Download - Peripheral Ulcerative Keratitis.Dr Ferdous
Peripheral Ulcerative Keratitis
Dr Md Ferdous IslamDepartment of Ophthalmology
CMH,Dhaka
Introduction• Peripheral Ulcerative Keratits (PUK) is a group
of inflammatory diseases whose final common pathway is peripheral corneal thinning
• Thinning and/or ulceration preferentially affecting the peripheral rather than the central cornea, and spreading around the margin
• It should be noted that any cause of corneal ulceration can affect the periphery
Types • Marginal keratitis• Mooren ulcer• Terrien marginal degeneration• Associated with systemic autoimmune
diseases• Dellen
Mooren Ulcer
• Rare autoimmune disease
• Characterized by– progressive, – peripheral, – circumferential, – stromal corneal ulceration – with later central spread
Forms
unilateral
•Older patient •Equal sex distribution•Slowly progressive•Responds well to medical therapy
bilateral
•younger patients•Indian males•more aggressive•likely to need systemic immunosuppression•poorer prognosis•associated with severe pain
Symptoms and signs
Peripheral ulcerationinvolving the superficial one-third of the stroma, variable epithelial loss,Several distinct foci and subsequently coalesce
Undermined and infiltrated leading edge is characteristic
Limbitis may be present but no scleritis
Progressive circumferential and central stromal thinning
Vascularization involving the bed of the ulcer up to its leading edge but not beyond
The healing stage is characterized by thinning, vascularization and scarring
Local peripheral ulceration
Undermining
Circumferential
Healing
Complications• Severe astigmatism due to extensive vascularization &
fibrosis
• Perforation following minor trauma
• Secondary bacterial infection
• Cataract
• Glaucoma
DDx
Mooren ulcer •not associated with any systemic abnormality, except for the occasional association with hepatitis C•No scleral involvement although associated conjunctival and episcleral inflammation •No clear zone exists between the ulcer and the limbus
PUK•associated with collagen vascular disease
Management• Topical – Steroids – Cyclosporin (weeks to show significant effect)– Artificial tears – Collagenase inhibitors (acetylcystine)
• bandage contact lenses may reduce discomfort and promote epithelial healing
• Conjunctival resection– may be combined with excision of necrotic tissue– performed if there is no response to topical steroids – The excised area should extend 4 mm back from the
limbus and 2 mm beyond the circumferential margins– Keratoepithelioplasty (suturing of a donor corneal
lenticule onto the scleral bed) may be combined to produce a physical barrier against conjunctival regrowth and further melting
– Steroids are continued postoperatively
Systemic
• Immunosuppression should be instituted earlier for – bilateral disease – advanced involvement at presentation
• Systemic collagenase inhibitors such as doxycycline• Lamellar keratectomy involving dissection of the
residual central island in advanced disease may remove the stimulus for further inflammation
Peripheral Ulcerative Keratitis Associated With Systemic Autoimmune Disease
• Destructive inflammation of the peripheral cornea associated with corneal epithelial sloughing and keratolysis
• The mechanism includes immune complex deposition in peripheral cornea, episcleral and conjunctival capillary occlusion with secondary cytokine release and inflammatory cell recruitment, the upregulation of collagenases and reduced activity of their inhibitors
Systemic associations
• Rheumatoid arthritis (most common)– PUK is bilateral in 30% and tends to occur in advanced RA
• Wegener granulomatosis (2nd most common)– In contrast to RA ocular complications are the initial presentation in
50%• Other conditions include polyarteritis nodosa, relapsing
polychondritis ,SLE , Churg – Strauss ,Microscopic Polyangiitis, Inflammatory Bowel Disease
Signs
Crescentic ulcerationwith an epithelial defect, thinning and stromal infiltration at the
limbusSpread is circumferential and occasionally centralin contrast to Mooren ulcer, extension into the sclera may occur
Limbitis, episcleritis or scleritis are usually presentContact lens corneaPerforationRheumatoid paracentral ulcerative keratitis (PCUK)
punched-out more centrally located lesion with little infiltrate in a quiet eye
Perforation can occur rapidly
Crescentic ulceration
Contact lens cornea
PCUK
Management
Principally with systemic immunosuppression in collaboration with a rheumatologist
TopicalArtificial tears (preservative-free)Antibiotics as prophylaxis Steroids may worsen thinning so are generally avoided
SystemicSteroids (via pulsed IV administration) are used to control
acute disease, with immunosuppressive therapy and biological blockers for longer-term management
Tetracycline for its anticollagenase effectSurgical management is generally as for Mooren ulcer, including
conjunctival excision if medical treatment is ineffective
Terrien Marginal Degeneration
• Idiopathic thinning of the peripheral cornea• Young adult to elderly patients• Uncommon• 75% males• Usually bilateral
Symptoms
• Asymptomatic • gradual visual deterioration can occur due to
astigmatism• A few patients experience episodic pain and
inflammation
Signs Fine yellow–white refractile stromal opacities, frequently associated
with mild superficial vascularization, usually start superiorly, spread circumferentially separated from the limbus by a clear zoneno epithelial defect, and on cursory examination the condition may
resemble arcus senilisSlowly progressive circumferential thinning
results in a peripheral gutter, the outer slope of which shelves gradually, while the central part rises sharply. A band of lipid is commonly present at the central edge
Perforation is rare but may be spontaneous or follow blunt traumaPseudopterygia sometimes develop
Pseudopterygium
Pseudopterygium vs Pterygium• Results from corneal burns,
perforation or longstanding corneal ulcer
• Differentiated by– Hx of prior inflammation– Unilateral– Location– Configuration other than the
wing shape– Nonprogressive– ability to pass a probe under
the neck
Management
• Safety spectacles if thinning is significant
• Contact lenses for astigmatism. Scleral or soft lenses with rigid gas permeable ‘piggybacking’
• Surgery – crescentic or annular excision of the gutter with lamellar or full-thickness transplantation
Marginal Keratitis
• Caused by hypersensitivity reaction against Staphylococcal exotoxin and cell wall proteins with deposition of Ag-Ab complex in peripheral cornea
• Leisons are culture negative but S. aureus can be isolated from lid margin
Signs and symptoms
• Chronic Blepheritis• Subepithelial marginal infiltrates seperated from
limbus by a clear zone• Conjunctival hyperemia• Coalescence and circumferential spread• Little or no AC reaction• Resolution usually occurs in 1-4 wks, occasionally
there is residual superficial scarring
Treatment
• Weak topical steroid• May be combined with a topical antibiotic• Tetracycline orally• For children , breastfeeding and pregnancy
erythromycin• Treatment of blepheritis
Dellen
• Localized corneal disturbance associated with drying of a focal area
• Usually associated with an adjacent elevated lesion as pinguecula or large subconjunctival haemorrhage that impairs physiological lubrication
Ref
• Kanski’s Clinical Ophthalmology A Systemic Approach by Brad Bowling. 8th Edition
• American Academy of Ophthalmology
Thank You