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 • 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 ’) 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

2. Rosacea keratitis

3. Phlyctenulosis

Circumferential and central Unlike Mooren ulcer 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. • 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 , lacrimation and blepharospasm 1. Bacterial keratitis Conjunctival phlycten Corneal phlycten 2.

3.

4. Infectious crystalline keratitis

5. • 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

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 • 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. • 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 • Bilateral • Usually 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 • 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 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 lesions Treatment Penetrating keratoplasty if severe Treatment - penetrating keratoplasty if severe Mask-like facies

Lattice dystrophy type III Granular dystrophy type I Inheritance - autosomal dominant Inheritance - autosomal dominant Onset - fourth decade Onset - first decade with recurrent corneal erosions

Progression

Initial superficial and Later deeper and Eventual confluence central crumb-like peripheral spread but opacities limbus spared • Thick, ropey lines and minimal intervening haze • May be asymmetrical and initially unilateral Treatment - penetrating keratoplasty if severe Treatment - penetrating keratoplasty if severe

Granular dystrophy type II Granular dystrophy type III (Avellino) Inheritance - autosomal dominant Inheritance - autosomal dominant Onset - fourth or fifth decade with mild recurrent erosions Onset - late in life; frequently asymptomatic

• Few, superficial, discrete, ring-shaped lesions Superficial, discrete crumb-like opacities • Increase in density and size with time Treatment - penetrating keratoplasty if severe Treatment - not required Macular dystrophy Fuchs endothelial dystrophy Inheritance - autosomal recessive Inheritance - occasionally autosomal dominant Onset - second decade with painless visual loss Onset - old age

Progression Progression

Gradual increase in Later central stromal Eventually bullous cornea guttata with oedema keratopathy Initial dense, poorly Later generalized Thinning peripheral spread delineated opacities opacification

Treatment - penetrating keratoplasty if advanced Treatment - penetrating keratoplasty

Posterior polymorphous dystrophy Inheritance - usually autosomal dominant CORNEAL ECTASIAS Onset - difficult to determine because asymptomatic

1.

2.

3. Pellucid marginal degeneration • Subtle, vesicular, geographic, or band-like lesions • Frequently asymmetrical Treatment - not required

Morphological classification of keratoconus Signs of keratoconus Bilateral in 85% but asymmetrical

Nipple cone Oval cone Globus cone

Oil droplet reflex Vogt striae Prominent corneal nerves

Bulging of lower lids on downgaze Small and steep curvature Larger and ellipsoidal Largest Fleischer ring & scarring Munson sign Acute hydrops Systemic associations of keratoconus Keratoglobus

Atopic dermatitis Down syndrome Ehlers-Danlos syndrome

• Onset usually at birth

• Bilateral protrusion and thinning of entire cornea Marfan syndrome Crouzon syndrome Osteogenesis imperfecta • Associations - Leber congenital amaurosis and blue sclera

Pellucid marginal degeneration CONGENITAL CORNEAL ANOMALIES

1. Microcornea

2. Megalocornea

3. Sclerocornea

4. Cornea plana

• Onset between 20 and 40 years 5. Keratectasia • Bilateral crescent-shaped inferior corneal thinning

Microcornea Megalocornea • Very rare, hereditary, unilateral or bilateral • Corneal diameter is 10 mm or less • Very rare, hereditary, • Shallow anterior chamber but other dimensions are normal bilateral • Corneal diameter 13 mm or more

• Very deep anterior chamber

• High and astigmatism

• Occasionally lens subluxation Systemic associations • Marfan, Apert, Ehlers-Danlos and Ocular associations Down syndromes , , cornea plana, leukoma and • Osteogenesis imperfecta abnormalities Associated systemic syndromes • Renal carcinoma and Turner, Ehlers-Danlos, Weill-Marchesani and mental handicap Waardenburg Sclerocornea Cornea plana • Very rare, bilateral severe decrease in corneal curvature • Very rare, usually bilateral • Hypermetropia and shallow anterior chamber • Peripheral opacification and vascularization of cornea • ‘Scleralization’ makes cornea appear smaller

Ocular associations Glaucoma, microcornea, microphthalmos and Peters anomaly

Keratectasia CORNEAL SURGERY • Very rare, usually unilateral • Severe corneal opacification and protruberance • Probably caused by intrauterine keratitis 1. Penetrating keratoplasty

2. Keratoprosthesis

3. Refractive surgery • Radial keratotomy • Photorefractive keratectomy (PRK) • Laser in-situ keratomileusis (LASIK) • Non-contact laser thermal keratoplasty

Penetrating Keratoplasty Technique of penetrating keratoplasty

1. Indications • Optical (e.g. bullous keratopathy, dystrophies) • Tectonic (e.g. severe stromal thinning, descemetocele) • Therapeutic (e.g. severe keratitis) • Cosmetic

2. Adverse prognostic factors • Severe stromal vascularization • Absence of corneal sensation • Progressive conjunctival inflammation (e.g. pemphigoid) a, b - Excision of host tissue Excision of donor tissue • Tear film dysfunction c - Fixation of donor tissue • Glaucoma Signs of late graft rejection Radial keratotomy Epithelial • Decreases myopia by flattening cornea

• Deep incisions from edge of optical zone to limbus

Linear epithelial opacitySubepithelial opacities Endothelial

Iritis and Endothelial precipitates inflammation at (Khodadoust line) Main indications Main complications graft-host junction Treatment • Stable myopia of up to 8D• Accidental perforation • Intensive topical and periocular steroids • Otherwise normal cornea • Intrastromal epithelial cysts • Occasionally systemic steroids

Photorefractive keratectomy ( PRK ) Laser in-situ keratomileusis (LASIK) Indications - similar to PRK but corrects higher degrees of myopia Indications Technique Complications • Stable myopia up to 6D with astigmatism no more than 3D • Hypermetropia up to 2.5D

Technique Main complication

• Wrinkles in flap • Thin flap of cornea fashioned Subepithelial haze which • Cellular interface proliferation Reshaping of cornea by excimer laser ablation of • Bed treated with excimer laser Bowman layer and anterior stroma usually resolves after 1-6 months • Flap repositioned

Non-contact laser thermal keratoplasty Indications • Patients over 40 years with hypermetropia up to 2D • Following overcorrection of myopia Red

• Corneal curvature is steepened by application of laser heat to stroma • Holmium laser spots applied to mid-cornea Basics Introdution – – Refers to hyperemia of the superficially visible vessels of • Relevance the , episclera, or the sclera – Caused by disorders of these structures themselves, or of – Red Eye adjacent structures like the , cornea, iris, and ciliary • Frequent presentation to GP body • Must be able to differentiate between serious vision threatening conditions and simple benign conditions

Differential diagnosis of red eye

• Conjunctival • – Blepharoconjunctivitis • – Bacterial • Masquerade syndrome – Viral conjunctivitis • Carotid and dural fistula – Chlamydial conjunctivitis • Acute angle glaucoma – – Toxic/chemical reaction • Anterior – Dry eye • /scleritis – /pteyrgium • Subconjunctival hemorrhage • Lid diseases • Factitious – Clalazion – Sty – Abnormal lid function • Corneal disease – Abrasion – Ulcer • Foreign body

Blepharitis

• Adults > children • Inflammation of the lid margin • Frequently associated with • Meibomian gland dysfunction • Lid hygiene, topical antibiotics, and lubricants are the mainstays of treatment Bacterial Conjunctivitis Viral Conjunctivitis – Acute, watery red eye with soreness, foreign body sensation and • Both adults and children photophobia • Tearing, foreign body sensation, burning, stinging and – Conjunctiva is often intensely hyperaemic and there maybe photophobia follicles, haemorrhages, inflammatory membranes and a pre- • Mucopurulent or purulent discharge auricular node • Lid and conjunctiva maybe edematous – The most common cause is an adenoviral infection – No specific therapy but cold compresses are helpful • Streptococcus pneumoniae, Haemophilus influenzae, and staphylococcus aureus and epidermidis • Conjunctival swab for culture • Topical broad spectrum antibiotics

Allergic Conjunctivitis – Encompasses a spectrum of clinical condition – All associated with the hallmark symptom of itching – There is often a history of rhinitis, asthma and family history of atopy – Signs may include mildly red , watery discharge, chemosis, papillary hypertrophy and giant papillae – Treatment consist of cold compresses, antihistamines, nonsteroidals, mast cells stabilizers, topical corticosteroids and cyclosporine

Chlamydial Conjunctivitis Dry Eye

– Usually occur in sexually active individuals with or • Symptoms without an associated genital infection – Burning or foreign body sensation – Tearing – Conjunctivitis usually unilateral with tearing, foreign – Usually bilateral body sensation, lid crusting, conjunctival discharge and • Etiology follicles – Idiopathic – There is often non-tender preauricular node – Collagen vascular diseases – Conjunctival scarring – Treatments requires oral tetracycline or azithromycin – Infiltration of the lacrimal gland – Vitamin A deficiency • Treatment – Artificial tears

Pterygium

Trichiasis Infectious keratitis

Corneal abrasion HSV dendrites Foreign Body Nasolacrimal Obstruction • Can lead to Dacryocystitis – Pain, redness, and swelling over the innermost aspect of the lower , tearing, discharge – Organisms – Staphylococci, streptococci, and diphtheoids – Treatment • Systemic antibiotics • Surgical drainage

Conjunctival tumor Iritis

Episcleritis Scleritis – Pain which maybe severe with tenderness, tearing and • Episcleritis photophobia – Can be localized (sectorial) or diffuse redness – Maybe localized, diffuse or associated with nodules – Often asymptomatic – Usually self limited – Can result in scleral necrosis (scleromalacia perforance) – 30 to 60 % may have an associated systemic diseases – Treatment is topical or systemic NSAIDs • RA ……… – May need systemic steroid Subconjunctival Hemorrhage Red Eye Treatment Algorithm • Usually asymptomatic • Blood underneath the conjunctiva, often in a sector of the eye • History • Etiology – Trauma – Valsalva (coughing or straining) – Contact lens wearer – Traumatic – Severe pain/photophobia – Hypertension – Significant vision changes – Bleeding disorder – History of prior ocular diseases – idiopathic YES

• Exam – Abnormal Refer urgently to – Ocular tenderness ophthalmologist – White – Increased intraocular pressure

Is it conjunctivitis? Thank you

• History • Signs – Itching – Discharge – Exposure to person – Lid and conjunctival with red eye edema – URTI – Conjunctival redness – Past history of – Preauricular lymph conjunctivitis node – Discharge with – Facial or eye lid morning crusting vesicles – Exposure to drugs