PERIPHERAL CORNEAL THINNING AND ULCERATION Corneal Pathologies 1. Without systemic disease • Dellen • Terrien marginal degeneration Dr. Pradeep Bastola • Mooren ulcer MD, Ophthalmologist Assistant Professor 2. With systemic disease 15th June, 2011 • Rheumatoid arthritis • Wegener granulomatosis • Polyarteritis nodosa • Common and unilateral Dellen • Innocuous Terrien marginal degeneration • Uncommon, bilateral but asymmetrical Signs Causes • Initially asymptomatic Progression • Saucer-like thinning with intact epithelium• Causes - chemosis, raised limbal lesions, • Fine stromal lipid deposition • Circumferential thinning and • Fluorescein pooling but no staining abnormal blinking separated by clear zone increasing astigmatism • Mild thinning and vascularization• Formation of pseudo-pterygia if longstanding Treatment - lubricants and elimination of cause Treatment of severe astigmatism - crescent-shaped excision of gutter Mooren ulcer Peripheral corneal involvement in rheumatoid arthritis • Limited form - usually unilateral, affects elderly • Progressive form - bilateral, affects younger patients Without inflammation With inflammation Progression Peripheral ulcerative keratitisCircumferential and centralEnd-stage scarring and • Chronic and asymptomatic • Acute and painful spread vascularization • Circumferential thinning with intact • Circumferential ulceration and epithelium (‘contact lens cornea’) infiltration Treatment - systemic steroids and/or cytotoxic drugs Treatment - systemic steroids and/or cytotoxic drugs Peripheral corneal involvement in PERIPHERAL CORNEAL INFLAMMATION Wegener granulomatosis and polyarteritis nodosa 1. Marginal keratitis 2. Rosacea keratitis 3. Phlyctenulosis Circumferential and central Unlike Mooren ulcer sclera may also 4. Acute stromal keratitis ulceration similar to Mooren ulcer become involved Treatment - systemic steroids and cyclophosphamide Marginal keratitis Rosacea keratitis • Hypersensitivity reaction to Staph. exotoxins • Affects 5% of patients with acne rosaeca • May be associated with Staph. blepharitis • Bilateral and chronic • Unilateral, transient but recurrent Progression Progression Subepithelial infiltrate Circumferential spread Bridging vascularization Peripheral inferior Subepithelial infiltration Thinning and perforation separated by clear zone followed by resolution vascularization if severe Treatment - short course of topical steroids Treatment - topical steroids and systemic tetracycline or doxycyline Phlyctenulosis • Uncommon, unilateral - typically affects children CORNEAL INFECTIONS • Severe photophobia, lacrimation and blepharospasm 1. Bacterial keratitis Conjunctival phlycten Corneal phlycten 2. Fungal keratitis 3. Acanthamoeba keratitis 4. Infectious crystalline keratitis 5. Herpes simplex keratitis • Small pinkish-white nodule • Starts astride limbus -Epithelial near limbus • Resolves spontaneously or extends • Usually transient and resolves onto cornea -Disciform spontaneously Treatment - topical steroids 6. Herpes zoster keratitis Bacterial keratitis Fungal keratitis Predisposing factors Frequently preceded by ocular trauma with organic matter • Contact lens wear • Chronic ocular surface disease • Corneal hypoaesthesia Greyish-white ulcer which may be Slow progression and occasionally surrounded by feathery infiltrates hypopyon Treatment Expanding oval, yellow-white, Stromal suppuration and • Topical antifungal agents dense stromal infiltrate hypopyon • Systemic therapy if severe Treatment - topical ciprofloxacin 0.3% or ofloxacin 0.3% • Penetrating keratoplasty if unresponsive Acanthamoeba keratitis • Contact lens wearers at particular risk Infectious crystalline keratitis • Symptoms worse than signs • Very rare, indolent infection (Strep. viridans) • Usually associated with long-term topical steroid use • Particularly following penetrating keratoplasty Small, patchy anterior Perineural infiltrates stromal infiltrates (radial keratoneuritis) Ulceration, ring abscess Stromal opacification White, branching, anterior stromal crystalline deposits & small, satellite lesions Treatment - chlorhexidine or polyhexamethylenebiguanide Treatment - topical antibiotics Herpes simplex epithelial keratitis Herpes simplex disciform keratitis Signs Associations • Dendritic ulcer with terminal bulbs• May enlarge to become geographic • Stains with fluorescein Treatment • Central epithelial and stromal oedema • Occasionally surrounded by Wessely ring • Aciclovir 3% ointment x 5 daily • Folds in Descemet membrane • Trifluorothymidine 1% drops 2-hourly • Small keratic precipitates • Debridement if non-compliant Treatment - topical steroids with antiviral cover Herpes zoster keratitis Acute stromal keratitis Acute epithelial keratitis Nummular keratitis • Uncommon, usually unilateral • Associated with non-necrotizing scleritis Progression • Develops in about 50% within • Develops in about 30% within 2 days of rash 10 days of rash • Small, fine, dendritic or stellate• Multiple, fine, granular deposits epithelial lesions just beneath Bowman membrane • Tapered ends without bulbs • Halo of stromal haze Superficial or mid-stromal infiltration • Resolves within a few days • May become chronic Opacification and vascularization Treatment - topical steroids, if appropriate Treatment - topical steroids and systemic NSAIDs CORNEAL DEGENERATIONS Arcus senilis • Innocuous and extremely common in elderly 1. Age-related • Occasionally associated with hyperlipoproteinaemia • Arcus senilis • Vogt white limbal girdle • Crocodile shagreen • Cornea guttata 2. Lipid keratopathy • Primary • Secondary 3. Band keratopathy • Bilateral, circumferential bands • Peripheral border separated of lipid deposits from limbus by clear zone • 4. Spheroidal degeneration Diffuse central and sharp • Clear zone may be thinned peripheral border 5. Salzmann nodular degeneration ( senile furrow) Vogt white limbal girdle Crocodile shagreen • Innocuous and very common in elderly • Uncommon and innocuous • Usually bilateral • Bilateral • Polygonal stromal opacities separated by clear space • White, crescentic line along nasal and temporal • Most frequently involve anterior stroma limbus (anterior crocodile shagreen) • Type 1 - separated from limbus by clear zone • Occasionally involve posterior stroma • Type 2 - not separated by clear zone (posterior crocodile shagreen) Cornea guttata • Common, bilateral and usually innocuous Causes of Band Keratopathy • Rarely progression to Fuchs dystrophy 1. Ocular (common) • Chronic iridocyclitis, particularly in children • Associated with phthisis bulbi • Silicone oil in anterior chamber 2. Metabolic (rare) • Increased serum calcium and phosphorus • Hyperuricaemia • Chronic renal failure 3. Hereditary (rare) • Familial band keratopathy • Hereditary ichthyosis • Tiny dark spots on central endothelium • Similar peripheral lesions are Hassell-Henle bodies 4. Age-related (uncommon) Spheroidal degeneration Chelation of band keratopathy • Rare, typically affects outdoor workers • Starts with peripheral, interpalpebral, small amber-coloured granules in superficial stroma Progression Central spread, coalescence and Advanced lesions become opacification nodular and elevated Removal of corneal epithelium Application of sodium versenate Treatment • Debridement or superficial keratotomy if mild • Keratoplasty if severe Salzmann nodular degeneration Spheroidal degeneration • Uncommon, unilateral or bilateral • Rare, typically affects outdoor workers • Secondary to chronic keratitis • Starts with peripheral, interpalpebral, small amber-coloured granules in superficial stroma Progression Central spread, coalescence and Advanced lesions become • Discrete superficial stromal opacities opacification nodular and elevated and nodules Treatment • Base of nodule may be surrounded by iron deposits • Debridement or superficial keratotomy if mild Treatment - similar to spheroid degeneration • Keratoplasty if severe CORNEAL DYSTROPHIES Cogan microcystic dystrophy 1. Anterior • Most common of all dystrophies • Cogan microcystic • Neither familial nor progressive .. • Recurrent corneal erosions in about 10% of cases • Reis-Bucklers • Meesmann • Schnyder Dots Cysts 2. Stromal • Lattice I, II, III • Granular I, II, III (Avellino) • Macular 3. Posterior • Fuchs endothelial • Posterior polymorphous Fingerprints Maps Signs of Cogan dystrophy .. Four types of lesions - in isolation or combination Reis-Bucklers dystrophy Dots Microcysts Inheritance - autosomal dominant Onset - early childhood with recurrent corneal erosions Maps Fingerprints Superficial polygonal Honeycomb appearance opacities Treatment - keratoplasty if severe Meesmann dystrophy Schnyder dystrophy Inheritance - autosomal dominant Inheritance - autosomal dominant Onset - second decade with visual impairment Onset - first decade with mild visual symptoms • Tiny, epithelial cysts, maximal centrally • Clear in retroillumination • Grey in direct illumination Subepithelial ‘crystalline’ opacities Treatment - not required Treatment - excimer laser keratectomy Lattice dystrophy type I Lattice dystrophy type II Inheritance - autosomal dominant (Familial amyloidosis with lattice dystrophy, Meretoja syndrome) Onset - late first decade with recurrent corneal erosions Progression Inheritance Autosomal dominant Onset Middle age with progressive facial palsy and lattice dystrophy identical to type I Systemic features • Cranial and peripheral neuropathy • Skin laxity • Renal and cardiac failure Fine, spidery, branching lines within Later general haze may submerge stroma