray to yellow arc, first in the inferior then the superior cornea • As the deposition progresses, the arcs meet - complete ring • Peripheral border - Sharp, Central edge - more diffuse • A lucent zone (lucid interval of Vogt) to the limbus, due to vessel’s ability to reabsorb the lipid in this area • Lipid is first deposited at Descemet’s membrane and subsequently at Bowman’s layer. • Gerontoxon • Aged population Arcus Senilis • Anterior Embryotoxon • Young population Arcus Juvenilis
  • 49.
    • Histopathologically –an hourglass appearance as the opacity extends into the corneal stroma • Contains – cholesterol, cholesterol esters, phospholipids, and neutral glycerides • LDL cross the capillary wall endothelium of blood vessels, in the presence of elevated circulating LDL • Corneal arcus has no visual significance, and thus no necessary treatment • ARCUS JUVENILIS : • Premature corneal arcus formation • Hyperlipoproteinemia types IIa and IIb • Primary or secondary • Xanthelasma may be present • Nephrotic syndrome, hypothyroidism, increased cholesterol intake, obstructive jaundice, and diabetic ketoacidosis
  • 50.
    LIPID KERATOPATHY • Primaryform (rare) and secondary form (common) • F : M = 70 : 30 • Secondary from occurs in vascularized cornea - associated with interstitial keratitis, trauma, corneal hydrops, corneal ulceration • Primary form - no prior history of - trauma, family history of similar conditions, corneal vascularization, and no known disorders of lipid metabolism • Either central or peripheral • Yellow or cream deposit with intermittent crystals, in superficial and deep stroma • Fan out in association with the blood vessels • Contains - Cholesterol, neutral fats, phospholipids, free fatty acids, fibrin, and glycoprotein • MANAGEMENT : • Superficial argon laser • Lipid will resolve with subconjunctival Bevacizumab. • Progresses - penetrating keratoplasty
  • 51.
    BAND KERATOPATHY • Termband keratopathy - Calcific form of keratopathy • Occurs in calcific and noncalcific forms • A corneal degeneration with deposition fine basophilic calcium hydroxyapatite or urate at the level of Bowman’s layer • Calcium deposits are white, while Urate deposits are brown. • Alteration of corneal metabolism - increased tissue pH and precipitation of calcium • Evaporation of the tears because of exposure - calcium precipitation • Early stages - asymptomatic. Later stages - decreasing vision, FB sensation, tearing • Begins at the periphery (3 and 9 o’clock) • Centrally in cases of chronic ocular inflammation.
  • 52.
    • Peripheral form- sharp peripheral edge separated from limbus by a lucent zone • Central edge - fades into normal cornea • Gray opacity – white and chalky with Lucent holes • A complete band from limbus to limbus may form in later stages. • A medical work-up should include serum calcium, phosphorus, uric acid, and RFT • MANAGEMENT • Symptomatic - Application of Ethylene Diamine Tetraacetic acid (EDTA 0.05 molar concentration) • Amniotic membrane has been used after the primary surgical removal of band keratopathy Topical Anesthetic Epithelium removed EDTA applied to calcific areas Diamond burr or No. 15 blade is used to remove any residual calcium
  • 53.
    SPHEROIDAL DEGENERATION • mayoccur in the cornea or the conjunctiva • Climatic droplet keratopathy, Hyaline degeneration, Labrador keratopathy • Primary corneal form •Secondary corneal form • Conjunctival form • Increases with age and varies with geographic location –high sunlight exposure and high winds. • M:F=2:1 • Drying of the cornea and repeated corneal trauma are also risk factors • Secondary forms - corneal scars, lattice dystrophy and glaucoma • Shows fine droplets, yellow or golden in color, beneath the epithelium • Droplets appear oily, although they are not of lipid origin
  • 54.
    • Protein material,of unknown source - action of ultraviolet light on proteins from limbal vessels • Patients with visual loss may - superficial keratectomy • Lamellar or penetrating keratoplasty have also been described PRIMARY FORM GRADE 1 GRADE II GRADE III Fine shiny droplets Large corneal nodules Only peripherally Central cornea No symptoms vision ≥ 20/100 (6/30) vision ≤ 20/200 (6/60)
  • 55.
    LIMBAL GIRDLE OFVOGT • A crescentic yellow-to-white band found in the interpalpebral limbus • Usually symmetric, occurring in nasal limbus more frequently • Incidence increases with age • Asymptomatic - No treatment is required. WHITE LIMBAL GIRDLE OF VOGT TYPE I – b/l WHITE LIMBAL GIRDLE OF VOGT TYPE II Narrow band of chalkywhite opacities and clear areas (Swisscheese) White fleck deposits - nasally and temporally Central border is relatively sharp Centrally - irregular linear extensions Separated from the limbus by a narrow lucent area No clear space Calcium deposits, superficially below epithelium In the limbal area peripheral to Bowman’s layer
  • 56.
    HASSALL HENLE BODIES •excrescences of Descemet’s membrane found in peripheral cornea • Aging changes • Due to age related endothelial cell loss • Nodular areas of thickening of DM in the periphery extend into the anterior chamber • Best seen on specular reflection • Dark, round holes in the specular reflection • They are clinically insignificant – no treatment
  • 57.
  • 58.
    FURROW DEGENERATION • Raredegeneration • Elderly patients • Corneal thinning (shallow) within the clear band of cornea between the limbus and the corneal arcus • In Peripheral cornea under intact epithelium. • Not associated with vascularization, inflammation, progression or perforation • The thinning may be an illusion caused by the arcus senilis. True thinning also can occur. • Rarely, there is an astigmatic effect on vision that can be treated with refraction.
  • 59.
    TERRIEN’S MARGINAL DEGENERATION •A rare peripheral corneal inflammatory condition with stromal thinning with a steep central wall and peripheral sloping • Unknown etiology • At any age, commonly between 20 and 40 years • M:F = 3 : 1 • Bilateral and symmetric • Begins superonasally with fine punctate opacities in anterior stroma • Superficial vascularization from the limbal arcades leading to lesion • Lucent area to the limbus • A gutter then forms between the opacity and limbus • Peripheral edge of the gutter gently slopes, whereas the central edge is often steeper • A yellow-white zone of lipid- at the advancing edge of the
  • 60.
    • Gutter -vascularized and wider • Extend circumferentially or centrally • Quiescent type - older patients, asymptomatic for a long time • Inflammatory - younger age groups, recurrent episodes of inflammation, episcleritis, or scleritis • Astigmatism may be produced • Histopath - fibrillar degeneration of collagen. Bowman’s layer is fragmented or absent. Breaks in Descemet’s membrane may be seen in thinned areas • Perforation - spontaneously or secondary to minor trauma • MANAGEMENT • Astigmatism may be corrected with refraction. • Lamellar or eccentric penetrating grafts in cases of large astigmatism, impending perforation, or perforation
  • 61.
    PELLUCID MARGINAL DEGENERATION •Peripheral corneal ectatic disorder characterized by a band of thinning 1– 2 mm in width, typically in the inferior cornea, extending from the 4 to 8 o’clock position • Atypical cases - >4 clock hours, superior location • 2nd – 5th decade, bilateral, M=F • Association with VKC, atopy, and frequent rubbing • Associated with keratoconus in the same or alternate eye. • Maximal corneal protrusion typically occurs just superior to the area of thinning - ‘beer belly’ contour • Shift in the axis of astigmatism from against-the rule, superiorly, to with- the-rule, near the point of maximal protrusion • Clear and avascular cornea, with no infiltrate or deposition • Stromal scars - at the level of Descemet’s extending into mid stroma
  • 62.
    • Acute hydropscan occur - edema, scarring, and vascularization • Topography - classic crab-claw or C configuration - flattening of the central cornea with inferior peripheral steeping • Videokeratograph - superior flattening with against-the-rule astigmatism superiorly and with-the-rule astigmatism inferiorly • MANAGEMENT • Large diameter, rigid gas-permeable contact lenses can be tried • Hybrid lenses, such as the SoftPerm lens, have been used successfully • Poor candidates for surgery • Large-diameter or eccentric PK - to encompass the area of peripheral thinning • 2-stage procedure - large-diameter lamellar keratoplasty is followed by a smaller central PK • Alternative - Thermokeratoplasty, crescentic lamellar keratoplasty, and crescentic or wedge excision
  • 65.
    DELLEN • Fuchs’ dimples •Age-related change or secondary to other ocular abnormalities • May last only 24 to 48 hours • Commonly in temporal periphery • Tissue thinning due to inadequate tear coverage & drying adjacent to a paralimbal elevation • Saucer-like areas of thinning or excavation in the corneal surface • Epithelium is usually intact, base can be gray or hazy • If Epithelium – not intact - infection and perforation are critical concerns. • Histopathologically - thinning of the epithelium, Bowman’s layer, and anterior stroma • Treatment with ocular lubricants or pressure patching will accelerate the healing process • After cataract extraction • Strabismus surgery • Glaucoma filtering surgey, filtering blebs • Episcleritis • Conjunctivitis • Pterygium/pingecula • Dermoids • Anesthestics - Cocaine • Lagophthalmos
  • 66.
  • 67.
    DERMOID • Solid benigncongenital tumor that frequently arise at the inferotemporal corneoscleral junction. • They are classified as choristomas • Contain ectodermal derivatives, such as hair follicles, sebaceous and sweat glands embedded in connective tissue and covered by squamous epithelium • Commonly as single lesions but may be multiple • Unilateral or bilateral • Yellowish-white, 2 to 15 mm in diameter, solid, vascularized, elevated nodules at the corneal limbus • Extend into deeper stroma without affecting Descemet’s & endothelium
  • 69.
    • MANAGEMENT : •usually a cosmetic rather than a visual problem • Astigmatism - correction of refractive errors with spectacles • Drying of surrounding cornea by lifting of the lid during blinking - tumor should be cut flush with the corneal surface • Central cornea involved - penetrating and lamellar keratoplasty
  • 70.
    BENIGN SQUAMOUS NEOPLASMS •Nonkeratinized stratified squamous epithelia of the cornea and conjunctiva are contiguous; therefore tumors arising within them are pathologically inseparable, but a transition occurs at limbus • Most conjunctival squamous epithelial tumors arise from limbus & spread to adjacent conjunctival and cornea • BENIGN HEREDITARY INTRAEPITHELIAL DYSKERATOSIS • AD, bilateral, first decade of life • Duplication of a gene on Chr 4 • V-shaped, hyperplastic, translucent, elevations that arise at limbus. Dilated vessels may be present • Biopsy - epithelial acanthosis, parakeratosis, and dyskeratosis, inflammatory cells in stroma. • Complete excision is the treatment of choice
  • 71.
    • PSEUDOEPITHELIOMATOUS HYPERPLASIA •Benign, rapidly growing proliferation of conjunctival or corneal epithelium • In response to some preexisting inflammation such as a pterygium • A white elevated mass with hyperkeratotic surface. • A central umbilication may be present • Lateral margins of the lesion show a smooth transition into surrounding normal epithelium • Can mimic ocular surface neoplasia • Histopathologically – acanthosis which may be pushed downward into substantia propria, forming lobules of squamous cells with keratin whorls. • Precarcinomatous • Simple excision is usually curative
  • 72.
    CONJUNCTIVAL INTRAEPITHELIAL NEOPLASIA •Neoplastic proliferation of dysplastic squamous epithelium that is noninvasive • Etiology - multifactorial – UV exposure, heavy smoking, light skin pigmentation, immunosuppression, HIV, HPV (6, 8,11- benign, 16,18-malignant), xeroderma pigmentosum • Mutation or deletions of the tumor suppressor gene p53 • 95% - at the limbus, nasal or temporal interpalpebral zone. • Slow-growing • 10% of lesions are leukoplakic • Most are translucent or gelatinous thickenings of the conjunctiva • Fine vascular pattern that has a hairpin configuration • Spreads to involve the adjacent corneal surface, anterior to Bowman’s layer.
  • 73.
    • The abnormalepithelium has a frosted appearance with a fimbriated margin, supported by pannus • Primary corneal dysplasia & primary corneal epithelial dysmaturation - to describe cases in which corneal involvement is disproportionate • Histologically - partial- to full-thickness conjunctival epithelial dysplasia. BM is not penetrated • MANAGEMENT • Newly diagnosed lesions - excised with 1 to 2 mm of surrounding clinically uninvolved margins. The sclera is left bare. Corneal debridement, cryotherapy should be applied • Recurrent lesions - wide local excision but more aggressive cryotherapy • Deep tissue invasion - Cutaneous cryogun - full-thickness transscleral ice ball • Topical mitomycin-C - incompletely excised or recurrent lesion • Interferon-alfa, 5FU
  • 74.
    INVASIVE SQUAMOUS CELL CARCINOMA• CIN – precursor • Occurs when dysplastic epithelial cells penetrate the underlying basement membrane & extend into subconjunctival space. • HPV is thought to play a role in pathogenesis • Lesions resemble those of CIN but are more elevated • Frequently at limbus - gelatinous, translucent, leukoplakic, or papilliform • Engorged feeder vessels - firmly fixed to underlying episcleral or scleral tissues • A frosted corneal epithelium and an extensive leukoplakic limbal component • Histopathologically – similar to severe CIN. BM - evidence of penetration with subepithelial neoplastic cells.
  • 75.
    • MANAGEMENT : •Minimally invasive - wide local excision and aggressive cryotherapy. Often, superficial lamellar sclerectomy or keratectomy is required • Preop and postop adjunctive topical mitomycin-C • Large lesions – excision with the adjunctive use of AMT • Alcohol or cocaine - complete removal of the affected corneal epithelium • Intraocular invasion - enucleation, orbital invasion - exenteration
  • 76.
    CONJUNCTIVAL MELANOMA • Mayarise from PAM, from pre-existing nevi, or de novo • Dark pigmented individuals • Atypical melanocytes invade from the epithelium into the substantia propria • Limbal/ Bulbar/ forniceal/ palpebral • Thickened, nodularity, dilator feeder vessels • May extend to cornea, lids, orbits • Cytologically - spindle-type cells or epithelioid-type cells • Spindle-shaped melanoma cells - amelanotic, highly invasive, with involvement of nerves and extension posteriorly into the orbit • MANAGEMENT : • Excision with clinically free margins & edges treated with double freeze thaw cryotherapy • Scleral adhesion – partial thickness sclerectomy • Corneal extension – removed by absolute alcohol keratoepitheliectomy • Intraocular – enucleation • Orbit - exenteration
  • 77.
    REFERENCES • CORNEA –Fundamentals, Diagnosis, Management – Krachmer, Manns, Holland – 3rd edition • Copeland & Afshari’s Principles and Practice of Cornea • OPHTHALMOLOGY – Yanoff and Duker, 5th edition • Posr Graduate Ophthalmology – Zia Chaudhuri • Kanski’s Clinical Ophthalmology – 8th edition

Editor's Notes

  • #3 Stems from the distinctive architecture of the limbus - a highly vascular zone with associated lymphatic tissue, scleral collagen, corneal collagen, and limbal stem cells.
  • #6 Gelsolin is an actin binding protein, thought to act as regulatory cytoplasmic and plasma protein. Mutated gelsoloin causes amyloid deposition. G to A substitution at nucleotide 654, resulting in a Asn-187 variant of gelsolin1 or a G to T transversion in position 654 at codon 187, resulting the substitution of tyrosine for aspartic acid. Recurrent erosions are unusual
  • #7 Reduction of long nerve fibre bundles – reduced sensitivity. The amorphous dots are fewer and more confined in distribution
  • #9 In serum, Cu binda to albumin, beta 1 globulin or ceruloplasmin (90%) Ocular Cu deposition – also due to retained intraocular Cu containing FB & prolonged Rx of trachoma with CuSO4 Pure Cu – turbulent exudative inflammatory reaction. Chalcosis – when Cu content of alloy is <85% - Cu deposits in iris, cornea, lens and vitreous
  • #11 Sclerization of cornea, lost transition between cornea & sclera Severe phenotype – vascularized opaque cornea, superficial vessels, cornea plana Milder phenotype - vascularized opaque cornea, superficial vessels, cornea plana, glaucoma, cataract, nystagmus
  • #18 secondary Sjögren’s syndrome also associated Diffuse anterior scleritis is the most common
  • #21 with artificial tear substitutes, fornicial lubricating inserts, and lubricating ointments. Topical application of 20–50% autologous serum prepared sterile in saline has also shown efficacy in ocular surface disease.
  • #23 oral prednisolone at an initial dose of 1 mg/kg/day, tapered rapidly to a 20 mg/day dosage over 2nd week, with a subsequent slower tapering
  • #26 nonspecific complaints of fever, malaise, joint and muscle pain, and weight loss
  • #29 WG - necrosis of the epithelium and superficial stroma, along with formation of chronic granulation tissue
  • #30 which tend to occur in locations where the lid margin crosses the limbus
  • #32 via many routes since staphylococcal colonization of the skin and mucous membranes is common
  • #33 Conj. Phlyctenules - but can occur anywhere on the bulbar conjunctiva This cycle of infiltration, ulceration, and resolution usually takes 1 to 2 weeks to complete.
  • #38 Perforation is uncommon
  • #40 Resection- blocks collagenase and the immune response to corneal antigen by providing a biological barrier
  • #47 contact lens keratopathy, manifested by superior corneal epitheliopathy preferably single-use daily disposable lenses are recommended Signs of delayed hypersensitivity or toxic reactions to contact lens solutions - peripheral, subepithelial opacities with conjunctival injection. Painful pseudodendrites, Ring infiltrates - Acanthamoeba keratitis
  • #49  Unilateral arcus may be seen with carotid artery occlusion on the side without the arcus Sharp peripheral border ending at the edge of Bowman’s layer
  • #50 secondary - increased levels of β-lipoproteins rich in cholesterol
  • #51 lipid is shown to be both extracellular and intracytoplasmic in histiocytes, Schwann cells, keratocytes, conjunctival epithelium, and the endothelial cells of blood vessels
  • #52 This zone is due either to the lack of Bowman’s layer at the periphery or from the buffering capacity of the limbal vessels, which prevent precipitation of calcium
  • #53 Lucent holes are scattered throughout the opacity and represent penetrating corneal nerves lesion is subepithelial but may break through the epithelium in advanced stages Calcium is extracellular in local disease versus intracellular with systemic disease EDTA will not penetrate the epithelium
  • #55 Histologically, deposits appear as extracellular amorphous globules
  • #60 may be asymmetric, with disease occurring in the second eye decades after the first Stroma progressively thins over many years
  • #61 lesion is superior, inducing against-therule astigmatism Epithelium may be normal, thick, or thinned
  • #62 Protruding cornea is of normal thickness
  • #63 rupture rarely occurs.
  • #70 perforation may occur - have corneal tissue available
  • #71 highly penetrant disorder
  • #73 i.e. confined to the epithelial layer by an intact basement membrane Leukoplakia refers to the white surface thickening
  • #74 a sharp demarcation between dysplastic and normal epithelium exists at the lateral edges of the lesion
  • #75 Due to the resistance of Bowman’s membrane, invasion occurs virtually exclusively within the conjunctival portion of the lesion may possess extensive pigmentation in pigmented patients. .