34. Corneal inflammation and surface disorders

Question 1

Corneal Anatomy
* Thickness = ...microns centrally
* Epithelium = ... cells thick, can therefore prevent diffusion of fluorescein
* ... membrane - significant in RCE
* .../ ... - cannot regenerate after trauma
* Stroma
* .../... - folds during oedema
* Endothlium = ... decreases with age and does not ...

Answer 1

Corneal Anatomy
* Thickness = 490-560microns centrally
* Epithelium = 6-8 cells thick, can therefore prevent diffusion of fluorescein
* Basement membrane - significant in RCE
* Anterior limiting lamina/ Bowman's - cannot regenerate after trauma
* Stroma
* Posterior limiting lamina/Descemet's - folds during oedema
* Endothlium = cell number decreases with age and does not regenerate

Question 2

Filamentary keratopathy is .... It arises due to the abonormal areas of the ... and excess ... in tears. The filaments arise from ... strands attached to abnormal ... which adhere strongly to the corneal surface. This is thought to be exacerbated by the ... in the components of the ... and/ or abnormalities of the ....
Filamentary keratopathy is associated with ..., ..., ..., ... and ....

Answer 2

Filamentary keratopathy is uncommon. It arises due to the abonormal areas of the corneal epithelium and excess mucous in tears. The filaments arise from mucous strands attached to abnormal epithelial cell plaques which adhere strongly to the corneal surface. This is thought to be exacerbated by the alteration in the components of the tear film and/ or abnormalities of the corneal surface.
Filamentary keratopathy is associated with severe dry eye, superior limbic kertoconjunctivitis, ocular Sx e.g. cataract, corneal graft, RCEs and neurotrophic keratopathy.

Question 3

**Filamentary keratopathy
**
Px symptoms:
* ...due to pulling on ... with blink
* ... due to stimulated ...
* Decreased ...
* ...

Clincial signs:
* ... filaments, several mm in length
* Stain well with ... & ..., but less well with ... but does pool around filaments
* Adherent to ... at proximal end of strand

Answer 3

**Filamentary keratopathy
**
Px symptoms:
* FB sensationdue to pulling on filaments with blink
* Watery eye due to stimulated reflex tears
* Decreased vision
* Photophobia

Clincial signs:
* Greyish filaments, several mm in length
* Stain well with Rose Bengal & Lissamine green, but less well with NaCl but does pool around filaments
* Adherent to corneal plaque at proximal end of strand

Question 4

Treatment of Filamentary Keratopathy
* Treat ... to prevent recurrences
* Removal of filaments under ... with ...
* Rx ... e.g.... *frequency* due to epithelila defect caused by remova l
* Rx ... for comfort and dry eyes e.g. ... *frequency*
* Rx ... e.g. ... *frequency* can help reduce inflammation and ...
* Rx ... = mucolytic agent used to ... however it is not available in Aus and is rarely used long term due to ... feeling
* Use of ... may be helpful in breaking cycle of epithelial damage and filament formation

Answer 4

Treatment of Filamentary Keratopathy
* Treat underlying conditions to prevent recurrences
* Removal of filaments under local anaesthetic with sterile forceps
* Rx topical antibiotics e.g.Chloramphenicol QID due to epithelila defect caused by remova l
* Rx ocular lubricants for comfort and dry eyes e.g. Hyloforte QID
* Rx topical corticosteroids e.g. FML BID can help reduce inflammation and filament formation
* Rx topical acetylcysteine = mucolytic agent used to dissolve filaments however it is not available in Aus and is rarely used long term due to stinging feeling
* Use of bandage CLs may be helpful in breaking cycle of epithelial damage and filament formation

Question 5

What are the 4 advices that should be given to a px with filamentary keratopathy?

Answer 5

• May take weeks to months before adequate resolution, depending on the etiology, severity and aggressiveness of care
• Underlying condition is often chronic and filaments may recur after therapy is discontinued
• Proper long-term care includes ongoing treatment for ocular surface disease with close monitoring e.g. 3-4 times annually
• Px with chronic or severe dry eye disease may benefit from a rheumatological investigation to determine the presence of Sjogren’s syndrome

Question 6

SPK stands for ..., also known as .... This is very ... and is caused by ... of the corneal .... There are a number of possible causes:
* ..., ... = Inferior
* ..., ... = Diffused
* ... = Tracking SPK
* ... = Central SPK
* ..., ...,..., ..., ... = Superior
* ...= 3 & 9 o’clock SPK
* ..., ..., ... = Interpalpebral

Answer 6

SPK stands for superficial punctate keratopathy, also known as punctate epithelial erosions. This is very common and is caused by superficial damage of the corneal epithelium. There are a number of possible causes:
* Lagophthalmos, Lid margin disease = Inferior
* Drug toxicity, Conjunctivitis = Diffused
* FB = Tracking SPK
* CL overwear = Central SPK
* Hypoxia, Tight lid,Vernal disease, Superior limbic keratoconjunctivitis, floppy eyelid syndrome = Superior
* Rigid CLs= 3 & 9 o’clock SPK
* Dry eye, Exposure, UV keratopathy = Interpalpebral

Question 7

What are the px symptoms and clinical signs of superficial punctate keratopathy?

Answer 7

Symptoms:
* May be asymptomatic
* If symptomatic: gritty, photophobia
* If dense may affect vision

Signs:
* Fine, coarse, dense or sparse areas of superficial corneal epithelial erosions
* No corneal infiltrates
* Eye remains white and clear

Question 8

What are the treatments and managements of SPK?

Answer 8

Treatment:
* Identify the cause and faciliate the recovery of the ocular surface
* Mild SPK = tear supplements e.g. Hyloforte QID prevent preservatives, BAK
* Severe SPK or full thickness = prophylatic topical antibiotics e.g. Chloramphenicol ointment QID for a week
* Cease CL wear until resolution

Management
* Mild SPK heals within 24 hours, no review required unless symptomatic
* Severe SPK or full thickness defect = review after 3 days or sooner if any worsening symptoms

Question 9

Subepithelial infiltrates are relatively ... and is caused by the .... These are ... areas of ... composed of ..., ... and .... They appear as ..., grey, roundish ... and can have ... overlying.
These are common caused by ..., ..., ..., ..., ... and ....
Symptoms of subepithellial infiltrates are:
* ...
* ...
* ...
* ...

Answer 9

Subepithelial infiltrates are relatively common and is caused by the inflammatory response within anterior cornea. These are focal areas of inflammation composed of neutrophils, cellular debris and protein. They appear as diffuse, grey, roundish opacities and can have epithelial defect overlying.
These are common caused by severe prolonged infection or inflammation, viral infection, blepharitis, Thygeson's SPK, preservative toxicity and contact lens wear.
Symptoms of subepithellial infiltrates are:
* FB sensation
* Photophobia
* Watery eye
* Decreased vision

Question 10

What are the treatments for subepithelial infiltrates?

Answer 10

• Depends on the underlying condition
• Rx topical corticosteroids with slow taper e.g. FML QID, reduced by one drop per week
• Rx ocular lubricants if symtomatic e.g. Hyloforte QID
• Rx topical antibiotics if significant epithelial breakdown over infiltrate e.g. Chloremphenicol (Chlorsig) QID for 1 week; if CL wearer use Tobramycin (Tobrex) QID

Question 11

How are sterile infiltrates and infectious infiltrates differentiated?

Answer 11

PEDALS = Pain, Epithelial defect, Discharge, Anterior chamber reaction, Location, Size
Infectious:
* Pain & photophobia
* Significant epithelial defect = size of staining = size of stromal lesion
* Mucopurulent discharge
* Anterior chamber reaction
* Lid edema, tear film debris, hypopyon
* More central/ mid-peripheral
* Larger lesion >1mm

Non-infectious:
* Minimal pain/ photophobia
* Partial/ intact epithelium
* No discharge
* No AC reaction
* Peripheral
* <1mm (staining smaller in size than infiltrate)

Question 12

Thygeson’s Superficial Punctate Keratopathy is ... and is usually a ... corneal ... condition of ... aetiology. The onset is greatest in the ... decade e.g. .... This is characterised by ... and ... of active phase. Active phase can last for ...months, followed by remission of up to ... months. Attacks tend to ... in frequency with ... and ... after many years. This often resolves without ... after 2-4 years, but may last up to ... years.
Px may experience ..., ..., ... and may have mild decrease in ....

Answer 12

Thygeson’s Superficial Punctate Keratopathy is rare and is usually a bilateral corneal inflammatory condition of unknown aetiology. The onset is greatest in the second decade e.g. young adults in early 20s. This is characterised by remissions and exacerbations of active phase. Active phase can last for 1-2months, followed by remission of up to 2 months. Attacks tend to decrease in frequency with age and cease after many years. This often resolves without sequelae/ consequences after 2-4 years, but may last up to 20 years.
Px may experience photophobia, tearing, FB sensation and may have mild decrease in vision.

Question 13

What are the 6 clinical signs of an active Thygeson’s SPK and 1 clinical sign of an inactive Thygeson’s?

Answer 13

Active phase:
* Raised epithelial lesions
* Negative or no fluorescein stain
* Can have faint epithelial haze - never scars
* Gives corneal surface an appearance of having coarse breadcrumbs on surface
* Mild conjunctival injection
* 1-2o lesiions most common presentation

Inactive phase:
* Lesion may disappear or leave flat epithelial grey area

Question 14

What are the treatments for Thygeson’s SPK?

Answer 14

• Rx ocular lubricants for discomfort e.g. Hyloforte, Hylofresh, Systane, Refresh
• Rx topical steroid during exacerbation + taper e.g. FML tapered over 1 month & monitor IOP
• Bandage CL to reduce symptoms if px uncomfortable
• Rx cyclosporine if px is a steroid responder e.g. Cequa 0.09%, Restasis 0.05%

Question 15

... (RCES) is a common disorder that involves repeated episodes of ... breakdown. This predisposes the cornea to .... The cause of RCES can be ..., ...(45-64%), ... and ....
Px may experience ..., unilateral ... upon waking.
Clinical signs include:
* ... - minor episodes that last ... but with an ...
* ... - more severe episodes that last for ..., with ... and an ....
* Px may have signs of underlying ... or ...
* ... with NaFl

Answer 15

Recurrent Corneal Erosions (RCES) is a common disorder that involves repeated episodes of corneal epithelium breakdown. This predisposes the cornea to infection. The cause of RCES can be idiopathic, corneal trauma(45-64%), posterior blepharitis and epidermolysis bullosa.
Px may experience sharp, unilateral pain upon waking.
Clinical signs include:
* Microform erosions - minor episodes that last 30 mins but with an intact epithelium
* Macroform erosions - more severe episodes that last for several days, with epithelial defects and an oedematous epithelium.
* Px may have signs of underlying anterior dystrophy or posterior blepharitis
* Negative staining with NaFl

Question 16

What is the 2nd most common cuase of RCEs? Briefly describe this condition.

Answer 16

Epithelial Basement Membrane Dystrophy (EBMD) = map-dot fingerprint dystrophy
* 10% of EBMD px experience RCE
* Grey chalky patches with intrepithelial cysts
* causes blurry VA, irregular astigmatism + negative staining

Question 17

What are the treatments for RCEs?

Answer 17

• Pain management: rest, cold compress, cycloplegia e.g. atropine 1% or cyclopentolate 1%, analgesics e.g. ibuprofen
• Rx lubrication e.g. Hyloforte QID or Vita-pos ointment every night = reduce friction & optimise tear film
• Rx bandage CL = reduce pain + help with healing
• Rx antbiotics e.g. FML QID
• More severe cases - Mechanical debridement, superifical keratectomy/ excimer laser phototherapeutic keratectomy (PRK/ PTK), alcohol delamination + oral tetracycline e.g. doxycycline

Question 18

Bandage contact lenses are fitted .... It aims to protect the ocular surface from the ... and provide pain relief. These CLs are made out of .... They are worn on a ... for ... weeks, which protects the epithelium white it ....
Examples include:
* J&J ... ...BC
* Alcon ... ...BC
* Coopervision ... ...BC

Answer 18

Bandage contact lenses are fitted loose/flat. It aims to protect the ocular surface from the shearing forces of the lids and provide pain relief. These CLs are made out of silicon hydrogel. They are worn on a continuous basis for 6-12 weeks, which protects the epithelium white it reattaches itself to the basement membrane.
Examples include:
* J&J Acuvue Oasys 8.8BC
* Alcon Night&Day 8.6BC
* Coopervision Biofinity 8.6BC

Question 19

Bullous keratopathy occurs due to ... causing .... This presents as epithelial of subepithelial ....
This is commonly caused by ..., ..., ..., ..., and contact lens wear.
Px may experience ..., ..., ... and ... around lights.
Clinical signs includes
* ...
* ... and ... in Descemet’s membrane
* ... changes - appears as roughened corneal surfaces
* ... - distinct raised areas with negative NaFl staining

Answer 19

Bullous keratopathy occurs due to endothelial decompensation causing stromal/ epithelial corneal oedema. This presents as epithelial of subepithelial bullae.
This is commonly caused by post-operative endothelial damage, endothelial dystrophy, trauma, angle closure glaucoma, and contact lens wear.
Px may experience pain, photophobia, decreased VA and haloes around lights.
Clinical signs includes
* stromal & epithelial corneal oedema
* corneal opacification and folds in Descemet’s membrane
* microcystic changes - appears as roughened corneal surfaces
* bullae - distinct raised areas with negative NaFl staining

Question 20

What are the 2 differential diagnosis of bullous keratopathy?

Answer 20

• Fuch’s dystrophy
• Post operative oedema usually from an undiagnosed pre-existing Fuch’s dystrophy

Question 21

What are the treatments for bullous keratopathy?

Answer 21

• Ocular lubricants e.g. Hyloforte QID
• Hypertonic saline e.g. NaCl 5% from compounding pharmacies
• Bandage CL for symptom relief
• Corneal graft Sx

Question 22

Interstitial keratitis is a rare condition resulting from ... without primary involvment of the .... This is an ...-mediated process triggered by an ....
Possible causes:
* Bacterial e.g. ..., ..., .... disease, ...
* Viruses e.g. ..., ...
* ... syndrome
* ...
Px may experience ..., ..., ..., reduced ... and increased ...

Answer 22

Interstitial keratitis is a rare condition resulting from inflammation of the corneal stroma without primary involvment of the corneal epithelium or endothelium. This is an immune-mediated process triggered by an antigen.
Possible causes:
* Bacterial e.g. syphilis, tuberculosis, Lyme disease, chlamydia
* Viruses e.g. HZO, HIV
* Cogan's syndrome
* Sarcoidosis
Px may experience pain, photophobia, ocular ache, reduced VA and increased tearing

Question 23

What are the 5 clinical signs of Interstitial keratitis?

Answer 23

• Salmon patch = limbitis + deep stromal vascularisation
• Granulomatous anterior uveitis
• Conjunctival injection
• Corneal haze
• Ghost vessels left in cornea after disease clears

Question 24

What are the treatments for interstitial keratitis?

Answer 24

• Rx potent topical steroids e.g. Prednisolone acetate 1% 1 drop every 2 hours with slow taper
• Oral antivirals if caused by HZV
• May req lab investigations

Question 25

Cogan syndrome is a rare systemic, bilateral, .... This syndrome highly associates with ... (80%). It affects ... (~...yo). It involves flares of ... and sudden onset of Meniere-like attacks = ....
Treatment involves ... for corneal involvment + systemic ... as soon as possible to reverse ....
40% of px develop ... manifestations:
* Life-threatening ...10%
* Necrotising ... of the ... systems 30%

Answer 25

Cogan syndrome is a rare systemic, bilateral, autoimmune vasculitis. This syndrome highly associates with interstitial keratitis (80%). It affects young adults (~25yo). It involves flares of interstitial keratitis and sudden onset of Meniere-like attacks = nausea, vomiting, tinnitus, vertigo and hearing loss.
Treatment involves topical steroids for corneal involvment + systemic steroids as soon as possible to reverse hearing loss.
40% of px develop systemic manifestations:
* Life-threatening aortic insufficiency10%
* Necrotising vasculitis of the renal, GIT, CV systems 30%

Question 26

Crystalline keratopathy is very rare. It has multiple causes: ..., ... or ..., which all result in a .... Infectious crystalline keratopathy can be ... or as a ... of Sx.
Clinical signs of crystalline keratopathy are .....
Px may experience ..., ... or ....
Px should be ... for urgent care and requires workup to establish .... First line treatment of infectious crystalline keratopathy is ....

Answer 26

Crystalline keratopathy is very rare. It has multiple causes: infection, corneal dystrophy or systemic diseases, which all result in a buildup of metabolic products in the cornea. Infectious crystalline keratopathy can be de novo or as a sequalae of Sx.
Clinical signs of crystalline keratopathy are crystals deposited in the corneal epithelium and/ or anterior stroma.
Px may experience pain, decreased vision or photophobia.
Px should be refered for urgent care and requires workup to establish etiology. First line treatment of infectious crystalline keratopathy is intensive topical antibiotics.

Question 27

Neurotrophic keratopathy is a ... disease characterised by ... reduction, spontaneous ..., and impairment of .... This can be caused by ..., ..., and ... that may damage the ... nerve.
Px rarely complain of symptoms due to the ....
Clinical signs include ... such as ..., ..., ... and .... The damage to ... sensory fibres also affect ... due to decreased stimulation of ....

Answer 27

Neurotrophic keratopathy is a degenerative disease characterised by corneal sensitivity reduction, spontaneous epithelium breakdown, and impairment of corneal healing. This can be caused by herpetic keratitis, diabetes, and ophthalmic and neurosurgical procedures that may damage the trigeminal nerve.
Px rarely complain of symptoms due to the lack of corneal sensation.
Clinical signs include corneal epithelial changes such as SPK, persistant epitheilal ulcers, stromal melting and corneal perforation. The damage to trigeminal sensory fibres also affect tear film production due to decreased stimulation of tear gland reflex.

Question 28

What are the treatments for neurotrophic keratopathy?

Answer 28

• Preservative-free artificial tears e.g. Hyloforte QID
• Topical steroids for inflammation
• Topical antibiotics with significant epithelial disruption
• Therapeutic CLs or Scleral Cls

Question 29

What are the 5 different peripheral corneal inflammatory disorders?

Answer 29

• Dellen
• Phyctenulosis
• PUK: Mooren’s ulcer
• Marginal keratitis
• Terrien’s marginal degeneration

Question 30

Dellen is a localised area of ... adjacent to the ..., along the .... They may be thinned to ... the corneal thickness. There may be local ... of the ..., which is primary to poor ...or .... They may also be secondary to ..., ..., ... e.g. ... or ....
Treatment includes topical ..., ... exercises, and ...if severe.

Answer 30

Dellen is a localised area of corneal thinning adjacent to the limbus, along the horizontal. They may be thinned to half the corneal thickness. There may be local dehydration of the stroma, which is primary to poor blinkingor tear film quality. They may also be secondary to scarring, neovascularisation, raised limbal lesion e.g. pinguecula or RGP wear.
Treatment includes topical lubricants, blinking exercises, and bandage CLs and patchif severe.

Question 31

Marginal keratitis is a common, ..., ... corneal lesion commonly associated with ... hypersensitivitiy, ..., and ....
Px may experience ..., discomfort, ..., ... and ....
Clinical signs include:
* ...separated from ... by a ...
* ... and ... of infiltrate
* ... with staining
* No ... reaction
* Adjacent ...

Answer 31

Marginal keratitis is a common, unilateral, peripheral corneal lesion commonly associated with staphylococcus exotoxin hypersensitivitiy, blepharitis, and acne rosacea.
Px may experience redness, discomfort, photophobia, water discharge and blurred vision.
Clinical signs include:
* Subepithelial marginal infiltrateseparated from limbus by a clear zone
* Coalescence and spread of infiltrate
* Epitheilal defect with staining
* No anterior chamber reaction
* Adjacent conjuncitval redness

Question 32

What are the treatments for marginal keratitis?

Answer 32

• Resolve in 3-4 weeks if untreated
• Rx topical corticosteroids e.g. FML 0.1% QID - to suppress immune response, then TID, BID, QD taper
• Rx topical antibiotics e.g. Choramphenicol 0.5% QID - to limit bacterial colonisation, cease once epithelium heals
• Treat underlying lid disease to prevent recurrence e.g. warm compresses, lid cleaners - Blephadex
• Review in 1/52 or sooner if uncertain

Question 33

Phlyctenulosis is an uncommon, ..., raised ... lesions, also known as .... This usually arises in .... It is caused by the ..., therefore can associate with ... blepharitis and ....
Px may experience ..., ..., ... and ....
Clinical signs:
* Begin as unilateral ... at ... margin at ...&... o’clock
* Intense local ... at ... or ...
* Can extend into ...
* Once healed can present as ... scar with ... and ...
* Blepharo...

Answer 33

Phlyctenulosis is an uncommon, unilateral, raised conjunctival/ corneal lesions, also known as phlycten. This usually arises in young children. It is caused by the delayed hypersensitivitiy to staphylococcus antigens, therefore can associate with staphylococcal blepharitis and tuberculosis.
Px may experience redness, discomfort, photophobia and watery discharge.
Clinical signs:
* Begin as unilateral nodule at limbal margin at 4&8 o’clock
* Intense local hyperaemia at conjunctiva or limbus
* Can extend into cornea
* Once healed can present as triangular scar with vascularisation and thinning
* Blepharospasm

Question 34

What are the treatments for phlyctenulosis

Answer 34

• Often spontaneous resolution in 2-3 weeks
• Topical steroids e.g. FML QID for 2 weeks + slow taper for 2 weeks to speed up healing
• If concerned for TB, refer for chest X-ray, mantoux and sputum culture
• Manage staphyloccal lid disease
• Review within 1 week

Question 35

Peripheral Ulcerative Keratitis (PUK) refers to a group of conditions that involve ... with thinning or ulcer. This is commonly caused by collagen or vascular diseases, such as ..., ..., ... and autoimmune diseases. ... ulcer is one of the autoimmune exacerbations.
Px may experience ..., irritation, ..., and redness
Clinical signs includes:
* ..., ..., tissue loss
* ... location
* Can have extensive ..., sometimes even ...
* Anterior chamber may have ...
* ..., ... or ...

Answer 35

Peripheral Ulcerative Keratitis (PUK) refers to a group of conditions that involve severe, persistent peripheral corneal infiltration with thinning or ulcer. This is commonly caused by collagen or vascular diseases, such as rheumatoid arthritis, Wegener granulomatosis, Lupus and autoimmune diseases. Mooren's ulcer is one of the autoimmune exacerbations.
Px may experience subacute onset increasing blur, irritation, photophobia, and redness
Clinical signs includes:
* Peripheral conreal ulceration, infiltration, tissue loss
* Paralimbal location
* Can have extensive tissue loss, sometimes even corneal perforation
* Anterior chamber may have inflammation
* Limbitis, episcleritis or scleritis

Question 36

What are the treatments for PUK?

Answer 36

• Urgent referral, may involve life-threatening systemic vasculitis
• Steroids, lubricants, antibiotics, Sx
• Lab investigation

Question 37

Mooren’s ulcer is extremely rare and is an ..., ... destructive condition of the .... Older px tend to have ... ulceration and respond ... to treatment; whereas younger px tend to have ... ulceration and respond ....
…. Clinical signs include ... and ... next to limbus which progressively spreads ... and ....

Answer 37

Mooren’s ulcer is extremely rare and is an idiopathic, autoimmune destructive condition of the peripheral cornea. Older px tend to have unilateral ulceration and respond well to treatment; whereas younger px tend to have bilateral ulceration and respond poorly to treatment.
Px may experience moderate to severe pain, conjunctival redness, photophobia, mucous/watery dischargeand blurred vision.
Clinical signs include ulceration and epithelial loss next to limbus which progressively spreads centrally and circumferentially.

Question 38

What are the treatments of Mooren’s ulcer?

Answer 38

• Urgent referral
• Topical steroids, propphylatic antibiotics, systemic immunosuppressants
• Corneal graft