ANTERIOR SEGMENT Flashcards

(266 cards)

1
Q

What stain is used for macular dystrophy?

A

Alician blue

MMA - GHM - LAC (marilyn monroe always gets her man in LA City, She likes Obesity.)

M - mucopolysaccharide (GAG)
M - macular dystrophy
A - Alicia blue

G - granular dystrophy
H - hyaline materials
M - Mason trichrome

L - Lattice dystrophy
A - Amyloid
C - Congo Red

S - Schnyder’s dystrophy
L - Lipids
O - Oil-Red Stain

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2
Q

What are the clinical features of macular dystrophy?

A
  1. Diffuse grey-white opacities across limbus to limbus (axial) with diffuse clouding and corneal thinning
    MACULAR
    Misty stromal haze
    Autosomal recessive: CHST6 mutation
    Central → peripheral spread/Corneal guttata present
    Uniform diffuse opacity
    Limbus involved ⭐
    Attenuated thickness (thin cornea)/ Alician Blue staining
    Reduced vision early

Mucopolysaccharide deposits: Alician blue positive

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3
Q

What is the treatment of acute hydrops?

A

ABCDK
A - Aqueous suppressants / air (SF6/C3F8)
B - BCL
C - Cycloplegics
D - Drops (steroids and hypertonic saline
K - Keratoplasty (PKP/DALK)

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4
Q

What are the causes of corneal hydrops? (5)

A

Advanced ectatic corneal diseases
1. Keratoconus
2. Keratoglobus
3. Pellucid marginal degeneration
4. Post LASIK ectasia (rare)
5. Leber’s congenital amaurosis (association)

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5
Q

What is the prevalence of map-dot fingerprint dystrophy?

A

2.5% - most common corneal dystrophy

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6
Q

What are the symptoms of map-dot fingerprint dystrophy?

A

Bilateral asymptommatic
Recurrent corneal erosions
Fluctuating blurred vision due to irregular astigmatism

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7
Q

What are the clinical features of map dot fingerprint dystrophy?

A
  1. Bilateral greyesh Epithelial maps/opacities (faint opacities)
  2. Dots/microcysts
  3. Whorled Fingerprints/curvilinear ridges
  4. Negative flourescein staining - where tear film breaks up over epithelial ridges
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8
Q

What is the management of map dot fingerprint dystrophy

Acute, Recurrent, Refractory

A

Acute erosion: Lubricants, hypertonic saline for corneal oedema and to improve epithelial adhesion, antibiotic ointment, cycloplegia, BCL.

Recurrent/persistent: oral doxycycline + topical steroids (to reduce MMP activity)

Refractory:
1. Anterior stromal puncture (usually for post-traumatic cases, not used centrally to to possibility of scarring)

  1. superficial keratectomy (allows for more uniform regrowth with alcohol delamination)
  2. Diamond Burr polishing - smoothen Bowmen’s membrane and promote better adhesion
  3. PTK: excismer laser treatment to remove superficial pathology and provide smooth surface for epithelial basement membrane attachment
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9
Q

What are the typical slit lamp findings of thygeson’s superficial punctate keratitis?

A
  1. Multiple (up to 40) elevated corneal epithelial infiltrates demonstrating negative staining with flourescein
  2. Small white, round oval grey/white opacities which wax and wane and change in location over time in the central cornea.
  3. Minimal/no conjunctival reaction
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10
Q

What is the management of thygeson’s superficial punctate keratopathy?

A

Treat: Weak steroids (FML), therapeutic CL.
Maintenance: Steroids

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11
Q

What are the indications of DSAEK? (4)

A

Clear stroma.
1. Endothelial dystrophy (FED, PPCD) and failure
2. Bullous keratopathy
3. ICE syndrome
4. Failed PK

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12
Q

What are the DSAEK contraindications?

A
  1. Anterior stromal opacity
  2. Keratoconus/ectasia
  3. Hypotony
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13
Q

What are the clinical features of acanthomoeba keratitis?

A
  1. Pain is out of proportion to signs
  2. Early: Epithelial ridges - flat diffuse microcysts with perilimbal sparing; pseudodendrites –> can be dendritic –> suspect in contact lens wearers with dendritic ulcer
  3. Late: Radial keratoneuritis, later paracentral ring stromal infiltrate
  4. Scleritis, uveitis
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14
Q

What are the investigations of a patient with acanthomoeba keratitis?

A

History:
- Disproportioning ocular pain to clinical findings
- Contact lens wear, poor CL hygeine, swim, sleep, shower,
- Trauma: Agricultural or rural setting

Ophthalmology
Scraping: Culture on non-nutrient agar with E coli overlay, DNA detection using PCR

Staining: Calcufluor white (stains cysts visualised under UV light) or gram staining

CL contact lenses: Send lenses, solutions for culture

Other: Invivo confocal microscopy: Direct visualisation of cysts (high contrast round bodies)

Corneal biopsy: If tests negative

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15
Q

What is the treatment of acanthomoeba keratitis?

A

Conservative
1. Admit with daily review, stop CL wear

Medical
1. Biguanides: PHMB 0.02% or chlorhexidine 0.02%
2. Diamidines: Hexamidine 0.1%
3. Cycloplegics
4. Scleritis: oral steroids +/- steroid sparing agents

Surgical
1. PK - after 3 months of discontinuation of anti-acanthomoeba medications
2. Extensve stromal necrosis and impending/overt perforation –> emergency PK - high risk of persistent / recurrent disease in grafted tissue
3.

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16
Q

What are the clinical features of fungal keratitis? (3)

A
  1. Feathery-margined grey-white infiltrate with satellite lesion (filamentous)
  2. Dense white infiltrate (stuck on appearance) like bacterial keratitis (yeast)
  3. Hypopyon: Immobile and fluffy
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17
Q

What is the difference between PUK and marginal keratitis and Moorens?

A

PUK: Crescenteric peripheral ulcer with stromal thinning/oedema and DM folds with scleritis

Marginal: Sterile peripheral infiltrates (2,4,8,10 o clock) with a clear limbal zone

Mooren’s: no perilimbal clear zone, no scleritis, painful, usually autoimmune form of PUK

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18
Q

What is the most common corneal dystrophy recurrence in grafts?

A

Reis bucklers
Lattice
Granular
Macular

Rude little green man

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19
Q

What are the systemic and ocular associations with keratoconus?

A

Systemic: Atopy, Down’s, Marfan’s, Ehler-Danlos

Ocular: VKC, AKC, floppy eyelid, RP, Leber’s congenital amaurosis

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20
Q

Roper Hall Classification for ocular surface injuries.

A
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21
Q

What further systemic treatment can you consider in microbial keratitis? (3)

A
  1. Oral ciprofloxacin - if progressive keratitis involving limbus
  2. Oral tetracyclines
  3. Vitamin C
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22
Q

What is the conservative, medical and surgical management of corneal perforation?

A

Conservative: Self-sealing perforation, BCL
If <3mm - cyanoacrylate (works better than fibrin glue)

Medical: Moxifloxacin PF, Cycloplegia, Steroids PF, Immunosuppression (oral steroids)

Surgical: Amniotic membrane graft, DALK, PK

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23
Q

What is the difference in findings between Filamentary fungal infections and Yeast infections in fungal keratitis?

A

Yeast (Candida)
1. Rapid progression, stuck on button appearance with expanding infiltrate and small epithelial defect. Satellilte lesions

Filamentary (Fusarium/aspergilus)
1. Slow progression, feathery appearance of stromal infiltrate with mild-severe AC activity

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24
Q

What is the management of fungal keratitis? Ocular and systemic

A

General:
1. Stop contact lens wear
2. Stop any topical steroid use

Candida
1. Voriconazole 1% hourly for 3 days
2. +/- Amphotericin B 0.15% hourly D + N

Filamentary
1. Natamycin 5% hourly
2. Clotrimazole 1% hourly
3. +/- Amphotericin B 0.15% hourly day and night

Topical cycloplegics for pain relief
Systemic
1. Oral fluconazole, oral voriconazole

Treat for 6-8 weeks, taper slowly

Surgical
1. Instrastromal voriconazole injections: If deep hypopyon
2. Therapeutic keratoplasty / PKP: If ulcer progresses

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25
What are the findings of HSK in 1. epithelial 2. stromal 3. endothelial
Epithelial: SPKs --> stellate erosions --> dendritic ulcer with terminal bulbs --> geographic ulcer. Reduced corneal sensation Stromal: Interstitial haze, mimics bacterial ulcer, diffuse opacities with corneal vascularisation and lipid exudation / scarring. Increased IOP, AC activity. Endothelial: Disciform, wessley ring (stromal halo with cornela oedema), endothelitis, central KPs
26
What is the management of HSK keratitis 1. epithelial 2. stromal 3. endothelial
Epithelial: topical aciclovir 3%/ganciclovir 0.15% 5 x day +/- debridement Stromal: Oral aciclovir 400mg 5xday / topical ganciclovir 5x day then 3x day, topical steroids if no epithelial defect 4 hours after Endothelial: Aciclovir 400mg 5 x day/ topical ganciclovir 5 x day then 3x day topical steroids 48 hours after
27
What are the findings in HZO keratitis?
1. Pseudodendrites (no terminal bulbs, supercifical) 2. Nummular (anterior stromal infiltrates) / disciform keratitis later
28
What is the treatment for HZO keratitis?
All : 800mg 5 times a day for 5 days Epithelial: Topical lubricants Stromal: Down-titrating topical steroids Endothelial: Down-titrating topical steroids
29
What are the most common ocular and systemic complications associated with HZO? (4 + 2)
Ocular 1. Glaucoma 2. Episcleritis/Scleritis 3. ARN/PORN 3. Optic neuritis 4. Cranial nerve palsy Systemic 1. Cerebral vasculitis (stroke) 2. Neuralgia
30
What are the infectious and non-infectious causes of interstitial keratitis?
Infectious 1. Bacterial: Syphilis, TB, Lyme 2. Viral: HSV, VZV, EBV 3. Parasitic: Acanthomoeba, Onchoceriasis Non-infectious Sarcoid Lymphoma Autoimmune (RA, GPA) Cogan's syndrome (interstitial keratitis + hearing loss) Trauma
31
What is the management of recurrent corneal erosion syndrome?
Conservative: BCL, patching Medical: Topical lubricants, topical Abx, cycloplegia Systemic: Tetracyclines Surgery: Mechanical debridement, alcohol delamination, PTK, anterior stromal micropuncture (if outside vision axis)
32
What is Mackie classification of neurotrophic keratopathy?
Stage 1 : Irregular epithelium Stage 2 : Persistent epithelial defect Stage 3 : Stromal involvement --> ulcer/melting/descmetocele/perforation
33
What are the causes of exposure keratopathy?
1. Cranial VII palsy 2. Lid abnormality : nocturnal lagophthalmos (most common), ectropion, floppy eyelid syndrome, surgical (overcorrection of ptosis) 3. Orbital disease: Proptosis, TED
34
What is the treatment for neurotrophic keratopathy?
Conservative: Lubricants, corneal Medical: Oral tetracycline, serum eye drops Surgical: Amniotic membrane graft, tarsorrhaphy
35
What are the drug causes of vortex keratopathy? (5)
CHAI - T C: chloroquine H: Hydroxychloroquine A: Amiodarone I : Indomethacin T : Tamoxifen
36
What should you examine for if you see vortex keratopathy? (2)
Macular pathology: hydroxychloroquine Optic neuropathy: amiodarone
37
What are the complications of blepharitis?
1. Hordeolum/chalazion 2. Phylectenular marginal keratitis 3. conjunctival injection 4. pannus/scarring 5. recurrent erosions 6. corneal thinning/perf (rosacea)
38
What is the management of all subtypes of blepharitis?
Staphylococcal: 1. antibiotic ointment to lid margin (erythromycin) 1-2 / day for several weeks 2. consider mild topical steroid of significant inflammation or marginal phylctenular keratitis Seborrhoeic: 1. Treat scalp/seborrhoea (selenium sulfide shampoo) alongside lid hygeine Posterior MGD 1. Doxycycline 100mg BD for 1-2 weeks (tapered) or pulses of erythromycin 2. Topical ciclosporin/lifitegrast Demodex 1. Collarette debridement + tea-tree oil derivatives 2. Topical / oral ivermectin 3. Lotilaner 0.25% emerging
39
Summary of Blepharitis
40
Where is the steepest meridian for against the rule astigmatism? Where is the steepest meridian for with the rule astigmatism?
41
Whats the difference between Rizutti sign and Munson's sign?
Rizutti: Presence of conical wedge shaped reflection on nasal cornea when penlight is shone from temporal side (advanced keratoconus) Munson: characteristic V-shaped indentation in the lower eyelid that appears when a person with advanced keratoconus looks downward (keratoconus)
42
What are the causes of band keratopathy?
4 Cs 1. Calcium levels (sarcoid, multiple myeloma, hyperparathyroidism) and Urate levels 2. Chronic eye disease (chronic retinal detachment, late HSV infection, chronic graft rejection, exposure keratopathy 3. Chemical exposure (intraocular silicone oil, thiazides, mercury, phosphate-containing drops) 4. Congenital (Norrie's disease)
43
Difference between seasonal conjunctivitis, atopic keratoconjunctivitis and vernal keratoconjunctivitis
44
What are the clinical features and investigations of Schnyder's crystalline dystrophy?
Bilateral Central yellow/white crystals in anterior stroma with haze + arcus (subepithelial) Loss of photopic vision Associated with hypercholesterolaemia and paraproteinaemias GENETIC DEFECT: UBIAD gene on chromosome 1 Investigations: 1. Slit lamp examination 2. Anterior Segment OCT 3. Staining: Stains Oil Red O Red (cholesterol) 3. Bloods: Fasting cholesterol and triglyceride levels, cystinosis screen
45
What are the corneal stromal dystrophies and their mutations? (5)
Lattice: AD (TGFB1) Macular: AR (CHST6 on 16) Granular: AD (TGFB1) Combined granular-lattice (Avellino): AD (TGFB1) Schnyder: AD: UBIAD1
46
What is the difference in clinical features between lattice and granular dystrophy?
Lattice: 3 types Refractile anterior stromal dots coalecsing into fine filamentous criss cross lines that spares the periphery. Reduced corneal sensation Type 1 is more common than Type 2 Type 2: Meretoja syndrome - type 2 lattice dystrophy + cranial (CN7) and peripheral neuropathy, skin laxity and cardiorenal disease (amyloidosis) Type 3: Rare, Japanese Granular Asymmetric bilateral central to paracentral crumb like opacities, spares peripheries. Spares limbus. Corneal sensation reduced
47
What are the clinical features of Avellino dystrophy (Granular/lattice corneal dystrophy type 2)
Fine superificial opacities progress to form stellate/annular lesions with deeper criss/cross linear opacities Can extend to limbus
48
Which stromal dystrophies are not typically treated / manifest as recurrent corneal erosion syndrome? (2)
1. Macular dystrophy 2. Schyner's crystalline dystrophy
49
What endothelial cell count makes the cornea suitable for transplant? What cell count makes it unsuitable for transplant? What cell count makes it susceptible for corneal decompensation?
Suitable: >2200cells/mm2 Unsuitable: <1000cells/mm2 Corneal decompensation: <700cells/mm2
50
Whats the CHED1 and CHED2?
CHED 1: AD - Bilateral mild corneal oedema from infancy. Progressive. CHED 2: AR - bilateral severe corneal oedema from birth, reduced VA, ambylopia with nystagmus. Deafness
51
What are the clinical features of Posterior polymorphous dystrophy
Cluster of lines/vesicles, irregular broad bands or diffuse haze across posterior cornea (snail tracks) + iridocorneal adhesion (PAS) --> epithelial like changes in the endothelium Associated with corectopia, glaucoma (open/closed angle) PPCD + retinal flecks = Alport syndrome PPCD GENE P - Posterior corneal involvement P - Polymorphous endothelial cell transformation (epithelial-like) C - Congenital / detected in early adulthood D - Dominant inheritance G - genetic mutations ZEB1 (most common, PPCD3) COL8AE2 (PPCD2) OVOL2 (PPCD1) E - Endothelial proliferation N - Non-inflammatory E - elevated IOP risk due to angle involvement
52
What are the clincial features of meesmann's dystrophy?
Discrete clear Epithelial vesicles centrally which spread peripherally
53
Whats the difference between Reiss Buckler and Thiel Behnke?
Both have grey/white subepithelial opacities --> most dense centrally and increase to form a reticular pattern Thiel is less individually defined, have "curly fibres" on electron microscopy
54
Summary of corneal dystrophies
55
What are the differences between filamentous and yeast/candida keratitis?
56
What is the hyphae difference between filamentous and yeast?
Filamentous: hyphae and enzymes that penetrate stroma Yeast: pseudohyphae with proteases/phospholipases aiding invasion
57
What are the main causes of cicatricial conjunctivitis? (7)
1. Ocular mucous membrane pemphigoid (OCP) 2. Steven Johnsons/Toxic Epidermal Necrolysis 3. Chemical burns 4. Trachoma 5. Chronic medicamentosa 6. Ocular rosacea 7. Trauma/radiation
58
What are the signs of cicatricial conjunctivitis?
1. Fornix foreshortening 2. Subepithelial fibrosis 3. Symblepharon --> Ankyloblepharon (look for inferior symblepharon on downgaze with lower lid traction)
59
What are the staging guidelines for cicatricial conjunctivitis / OCP / MMP ?
Foster: clinical milestones 1. Subconjunctival scarring 2. Fornix foreshortening 3. Symblepharon 4. Ankyloblepharon / Frozen Globe Mondino: Inferior fornix depth loss: 0%, 25%, 50%, 75%, Tauber modifiers: Adds on symblepharon, guides monitoring/therapy escalation
60
Cicatricial diseases
61
What is the WHO classification of trachoma
1. Trachoma inflammation - follicular: >5 follicles on upper tarsal conjunctiva 2. Trachoma inflammation - intense: Pronounced thickening/ inflammation > 50% of tarsal area 3. Trachomatous scarring - conjunctival scarring visible 4. Trachoma trichiasis - 1 eyelash rubbing on globe 5. Corneal opacity - opacity involving the pupillary margin
62
What are the causes of limbal stem cell deficiency ? (LESC)
Congenital: Aniridia, Xeroderma pigmentosum Acquired: 1. Injury - chemical, thermal, UV 2. Inflammatory - OMP, SJS, PUK, VKC, Graft vs host disease 3. Ocular - contact lens wear, neurotrophic cornea, preservative drop toxicity, multiple pterygium surgeries
63
What are the clinical features of limbal stem cell deficiency? (2)
1. Conjunctivalisation onto corneal surface 2. Persistent epithelial defects/inflammation/calcification 3. Loss of palisades of vogt 4. Whorled appearnce of corneal epithelium 5. Limbal ischaemia
64
What investigations for a patient with limbal stem cell deficiency?
History: Chemical injuries, UV, contact lens wear, previous pterygium surgery Investigations 1. Impression cytology: Mucin-containing goblet cells on corneal epithelium, absence of CK 3 and CK12. 2. Immunoflourescence: Check for IgG, IgA, C3 deposits in OMP 3. Confocal microscopy: Limbal pallisades viewing.
65
What are the clinical features of meesmann's corneal dystrophy
MESS M - Microcysts E - Epithelium involved S - Symptoms mild S - Slit lamp shows vesicles
66
What are the surgical options for patients with significant cicatricial disease?
Lash and Lid Malposition 1. Epilation/electrolysis 2. Tarsal fracture (Wies) for marginal ciactricial entropion 3. Posterior lamellar replacement Symblepharon 1. Lysis + AMT/conjunctival or buccal mucous membrane graft 2. Rings/Scleral shells to maintain fornices Ocular surface reconstriction (LSCD) Cultivated oral mucosal epithelial transplant (SJS)
67
What are the serotypes of epidemic keratoconjunctivitis and pharyngoconjunctival fever conjunctivitis?
EKC: serotypes 8, 19, 37 - keratitis 80% and more severe PCF: serotypes 3,7, mild keratitis <30%
68
What are the clinical features of viral EKC / PCF ?
1. Inferior tarsal follicles 2. Tender preauricular node 3. Subconjunctival haemorrhages 4. Subepithelial infiltrates 5.Membrane/pseudomembranes EKC specific: pseudomembranes, large erosions, sub-epithelial infiltrates
69
What are the indications for topical steroids in EKC / PCF?
1. Membranes/pseudomembranes 2. Visually significant subepithelial infiltrates --> can delay viral clearance
70
What are the infection control guidelines for EKC?
Shedding takes 2 weeks - no sharing towels, meticulous hand hygiene
71
What is the difference between AKC and VKC?
72
What is the difference between HSV and HZO epithelial keratitis?
73
What is the pathophysiology / histology of limbal dermoid?
Benign congenital choristoma containing ectodermal and mesodermal tissues --> can cause astigmatism and ambylopia
74
What is the associated condition of limbal dermoid?
Goldenhar syndrome
75
Difference between SAC/VKC/GPC
76
What are the causes and location of these common corneal iron lines 1. Ferry 2. Stocker 3. Hudson-Stahli 4. Fleischer
1. Ferry - Trabeculectomy margin 2. Stocker - Pterygium margin 3. Hudson-Stahli - horizontal inferior 1/3rd of cornea (idiopathic) 4. Fleischer - base of cone in epithelium - keratoconus
77
What are the differentials of Fuch's endothelial dystrophy ?
78
What are the surgical options for Fuch's? (3)
1. DMEK: only Descemet's and endothelium - (10-20um thickness) - Faster visual recovery - Lowest induced hyperopia/astigmatism - Technically difficult, higher early graft detachment/rebubbling rates 2. DSAEK/DSEK: Posterior stroma + Descemet's + Endothelium (80-150um) - Easier - Slower visual recovery 3. PK: Stromal scarring, ectasia, previous graft failure - Higher risk of astigmatism - Higher risk of rejection
79
What is PAM? What is the difference of PAM without atypia and with atypia?
PAM: acquired unilateral flat pigmented lesion of conjunctiva affecting middle-aged white patients arising de novo PAM without atypia: Benign proliferation of melanocytes PAM with atypia: Premalignant abnormal melanocytes with nuclear atypia --> can breach the basement membrane
80
What are the differentials of PAM? (4)
81
When is the indication for surgery with PAM?
1. Histological atypia 2. Growth/irregular borders 3. Involvement of palpebral conjunctiva or caruncle
82
What is the treatment for PAM with atypia? (3)
1. Excisional biopsy with no-touch technique with cryotherapy to adjacent conjunctiva to reduce recurrence 2. Topical mitomycin C --> widespread PAM with atypia 3. Amniotic membrane graft if large areas excised
83
Adult inclusion vs neonatal vs trachoma
84
What is the difference between ligneous conjunctivitis and infective membranous / pseudomembranous conjunctivitis?
85
What are the risk factors for OSSN? (6)
1. UV exposure (interpalpebral limbus) 2. HPV (16/18) 3. HIV 4. Immunosuppresion 5. Smoking 6. Xeroderma pigmentosum 7. CIN
86
What are the clinical features of OSSN?
Conjunctival/limbal: Gelatinous/papilliform/ plaque like leukoplakic mass with sharp borders, feeder vessels interpalpebral zone Corneal: Translucent grey epithelial sheet from limbus with fimbriated/pseudopodial margins (islands possible)
87
What is the management of OSSN?
Conservative 1. Address risk factors: Stop smoking, UV protection 2. Punctal occlusion during chemo to reduce systemic absorption risk Medical 1. Topical chemotherapy with mitomycin C or interferon alpha2beta Surgical 1. Excisional biopsy with wide margins, intraoperative and postoperative MMC 2. Lamellar sclerectomy if deep scleral involvement 3. Exeenteration for orbital invasion Follow up: 3-6 monthly in year 1-2, then yearly Full ocular survey (double eversion) + regional node palpation
88
What are the investigations for a patient with OSSN?
History: Ask about risk factors, HPV, HIV, Smoking, Immunosuppresion, UV exposure, check preauricular and submandibular lymph nodes Ophthalmic - Anterior segment: Photos and OCT - Biopsy: Dysplastic changes breaching basement membrane - Impression cytology if biopsy not available
89
What are the surgical treatments of epithelial basement membrane dystrophy?
90
What are the differentials of marginal keratitis?
91
What are the clinical features of marginal keratitis?
MARGIN M: medial/peripheral location A: Antigen-antibody reaction R: Rosacea or blepharitis association G: Grey-white infiltrates I: Immune mediated N: No epithelial defect
92
What are the indications for DMEK? (6)
FEED ME F: Fuch's E : Endothelial failure post-cataract surgery (pseudophakic bullous keratopathy) E: Endothelial rejection post-PK / DSAEK D: Descemet's membrane detachment M: Microcystic oedema due to endothelial dysfunction E: Endothelial feailure in irido-corneal endothelial syndrome (selective cases)
93
Comparison of different keratoplasty procedures
94
What are the clinical features of oculodermal melanocytosis?
Ipsilateral iris hyperchromia Iris mammillations Ipsilateral hyperpigmentation of face Subconjunctival flat/grey lesion More common in males/orientals
95
Which corneal dystrophies are associated with TGFB1 (BIGH3)
LARGE L: Lattice A: Avellino R: Reis-Buckler G: Granular E: EMBD
96
What are the clinical manifestations of thygeson superficial punctate keratopathy?
THYGESON T - Teens/young adults H - Hazy central coarse opacities Y - Years of relapses G - Grey granular crumb like epithelial spots E - Epithelium only S - Steroid responsive O - Often bilateral, asymmetric N - Non-minimally staining / negative staining
97
Corneal dystrophies summary
98
What is the treatment for ocular mucous membrane pemphigoid?
1. Systemic immunosuppresion - Dapsone (mild after G6PD testing), methotrexate, mycophenolate, azathioprine 2. Severe - cyclophosphamide (oral/IV) + systemic steroids 3. Biologics - rituximab
99
How can you differentiate PMD from keratoconus? (3)
Age of onset: PMD in adults (30-50s), keratoconus begins in adolescence/early adulthood Location of protrusion/thinning: PMD - protrusion lies above area of maximal thinning Keratoconus: Protrusion and thinning coincide, typically inferocentrally Associated findings: Hydrops, scarring, Vogt's striae in Keratoconus, rare in PMD
100
What are the clinical features of phlyctenulosis?
1. Unilateral redness and photophobia 2. Small white/pink nodules on limbus or bulbar conjucntiva surrounded by engorged vessels 3. Corneal phyctelnules may ulcerate and cause pannus
101
What are the differentials of phlyctenulosis?
102
What is in the Dresden protocol for corneal cross-linking? Contraindications?
1. Topical anaesthesia 2. Central 9mm of corneal epithelium removed 3. 0.1% Riboflavin every 1-5 minutes for 30 minutes 4. Exposure to UVA light (365nm wavelength) for 30 mins (5.4 J 5. Topical antibiotic 6. BCL insertion Contraindications 1. CCT <400 2. Age >35yo
103
What is the treatment for microbial keratitis? 1. Routine ulcer 2. Large central deep ulcer/prior failure 3. MRSA risk 4. Pseudomonas high risk
104
What is the difference in findings for these chlamydial infections 1. Adult inclusion 2. Trachoma 3. Chlamydia psittaci
1. Adult inclusion - inferior palpebral conjunctiva findings 2. Trachoma - superior palpebral conjunctiva findings 3. Chlamydia psittaci: equal involvement of superior and inferior palpebral conjunctiva
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What is the difference between Lisch epithelial corneal dystrophy and Meesman corneal dystrophy?
Both have intraepithelial microcysts --> Meesman has diffuse evenly spaces cysts, X-linked Lisch has densly packed cysts.
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What is the gold standard diagnosis for ocular mucous membrane pemphigoid?
Conjunctival biopsy with direct immunofluorescence --> Linear deposition of IgG, IgA, C3 along the epithelial basement membrane zone
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Conjunctival papilloma associated with what HPV type? Which HPV type is associated with malignancy?
HPV 6, 11 (papilloma) HPV 16, 18 (malignancy)
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What are the clinical features of salzmann nodular degeneration?
1. Subepithelial elevated nodules (gray/white/blue) in a circular configuration involving central or paracentral cornea 2. Subepithelial fibrosis at 3 and 9 o clock in patients who wear hard contact lenses 3. Irregular astigmatism, photophobia and foreign body sensation
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What are the two forms of Mooren's ulcer?
1. Limited - affects middle-aged to elderly Caucasian patients, unilateral and responsive to treatment 2. Aggresive - young African patients, bilateral, resistent to treatment and higher risk of perforation --> associated with helminthic toxins (parasites)
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What are the clinical features and associations of Mooren's ulcer?
1. Grey infiltrate at leading udge with no limbal clear zone (unlike marginal keratitis and terrien's) 2. No scleritis 3. Stromal melt, progresive peripheral ulceration Associations 1. Hepatitis C 2. Autoimmune
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What are the 3 main complications of corneal graft suture abscess?
1. Endophthalmitis - immunocompromised patients 2. Descemetocele - progressive stromal melting --> descemetocele 3. Wound dehisence - localised infection at suture site weaken the graft-host junction in the early post-operative period
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What are the conditions associated with congenital corneal opacity?
STUMPED S: Sclerocornea T: Trauma/tears in descemet's U: Ulcer M: Metabolic conditions P: Peters E: Endothelial defect D: Dermoid
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What are the poor prognostic factors for conjunctival melanoma? (7)
1.FPC location (fornix, palpebral, caruncle 2.Thickness > 1mm 3.Recurrence 4.Multifocal lesion 5.De novo origin 6.Tumours not involving limbus 7.Pagetoid/lymphatic spread
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What are the clinical features of conjunctival SCC? (3)
1. Persistent unilateral keratoconjunctivitis 2. Infiltrates cornea, sclera and globe 3. Rarely metastasise
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What is the earliest sign in ocular involvement of SJS? What is the most important diagnostic indicator sign of SJS?
Earliest: Lid margin staining Diagnostic indicator: Bulbar conjunctival staining
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Which stromal corneal dystrophy is more commonly associated with recurrent erosions?
Lattice
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What are the differentials for granular corneal dystrophy?
Other stromal corneal dystrophies 1. Macula 2. Lattice 3. Avellino 4. Schnyder's corneal dystrophy Deposition 1. Vortex Keratopathy 2. Crystalline keratopathy
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How do you differentiate granular dystrophy from macular dystrophy? (5)
1. Granular --> Clear stroma, Macular --> Cloudy stroma 2. Granular --> central involvement, Macular --> peripheral and central 3. Granular --> No corneal thinning, Macular --> corneal thinning 4. Granular --> hyaline stains with masson trichrome, Macular --> muco-polysaccharides stain with Alician blue 5. Granular --> AD inheritance Macular --> AR inheritance
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What is the management for a patient with granular corneal dystrophy?
Acute erosion: Lubricants, hypertonic saline for corneal oedema and to improve epithelial adhesion, antibiotic ointment, cycloplegia, BCL. Conservative: Photophobia - tinted glasses Refractory: 1. Anterior stromal puncture (usually for post-traumatic cases, not used centrally to to possibility of scarring) 2. superficial keratectomy (allows for more uniform regrowth with alcohol delamination) 3. Diamond Burr polishing - smoothen Bowmen's membrane and promote better adhesion 4. PTK: excismer laser treatment to remove superficial pathology and provide smooth surface for epithelial basement membrane attachment 5. Reduced vision: DALK or PK (can recur)
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What are the clinical features of granular dystrophy?
1. Small discrete sharply demarcated grayish/white opacities in anterior central stroma, with intervening clear stroma and sparing of periphery 2. Opacities vary in shape and can be either drop-shaped, crumb-shaped or ring shaped (Crumb-like opacities)
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Which corneal dystrophy has earliest onset?
My lovely Granny Macula Lattice Granular
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What are the differentials of corneal opacities?
***I would classify causes of corneal opacity by affected corneal layer and tailor further assessment based on depth, laterality, vascularisation and associated inflammation Epithelium: - Epithelial scarring - recurrent erosions, trauma - Limbal stem cell deficiency (chemical burns, steven Johnsons) - Band keratopathy - Lipid keratopathy - Neurotrophic keratopathy Bowman's layer: - Trauma (Bowman's doesn't regenerate, permanent scar) - Reis-Buckler's dystrophy - Thiel-Behnke dystrophy Stroma -Infective : Herpes simplex, bacterial, fungal, acanthomoeba - Inflammatory/Immune: Interstitial keratitis (syphilis, TB), PUK, Mooren's ulcer - Ectatic - Keratoconus - Degenerative - Salzmann, Terrien's - Dystrophies: Macular, granular, lattice - Trauma: Corneal scars, mucopolysaccharidosis, cystinosis Stromal opacities tend to be visually significant due to light scatter Descemet's membrane - Haab striae (congenital glaucoma) - Descemet's membrane rupture - Hyodrops scars Endothelium - Fuch's - Endothelitis (HSV, CMV) - ICE syndrome Congenital 1. Peter's anomaly 2. Sclerocornea 3. Congenital hereditary endothelial dystrophy 4. Birth trauma
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What are the causes of corneal opacity with vascularisation?
Superficial causes - chronic blepharitis/ocular surface disease - limbal stem cell deficiency - ocular cicatricial pemphigoid - exposure keratopathy - vitamin A deficiency Deep stromal causes - interstitial keratitis (syphilis, tb lyme) - Herpes (simplex + zoster) keratitis - PUK - Moorens - Severe microbial keratitis Mixed - Chemical injuries - Ocular cicatricial pemphigoid - Graft failure/rejection
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What are the indications for a penetrating keratoplasty?
Optical - restoration of corneal clarity : 1. keratconus 2. corneal scarring secondary to herpes simplex, infections, trauma, 3. Deep stromal corneal dystrophies Tectonic - restoration of corneal integrity: 1. Corneal thinning - inflammatory/immune (PUK), Infective, Ectatic, Traumatic (burns), neurotrophic 2. Threatened / actual perforation - infective, inflammatory, neurotrophic, trauma, ectatic (post-hydrops rupture) Therapeutic: Infective keratitis
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What are the contraindications for a PK? (4)
No absolute, just relative 1. Epithelial dysfunction secondary to limbal stem cell deficiency 2. Severe neurotrophic cornea 3. . Stromal vascularisation involving more than 2 quadrants 4. . Multiple (2+) graft failures
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What are the risks of PK? (3)
1. Higher risk of graft rejection 2. Risk of suture related complications 3. Risk of traumatic wound dehiscence and globe rupture
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What are the complications of a PK?
Intraoperative 1. Retained Descemet's membrane - presence of double AC on post-op day 1 2. Scleral perforation from bridal suture placement 3. Haemorrhage: AC haemorrhage from iris damage or suprachoroidal explusive haemorrhage Early post-operative 1. Wound leaks (shallow AC/Seidels +ve) - can be controlled by BCL, resuturing not necessary 2. Epithelial defect: Lubricants/BCL/ tarsorrhaphy, decreased steroids 3. Suture related complications: - immune infiltrates (multiple on recipient side of cornea and treat with topical steroids and removal of suture) - Kaye dots (small epithelial dots a short distance from sutures on donor side, not pathological and disappears after suture removal) 4.- **Endophthalmitis** Late post-operative
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What is the reason for a small graft rather than a large one?
Tectonic:
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What is the difference in aetiology between graft failure and graft rejection?
Graft rejection: Immune attack against donor endothelium (usually first 3 years) Causes: - Epithelial rejection: elevated epithelial rejection line those stains with fluorescein—responds to topical steroids * Subepithelial infiltrates: immune reaction seen only in the donor tissue without associated conjunctivitis—responds to topical steroids Endothelial rejection line (khodadoust line): does not extend beyond graft-host junction with KP’s at the leading edge with an overlying hazy and oedematous stroma +/− DM folds—if left untreated this line will proceed across the donor endothelium from the point of origin at the graft wound leaving damaged endothelium behind it—treat with intensive topical steroids * Diffuse endothelial rejection: isolated or diffuse KP’s are scattered across the endothelium and are limited to the donor endothelium and often associated with an AC reaction—treat with intensive topical steroids Graft failure: permanent loss of graft clarity or function either primary or secondary causes Primary: Straight away, corneal opacity and haze never clears Secondary causes: - irreversible rejection - chronic endothelial cell loss -poor donor quality - intraoperative trauma to donor endothelium - prolonged flat AC in the immediate postoperative period - HSV reactivation
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What are the signs of graft rejection?
- red eye - AC activity - Raised IOP - Keratic precipitates - Corneal oedema - Khodadoust line
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What are the risk factors for immunological rejection? (5)
- Corneal stromal vascularisation - Reduced corneal sensation - Ocular surface disease (Dry eye, OCP, SJS) - Previous graft failure - Large graft size (8–8.5 mm) and eccentric grafts
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What are clinical findings of spheroidal degeneration (labrador keratopathy)?
Early: Amber or golden spherical deposits peripherally in interpalpebral zone - subepithelial / anterior stromal Moderate: Central involvement Advanced Visual axis involvement Bilateral small translucent amber-colored oil droplet spherules in the superficial corneal stroma located in interpalpebral zone Droplets can coalesce to form larger, nodular elevated opacities causing hazy appearance
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What are the relevant differentials for spheroidal degeneration? (3)
1. Salzmann nodular degeneration + : both have superficial nodules -: Salzmann are blueish white and follow ocula rsurface inflammation, whereas spheroidal degeneration are amber coloured and UV related 2. Band keratopathy + : Both are lesions which occur in interpalepbral space -: Band - calcium deposits, usually white/grey and leave clear zone at limbus, spheroidal degenertation starts at limbus 3. Corneal dystrophy
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What is the classification of spheroidal degeneration?
Grade 1: Fine droplets in peripheral nasal/temporal limbus Grade 2: Droplets spreading to central cornea Grade 3: Large prominent nodules significantly impairing vision and cause surface irregularity
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What are the risk factors for spheroidal degeneration? (3)
1. Occupational/environmental exposure: History of working outdoors in high UV environments (fishermen, farmers) 2. Exposure to wind/sand/extreme cold 3. Previous ocular surface disease/trauma
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What are the investigations for spheroidal degeneration? (3)
1. Slit lamp biomicroscopy - assess depth and extent of spherules 2. Anterior segment OCT: Localise depth of deposits (usually superficial stroma) 3. Topography - quantify degree of irregular astigmatism caused by nodules
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What is the management of spheroidal degeneration?
1. Conservative: UV protection with wrap-around sunglasses and hats, environmental protection 2. Medical: PF lubricants to improve tear film and reduce foreign body sensation 3. Surgical: - **superficial keratectomy:** manual debridement of nodules of they affect vision or cause persistent irritation - **phototherapeutic keratectomy (PTK)** - using an excimer laser to smooth corneal surface after manual removal - **Lamellar or penetrating keratoplasty** - only in rare cases where opacification is deep and VA cannot be restored by superficial means
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What are the differentials of a patient with an iris naevus and hyphaema? (4)
1. **Iris melanoma with hyphaema**: melanomas typically >3mm in diameter, thicker than 1mm more likely to progressively grow 2. **Ciliary body melanoma with anterior extension** 3. **Iris neovascularisation** : rubeosis iridis due to neovascular glaucoma can cause sponatenous hyphaema 4. **Traumatic hyphaema**
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What are the risk factors of iris naevus turning into malignancy?
ABCDEFG A: Age (younger age at diagnosis) B: Blood (spontaneous hyphaema) C: Clock hour (inferior location) D: Diffuse growth pattern E: Ectropion uveae F: Feathery / satellite margins G: Glaucoma (secondary)
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What are the clinical features of an iris melanoma?
1. Inferior iris lesion 2. Ectropion uvae 3. Corectopia Angle findings 1. Tumour seeding, infiltration
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What is the treatment of a uveal melanoma?
**Conservative: 1. Serial anterior segment photographs every 3-6 months for low risk lesions 2. ultrasound B scan **Medical **1. Hyphaema management - cycloplegics, steroids, IOP agents **Surgical **1. Iridectomy/Iridocyclectomy with cosmetic contact lens 2. Radiotherapy - plaque Ruthenium-106/Iodine 125 for medium size tumours 3. Proton beam therapy - alternative for larger/diffuse melanomas 4. Enucleation : Extensive lesions, extrascleral extension, uncontrolled secondary glaucoma******
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What are the clinical findings for this patient?
Marked displacement of the pupil (eccentric, not central) - correctopia Irregular pupil shape Distorted iris architecture with areas of traction and iris strands Poor central pupillary alignment with the visual axis Want to check VA, IOP, angle findings, trauma history, uveitic history
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Differentials between Axenfield rieger and ICE syndrome Differentials for these type of pupil abnormalities
144
What are the clinical features of Terrien's Marginal Degeneration?
Ts Thinning: Peripheral stromal thinning which spreads circumferentially Top thinning: Superior thinning more common Thin vascularisation: Superficial Lipid deposition at edge of thinning Epithelium intact Irregular astigmatism/against-the-rule: Gutter thinning
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What are the investigations of a patient with Terrien's Marginal Degeneration?
HISTORY 1. Demographics : 20-40 male 2. Visual symptoms: Blurring due to irregular astigmatism 3. Pain: Confirm absence of pain to rule out inflammatory causes 4. Systemic health: Rule out systemic vasculitis INVESTIGATIONS Slit lamp: assess depth of gutter and presence of lipid/pannus Anterior segment OCT: measure residual stromal thickness Corneal topography: quantify degree of irregular astigmatism
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What is the management for a patient with Terrien's Marginal Degeneration?
Conservative 1. Glasses / RGPs to correct high against the rule irregular astigmatism 2. Safety glasses : polycarbonate lenses to protect thinned cornea Medical - nil Surgical - Indications: Impending perforation, uncorrectable astigmatism Crescenteric lamellar keratoplasty: tectonic or optical Annular ring keratoplasty: advanced circumferential disease Full thickness banana graft: if perforation
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What are the differentials for a patient with Terrien's Marginal Degeneration?
1. Moorens' Ulcer: painful active epithelial defect with an overhanging edge 2. PUK : painful, active stromal loss, 3. Marginal keratitis: Clear limbal zone 4. Corneal Arcus: clear inteval and does not involve corneal thinning
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What are the differentials of a patient with Salzmann nodular degeneration?
1. Spheroidal degeneration: Golden-brown oil droplets in interpalpebral zone 2. Band keratopathy: Calcium deposits in interpalpebral zone 3. Terrien's marginal degeneration: superior peripheral thinning with intact epithelium 4. Phylencticular conjunctivitis: acute, painful, intense injection with vessels
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What are the risk factors for Salzmann nodular degeneration?
1. Chronic MGD 2. Contact lens-wear (long-term) 3. Corneal scarring (interstitial keratitis, trachoma, trauma)
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What are the investigations of a patient with Salzmann's Nodular Degeneration?
Foreign body sensation, gradual blurring of vision (nodules encroaching visual axis) 1. Slit lamp examination with flourescein staining - check for epithelial defect and negative staining above raised nodules (tear film thinning over nodules) 2. Corneal topography: Degree of irregular astigmatism 3. Anterior segment OCT: Can show subepithelial nodular changes
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What is the management of a patient with Salzmann Nodular Degeneration?
Conservative Lid Hygeine: underlying MGD Scleral lenses (RGPs): irregular astigmatism and comfort Surgical: SK: Superficial keratectomy: remove nodules in subepithelial plane PTK: Smooth basement membrane after manual removal and reduce risk of recurrence
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What are the indications for ICRS (intrastromal corneal ring segments)? (4)
1. Keratoconus: reduce corneal irregularity and astigmatism in patients intolerant of contact lenses 2. Post-LASIK ectasia: 3. Pellucid Marginal Degeneration: regularise the topography 4. Low Myopia: historically.
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What is the mechanism of action of ICRS (intrastromal corneal ring segments?)
Inserted in mid-peripheral corneal stroma Flatten the central cornea Often combined with corneal cross-linking
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What are the early post-operative complications of corneal grafts?
1. Raised IOP secondary to steroid response or PUPILLARY BLOCK 2. Wound leak: Shallow AC, Positive seidel's test 3.Infection: Fungal/Bacterial keratitis or endophthalmitis 4. Epithelial defects 5. Primary Graft Failure: persistent corneal oedema from day 1.
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What are the most common reasons for graft failure?
“GRAFT FAILS” G – Glaucoma R – Rejection A – Astigmatism F – Failure T – Trauma (wound dehiscence) F – Infection A – Abscess (suture) I – Irregular epithelium L – Leak S – Suture problems
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What are the intermediate post-operative complications following graft surgery?
Graft rejection: Epithelial rejection: raised epithelial lines Stromal rejection: Stromal oedema Endothelial rejection: Khadodoust line Astigmatism Irregular astigmatism due to suture tension or graft/host mismatch Suture related complications 1. Loose sutures 2. Suture abscess 3. Vascularisation
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What are the late post-operative complications?
1. Graft failure: Corneal decompoensation 2. Corneal vascularisation: Increased rejection risk 3. Recurrence of original disease: 4. Post-keratoplasty glaucoma: Chronic IOP elevation 5. Infectious crystalline keratopathy: Needle-like branching crystalline stromal infiltrates in corneal graft and immunocompromised corneal patients
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What are the clinical features of lipid keratopathy?
1. Yellow white crystalline stromal opacity 2. Starts peripherally and extends centrally 3. Associated superficial/deep vessels 4. Opacity follows the direction of corneal vessels
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What are the differentials of a patient with lipid keratopathy?
1. Interstitial keratitis: Salmon patch injection, stromal oedema, deep vascularisation 2. Schnyder Corneal Dystrophy: Bilateral central crystalline opacities with corneal arcus (UBIAD gene) 3. Band keratopathy: Calcium deposits in Bowman's layer, horizontal interpalpebral distribution / swiss cheese holes 4. Spheroidal degeneration
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What are the causes of lipid keratopathy?
1. Primary (rare): no corneal vascularisation, bilateral, associated with systemic lipid disorders 2. Secondary: associated with corneal neovascularisation, often unilateral - Infectious: HSK, HZO, microbial - Inflammatory: Interstitial keratitis, phlyctenular keratoconjunctivitis, rosacea - Degenerative: chronic blepharitis, exposure keratopathy - Trauma/surgery: penetrating injury, graft rejection
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What are the investigations of a patient with lipid keratopathy?
Ophthalmic 1. Slit lamp photography: document extent of lipid and location of feeder vessels 2. Anterior segment OCT: assess dephth of lipid deposition within stroma 3. Corneal topography: Assess for irregular astigmatism Systemic 1. Fasting lipid profile: rule out hyperlipidaemia 2. Syphilis/TB serology: Rule out IK causes 3. Autoimmune screening: ANA/ANCA if autoimmune disease suspected
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What is the management of lipid keratopathy?
Conservative: Treat underlying cause (blepharitis/herpetic disease) RGP lenses Medical 1. Control inflammation: topical steroids or cyclosporine (to control feeder vessels) Surgical 1. Argon laser photocoagulation: Target feeder vessels at limbus to induce regression 2. Fine Needle Diathermy: Cauterisation of vessels under slit lamp 3. Anti-VEGF injections: Subconj or intrastromal (Bevacizumab) to induce regression 4. Superficial keratectomy, lamellar keratoplasty (deep stromal lipid deposition) 5. Penetrating keratoplasty: Visual axis involvement with significant scarring --> higher graft rejection risk due to pre-existing neovascularisation
163
What are the clinical features of silicon oil in AC?
1. Superior AC oil bubble floats upwards 2. Contact with corneal endothelium 3. Shallow AC with elevated IOP Longstanding cases 1. Band-shaped keratopathy 2. Corneal decompensation 3. Endothelial failure in aphakia: create inferior peripheral iridectomy (Ando iridectomy) : Allows aqueous flow beneath oil bubble to prevent pupillary block
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What are the complications of silicon oil in AC?
1. Corneal endothelium damage/corneal decompensation (bullous keratopathy) 2. Secondary glaucoma: Pupillary block, angle obstruction, trabecular infiltration
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Why does silicon oil enter the AC/ what are the risk factors?
Normally prevented by: intact posterior capsule lens barrier iris–lens diaphragm Migration occurs when these barriers are disrupted. Common mechanisms 1️⃣ Aphakia (most important) ⭐ No lens → direct passage into AC 2️⃣ Posterior capsule rupture 3️⃣ Zonular weakness 4️⃣ Large iridectomy / surgical defects 5️⃣ Overfill with silicone oil 6️⃣ Supine positioning early post-op Risk factors: Aphakia Complicated cataract surgery Trauma Large peripheral iridectomy Long-term silicone oil tamponade Hypotony Repeated intraocular surgery
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What are the investigations of a patient with silicon oil in AC?
Slit lamp examination: Size of oil bubble, endothelial touch, AC dephth, lens status, peripheral iridectomy patency IOP, Gonioscopy (silicon oil drops, PAS) Anterior segment OCT + Specular microscopy + corneal pachymetry: Assess for endothelial cell loss. B scan: PVR/retinal detachment recurrrence
167
What is the management of a patient with silicone oil in the AC?
Conservative: observe if no cornela touch, normal IOP, early post-operative Medical 1. Positioning (face-down) 2. IOP control drops Surgical. Indications: Corneal touch, uncontrolled IOP, endothelial compromise 1. Anterior chamber washout 2. Silicon oil removal 3. Inferior peripheral iridectomy if pupillary block present
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What are the clinical features of lattice corneal dystrophy?
1. Fine branching refractile lines resembling spider webs --> Begin centrally and spread peripherally 2. Intervening stroma is clear, but can progress to diffuse subepithelial haze with limbal sparing (peripheral 1-2mm) 3. Usually bilateral 4. Evidence of recurrent erosions: Epithelial irregularity, microcysts, focal staining
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What are the differentials of a patient with lattice corneal dystrophy?
1. Granular dystrophy 2. Macular dystrophy 3. Reis-Bucklers dystrophy
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What are the investigations of a patient with lattice corneal dystrophy?
History: typical history present in first decade of life with RCES, and significant visual impairment occurs in 4th/5th decade Investigations - Anterior segment OCT: hyperreflective deposits in subepithelial/anterior stromal layers - Corneal topography: check for irregular astigmatism Histology: Amyloid deposits, Congo Red Positive, apple-green bifringence under polarised light Systemic: - Neuro examination to rule out cranial nerve palsies - Check for systemic amyloidosis - Check for cardiorenal disease Genetic testing: TGFB1 mutation
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What are the types of lattice dystrophy?
1. Type 1: Classic - isolated corneal disease 2. Type 2: Meretoja syndrome - systemic amyloidosis, cranial neuropathies, facial nerve palsy, lax skin. Corneal signs START PERIPHERALLY and move centrally
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What is the management of a patient with lattice corneal dystrophy?
Conservative - Lubricants, BCL Medical - Lubricants, hypertonic saline, topical antibiotics (if epithelial defect) Surgical - Mild: PTK (clearing subepithelial haze) , anterior stromal puncture (peripheral) epithelial debridement - Severe: DALK, PK --> recurrence can occur after graft.
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What are the subtypes of anterior scleritis?
Diffuse: Generalised inflammation, painful deep violacious hue (moderate) Nodular: Localised, tender, immobile nodule Necrotising without inflammation (scleromalacia perforans) : Classically in long-standing chronic seropositive RA; quiet eye, painless scleral thinning and blue hue. Angiography --> vascular occlusion Necrotising with inflammation: Severe pain, avascular white patches, scleral thinning, risk of perforation
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When would you consider GPA in a patient with anterior scleritis?
Ophthalmology: Scleritis + peripheral corneal infiltrates (PUK) Other: Glomerulonephritis/pneumonitis and saddle nose deformity
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When would you consider relapsing polychondritis with anterior scleritis?
Inflammation of cartilage affecting the ear, nose (saddle-nose), trachea/larynx and large cardiac vessels. Can also cause episcleritis.
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What is usually the main cause of surgically induced necrotising scleritis? (SINS)
Non-infectious and infectious. Infectious scleritis normally caused by pseudomonas most commonly after.. 1. pterygium surgery 2. strabismus surgery 3. trabeculectomy 4. VR surgery
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What is the treatment of anterior scleritis?
Non-necrotising: 1. Oral NSAIDS: Flurbiprofen or indomethacin 50mg TDS 2. systemic immunosuppresion with steroids: oral 1mg/kg/day then gradual taper. Topical steroids help with symptoms. Necrotising: needs systemic immunosuppresion - MTX, cyclophosphamide, mycophenolate, inflixamab - with rescue therapy with IVMP, PO steroids Subconjunctival steroid is controversial: risk of perforation / high IOP. INFECTIOUS CAUSES MUST BE RULED OUT PERIOR TO IMMUNOSUPPRESION Referral to rheumatology to co-ordinate care.
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What are the differentials for posterior T sign?
RCS thickening (retina, choroid, sclera) in VKH and sympathetic ophthalmia Posterior scleritis
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What are the clinical features of Fuch's heterochromic iridocyclitis?
FUCHS F - Fine stellate KPs U - Unilateral uveitis with spillover with no posterior synechiae C - Cataract - PSC common H - Heterochromia due to diffuse iris stromal atrophy with iris nodules S - Secondary glaucoma
181
What are the differentials of blue sclera? (4)
SOCK 1. Scleromalacia perforans 2. Osteogenesis imperfecta - brittle bones, hyperextended joints, sensorineural hearing loss and hyperopia 3. Connective tissue disorders (EDS, Marfans, pseudoxanthoma elasticum) 4. Keratoconus/Keratoglobus
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What are the risk factors for JIA ocular involvement? (6)
1. ANA positive 2. RF negative 3. Oligoarthritis 4. Lower extremity arthritis 5. Lack of wrist arthritis 6. Female
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What are the indications of a cosmetic contact lens?
Gross corneal opacities Iris abnormalities: Polycoria, Anridia, post-traumatic causes
184
What are the differentials of a patient with vortex keratopathy?
1. Herpes keratitis 2. Prominent/enlarged corneal nerves
185
What is the history/examination strategies for a 3 month old child with epiphora?
Step 1: Rule out congenital glaucoma first 🚨 photophobia ⭐, blepharospasm, tearing + irritability corneal clouding, enlarged eye Examine: corneal diameter (>12 mm suspicious) corneal haze Haab striae IOP (if possible) optic nerve tear meniscus height ↑ crusting at medial canthus reflux from punctum with pressure eyelid malposition (rare) Lacrimal sac compression test ⭐ Press over lacrimal sac: mucopurulent reflux from punctum → diagnostic of CNLDO Fluorescein dye disappearance test (FDDT) Instil fluorescein: Normal: clears within 5 minutes Delayed clearance: nasolacrimal obstruction likely History: Onset since birth → likely CNLDO later onset → consider infection, trauma, glaucoma Laterality unilateral more common in CNLDO bilateral possible but raises suspicion of glaucoma Discharge type watery → obstruction mucopurulent → secondary infection Redness Suggests: conjunctivitis, dacryocystitis
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What are the treatment options for a patient with congenital nasolacrimal duct obstruction?
Conservative: 1. Crigler massage: Downward pressure over sac 4-5 times a day Surgical 1. If persistent > 12 months -->syringing and probing 2. repeat probing, balloon dacryoplasty, silicone tube intubation, DCR (rare in infants)
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What are the investigations for a patient with macular dystrophy?
History: Ask about recurrent corneal erosion symptoms 1. Slit lamp examination: 2. Staining: Stains Mucopolysaccarides Alician Blue 3. Anterior segment OCT: Corneal thinning 4. Systemic: Urinary GAGs 5. Genetic testing: CHST6 (autosomal recessive)
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What is the management of a patient with macular dystrophy?
Acute erosion: Lubricants, hypertonic saline for corneal oedema and to improve epithelial adhesion, antibiotic ointment, cycloplegia, BCL. Conservative: Photophobia - tinted glasses Refractory: 1. Anterior stromal puncture (usually for post-traumatic cases, not used centrally to to possibility of scarring) 2. superficial keratectomy (allows for more uniform regrowth with alcohol delamination) 3. Diamond Burr polishing - smoothen Bowmen's membrane and promote better adhesion 4. PTK: excismer laser treatment to remove superficial pathology and provide smooth surface for epithelial basement membrane attachment 5. Reduced vision: DALK or PK (less commonly recurs)
189
What are the differentials of a patient with microbial keratitis?
1. Bacterial keratitis: Wet infiltrate, pseudomonas/staphylococcus 2. Fungal keratitis: Feathery margins, satellite lesions, immobile 'sticky' hypopyon 3. Acanthamoeba keratitis: Ring infiltrate, history of contact lens water exposure 4. Herpetic geographical ulcer: Recurrence, reduced corneal sensitivity 5. Post infective keratitis/endophthalmitis/TASS 6. Non-infectious keratitis: Marginal keratitis, Mooren's ulcer, PUK.
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What are the investigations for a patient with microbial keratitis?
History 1. Contact lens wear: Swimming/Shower/Sleeping 2. Recent vegetative trauma/use of steroids 3. Immunosuppresion Ophthalmic 1. Corneal scraping: Scraping for culture and gram stain: Gram stain, Giemsa stain, KOH mount. Plate samples on Blood agar, Chocolate agar and Sabouraud agar 2. Corneal PCR 3. Corneal impression Membrane 4. Corneal biopsy 5. Anterior segment OCT: Depth of stromal involvement and monitor for thinning 6. Confocal microscopy: Consider if fungal
191
What is the management of a patient with microbial keratitis with hypopyon?
Admission Medical: 1. Fortified intensive antibiotics: Ceftazidime (50mg/ml), Vancomycin (50mg/ml) 2. Cycloplegia: Atropine 1% TDS to manage ciliary spasm and prevent formation of posterior synechiae 3. No steroids until epithelial defect has healed 4. Oral ciprofloxacin 500mg BD/moxifloxacin 400mg OD If not responding.. 1. Add second agent (eg cefuroxime) 2. Consider witholding treatment for 12-24 hours and then re-scraping 3. Consider alternative causing organism 4. CORNEAL BIOPSY 5. Amniotic membrane for persistent epithelail defect 6. Corneal glue for small perforation <3mm 7. Tectonic corneal graft >3mm for larger perforation
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What are the investigations of a patient with PPCD?
History - Alport syndrome: XR condition - sensorineural deafness, glomerulonephritis Examination - Vesicular lesions: transparent round cysts in line/clusters - Band lesions: Horizontal with scalloped edges - Gonioscopy: PAS seen - Lens: Anterior lenticonus - Fundus: Pigmentary retinopathy Bloods 1. Renal function tests and Urinanalysis: Alports 2. Hearing Tests: Alports
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What is the management of a patient with PPCD?
Conservative: Observe: usually asymptommatic Medical: Screen for glacuoma: Treat medically/surgically Surgical 1. PK - if significant reduced vision 2. GDD if uncontrolled glaucoma
194
What are the differentials of a patient with PPCD?
1. Endothelial dystrophies: Fuchs', CHED 2. ICE syndrome: PAS, corectopia, glaucoma 3. Axenfeld-Reiger syndrome 4. Congenital Glaucoma
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What are the clinical features of Fuch's endothelial dystrophy?
1. Endothelial corneal guttata: Beaten metal apperance 2. Corneal stromal oedema with MD folds 3. Microcystic epithelial oedema --> bullae --> erosions 4. Subepithelial fibrosis scarring between epithelium and Bowman's membrane 5. POAG 6. TCF4 genetic association
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What are the differentials to consider in a patient with corneal guttata / Fuch's endothelial dystrophy?
1. Pseudophakic bullous keratopathy 2. ICE syndrome (Chandler's) 3. PPCD 4. CHED 5. Pseudoguttae: Post-trauma, post-intra-ocular inflammation --> transient and disspear with resolution of underlying condition 6. Hassall-Henle Bodies: Normal aging change - guttata located in peripheral cornea rather than centrally 7. PDS: Krukenburg's sspindle can mimic guttata
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What is the staging of Fuch's Endothelial Dystrophy?
Stage 1: Central guttata, asymptommatic Stage 2: Confluent guttata, stromal oedema Stage 3: Epithelial oedema and bullae formation Stage 4: Subepithelial fibrosis and scarring
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What are the investigations of a patient with Fuch's Endothelial Dystrophy?
History 1. Transient blurring in morning (overnight tear film evaporation reducing corneal hydration) 2. Pain: Suggests ruptured bullae 3. Family history: AD with female preponderance Investigations 1. Pachymetry: CCT - 600-640um in presence of guttata suggests significant oedema 2. Specular Microscopy: Endothelail cell density (ECG), pleomorphism (shape variation), polymegathism (size variation). ECD <1000 cells/mm2 increases risk of decompensation 3. AS-OCT: Visual Decemet's membrane and map stromal oedema 4. Genetic testing: TCF4 gene association
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What is the management of Fuch's Endothelial Dystrophy?
Medical 1. Hypertonic saline 5% drops: Drops during day and ointment at night to dehydrate cornea via osmosis 2. Warm Air: Use a hair dryer at arms length to speed up tear evaporation Surgical 1. DMEK: Gold standard: Transplants only Descemet's and endothelium - faster recovering and lower rejection 2. DSEK/DSAEK: Transplanting endothelium, Descemet's and posterior layer of stroma: Significant corneal scarring 3. DSO: Descemet stripping only. Removing central guttata without transplant --> allows patient own peripheral cells to migrate centrally (only for specific cases with healthy peripheral counts)
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What are the clinical features of spheroidal degeneration?
AMBER A: Amber/Gold droplets peripheral interpalpebral cornea M: Many Glistening /coalescent nodules nodules B: Band-shaped distribution E: Epithelial irregularity R: Rough surface Clear superior cornea under upper lid
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What are the differentials of a patient with Spheroidal Degeneration?
BANDS B: Band keratopathy: Both in interpalpebral space - caclium deposits limbal sparing, spheroidal generally starts peripherally (at limbus) A: Amyloid dystrophy N: Nodular Salzmann Degeneration: superifical nodules - typically bluish white and follow chronic ocular surface inflammation (phlyctenular keratitis) D: Droplet keratopathy (Lipid keratopathy), crystalline keratopathy, drug-induced keratopathy
202
Which drugs cause vortex keratopathy? Which drugs cause crystalline keratopathy?
Vortex: ACATS A: Amiodarone C: Chloroquine A: Antimalarials (hydroxychloroquine) T: Tamoxifen S: Some others : Indomethacin, phenothiazines Crystalline keratopathy: CIG C: Ciprofloxacin I: Indomethacin G: Gold
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What are the investigations of a patient with spheroidal degeneration?
History: High UV exposure, chronic eye disease, ask about irritants (wind/sand) Investigations 1. Anterior segment OCT 2. Topography (irregular astigmatism)
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What is the management of a patient with spheroidal degeneration?
Conservative: 1. UV protection: Wrap-around sunglasses and wide-brimmed hats Medical 1. Preservative-free lubricants to improve tear film and reduce foreign body sensation Surgical 1. Superficial keratectomy: Manual debridement 2. PTK: Excimer laser to smoothen corneal surface 3. Lamellar/penetrating keratoplasty: Rare when opacification is deep and significant VA
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What are the clinical features for lipid keratopathy?
WHITE W: White/Yellow hazy stromal deposits near vessels H: Hazy cornea I: Irregular margins T: typically peripherally and enlarge towards vessel E: Enlarging lipids adjacent to feeder vessels
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What are the causes of lipid keratopathy?
Corneal inflammation --> neovascularisation --> vessels leak serum lipids and deposit into stroma Primary: Rarer, no corneal vascularisation, no prior inflammation, often central (idiopathic lipid deposition) Secondary - Infective: HSK, Trachoma, IK causes (syphilis) - Inflammatory/atropic: VKC, Moorens, Marginal, Rosacea - Contact lens related: hypoxia - Surgery: PK, LASIK complications
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What are the investigations of a patient with lipid keratopathy?
History - HSK history, syphilis history Investigations - Anterior segment OCT (depth of lipid deposition) Bloods - Lipid profile (systemic hyperlipidaemia) - Syphilis serology
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What is the management of a patient with lipid keratopathy?
Conservative - Treat underlying cause (blepharitis, HSK) Medical - Topical steroids/cyclosporin can reduce leakiness of vessels Surgical - Fine needle diathermy: Needle used to cauterise vessels - Argon Laser photocoagulation: Feeder vessels at limbus to induce regression - Anti-VEGF injections: Intrastromal/subconj to induce regression of neovascularisation - PK: Significant scarring - high risk of graft rejection
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What are the clinical findings of Peters Anomaly?
Central corneal opacity + iridocorneal adhesions CORNEA: - Central well-demarcated corneal leukoma - Peripheral cornea clear - Absent endothelium and Descemets membrant at leukoma site - Iridocorneal adhesions - Keratolenticular adhesions TYPE 1: Opacity + iridocorneal adhesions + normal lens (best prognosis) TYPE 2: Opacity + keratolenticular adhesions + lens abnormalities (cataract, lenticulocorneal touch) (worse prognosis) PETERS PLUS: Ocular findings + systemic associations (short stature, cleft palate, ear abnormalities
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What are the differentials of a patient with Peters anomaly?
STUMPED S: Sclerocornea T: Trauma (forceps injury at birth) U: Ulcer (neonatal keratitis) M: Metabolic: Mucopolysaccharidosis P: Peters anomaly E: Endothelial Dystrophy (CHED) D: Dermoid (limbal)
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What are the investigations of a patient with Peters anomaly?
History: Birth history (trauma), FH of eye disease, systemic developmental delays or abnormalities (cleft palate, heart defects) Investigations EUA: IOP, corneal diameter (megalocornea), A scan UBM: visualize the iridocornela and lenticulocorneal adhesions behind opacity Genetic testing: PAX6, PITX2, FOXC1, CYP1B1 associations Renal/Cardiac/Paediatric screen: Peter plus
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What is the management for a patient with Peters Anomaly?
Ophthalmic 1. Amblyopia prevention: Aggressive patching of better eye and pupillary dilation (to allow vision through clear periphery) started early 2. Glaucoma control : medical management with aqueous suppressants Surgical 1. Optical iridectomy: Create new pupil via iridectomy 2. PK: guarded prognosis 3. GDD/Cyclodiode: Manage glaucoma
213
What are the clinical features of a patient with aniridia? Types?
I-FOCAL-G I: Iris hypoplasia - large pupil appearance, thin iris stump, transillumination defects F: Foveal hypoplasia: Reduced VA, Nystagmus. O: Optic nerve hypoplasia C: Cataract (50-85%) A: Aniridia-associated keratopathy due to LSCD: Vascular pannus, epithelial instability L: Lens subluxation G: Glaucoma: 50-75%: Angle dysgenesis 80%: Primary due to ADD of PAX6 (11p13) 20%: Sporadic: WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, developmental delays
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What are the differentials of a patient with aniridia?
1. Axenfield-Rieger anomaly: Iris strands and crossing angle 2. ICE syndrome: Iris atrophy/holes 3. Iris Coloboma: inferonasal --> part of CHARGE syndrome 4. Traumatic iridialysis: Unilateral, history of trauma
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What are the investigations of a patient with aniridia?
History: Poor vision, photophobia, family history of eye problems, childhood kidney tumours EUA: Slit lamp examination: megalocornea, gonioscopy, posterior segment examination OCT macula: foveal hypoplasia, optic nerve hypoplasia High-Frequency UBM: Visualise iris stump and ciliary body Systemic: 1. Renal US: screen every 3 months until 8yo 2. Genetic testing: PAX6 mutations (11p3 deletion)
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What is the management of a patient with Aniridia?
Conservative 1. Foveal/optic nerve hypoplasia/nystagmus: Tinted contact lenses, sunglasses 2. Foveal/optic/nystagmus: Ambylopia management Medical 1. Glaucoma: IOP lowering drops 2. LSCD/AAK: Lubricants Surgical 1. Cataract: High risk of zonular instability and aniridia fibrosis syndrome (membrane across posterior IOL surface) 2. Glaucoma surgery: GDDs/Cyclodiode 3. LSCD/AAK: Keratolimbal allograft, boston keratoprosthesis
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What are the syndromic associations of colobomas?
CHARGE syndrome: Coloboma, Heart Defects, Atresia, Retardation, Genital abnormalities, Ear abnormalities
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What are the ocular manifestations of colobomas?
1. Eyelid, iris, lens, chorioretinal, optic nerve coloboma 2. Microphthalmia 3. Nystagmus 4. Cataract 5. Glaucoma 6. Retinal Detachment
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What are the investigations for a patient with fungal keratitis?
1. Corneal scraping - Gram Stain & Giemsa staining - Grocott's Methenamine Silver (Fungal hyphae) - Calcofluor White: If flourescent microscopy available - Cultures: Sabouraud Dextrose agar at room temp 2. AS/OCT, Confocal Microscopy: Visualise fungal hyphae deep in stroma if surface too scared 3. Anterior chamber tap for PCR and culture 4. Corneal biopsy
220
What are the differentials of a patient with Terriens Marginal Degeneration?
1. Mooren's Ulcer: Painful, active epithelial defect with overhanking edge 2. PUK: Inflammatory, painful, associated with scleritis, shows active stromal loss/ulceration 3. Arcus Senilis: Lucid interval at the limbus and does not involve corneal thinning 4. Dellen: Localised dehydration thinning
221
What are the complications of a patient with a radial keratotomy?
1. Hyperopic Shift - cornea flattens decades after surgery --> hyperopia 2. Diurnal instability: Fluctuations in IOP and corneal hydration --> refractive error change between morning and evening 3. Incision Gaping: Scars never reach full tensile strength of normal cornea --> prone to traumatic rupture 4. Infectious Keratitis
222
What are the risk factors of keratoconus?
RUB EYES 1. Rubbing eyes: Strongest risk factor 2. UV exposure 3. Behavioural atopy: Eczema/asthoma 4. Ehlers-Danlos syndrome/Marfans/Down's syndrome/Osteogenesis Imperfecta 5. Young age 6. Excess contact lens wear 7. Sleep apnoea 8. Others: Previous craniosynstosis, previous LASIK
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What are the clinical findings of keratoconus?
Vogt striae: Vertical stress lines in deep stroma --> disappear with gentle pressure on globe Fleischer ring: Iron deposition at base of cone, seen with blue cobalt light Munson sign: V-shaped lower indentation in downgaze Rituzzi sign: conical light reflex on nasal limbus Retinoscopy signs: Scissoring reflex, oil droplet reflex
224
What are the differentials of a patient with keratoconus?
1. Corneal ectasias: Pellucid : Bilatearl inferior corneal thinning with max protrusion just superior to thinning, Crab-claw appearance on power map on pentacam 2. Keratoglobus: Bilateral globular protrusion of cornea --> generalised thinning more marked in periphery 3. Post-traumatic ectasia (Post-LASIK) 4. Protrusion of cornea subsequent to corneal thinning
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What ocular conditions are associated with keratoconus?
Anterior: VKC, Floppy eyelid syndrome Posterior: LCA, RP,
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What are the investigations of a patient with keratoconus?
History - Atopic disease - Hx of connective tissue disorders Examination - Lids: Floppy eyelid syndrome, upslanting palpebral apeture (Downs) - Conjunctiva: Papillae, Horner-Trantas Dots (VKC) - Sclera: Blue sclera (osteogenesis imperfecta) - Lens: Ectopia lentis (Marfan's, EDS) - Retina: Bone spiculed pigmentation, - Eye movements: nystagmus (LCA) - Refraction: Scissoring, oil droplet reflex Investigations Corneal topography (Rabinowitz-McDowell indices) - Central corneal power > 48.7D - Sim-K Astigmatism > 1.5D - Inferior-superior dioptric asymmety >1.2 - Skewed radial axes (SRAX) >21 degrees Inferior steeping, asymmetric bow-tie, skewed radial axes Pachymetry: Reduced central thickness: Thinnest point (inferior/paracentral) Amsler-Krumeich classification based on K readings, refraction, scarring and corneal thickness Stage 1: Mild astigmatism Stage 2: Myopia and thinning Stage 3: Marked ectasia Stage 4: Central scarring
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What is the management of a patient with keratoconus?
Conservative 1. Spectacles: Regular astigmatism 2. RGP lenses: Irregular astigmatism Surgical 1. CXL : Progressive keratoconus to strength stromal collagen and prevent progression: Dresden protocol 2. ICRS: Intrastromal corneal ring segments to remodel and flatten central cornea. Indications: Clear central cornea, contact lens intolerance, >400microns centrally 3. Keratoplasty (PK/DALK - if normal DM). Indications: Intolerance to CL, severe irregular astigmatism, significant stromal scarring affecting visual axis
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What are the clinical features of pellucid marginal degeneration?
1. Inferior peripheral thinning with ectasia above thinning 2. Irregular thinning 3. Inferior crescentic thinning with no vessels/lipid 4. High against-the-rule astigmatism
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What are the investigations of a patient with pellucid marginal degeneration?
Corneal topography: Crab-claw pattern Pachymetry: Inferior peripheral thinning Anterior segment OCT: Location and extent of thinning
230
What are the clinical features of post-LASIK ectasia?
6 months --> 10 years later: Progressive inferior corneal steepening and irregular astigmatism progression (myopic shift after LASIK) 1. Residual stromal bed <250-300 microns - highest risk factor 2. Posterior elevation on tomography MANAGEMENT: EARLY CXL to halt progression
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What are the investigations of a patient with scleritis?
Examination: - 10% blanching with phenylephrine - Posterior scleritis (T-sign on US), choroidal folds, choroidal effusions, exudative RD, CMO, optic disc oedema Systemic examination - Hands: MCP/PIP joints, any deformities - Rashes: SLE (discoid, malar rash), PAN (livedo reticularis) Bloods Autoimmune screen: c-ANCA (GPA, PAN), p-ANCA (Churg-Strauss syndrome/EGPA), ANA (SLE), Rheumatoid Factor, anti-CCP (RA), HLA-B27 (IBD) CXR: pulmonary haemorrhage Urinalysis: Proteinuria and haematuria: GPA
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What are the clinical features of ocular cicatricial pemphigoid? SJS/TEN?
OCP: Foster's classification Stage I: Subepithelial conjunctival fibrosis Stage II: Fornix shortening Stage III: Sympblepharon formation Stage IV: Ankyloblepharon / keratinisation of surface Other: Entropion, Trichiasis Corneal: Punctate keratopathy, keratinisation, vascularisation SJS/TEN - pseudomembranous conjunctivitis - corneal ulceration - lid margin keratinisation, scarring - symblepharon, trichiasis, instable tear film - stromal neovascularisation, LESC failure - persistent dryness - keratoconjunctivitis sicca Trachoma (C Trachoma serovirus A-C) - Herbets Pits - Alrt's line - Cicatraicial lid changes
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What are the investigations of a patient with ocular mucous membrane pemphigoid? SJS/TEN/Trachoma
Histopathology: OCCP: 1. Conjunctival biopsy: Michel's transport medium from bulbar conjunctiva: Look for subepithelial fibrosis and chronic inflammation 2. Direct Immunoflourescence: IgG, IgA and C3 deposition in linear pattern across epithelial basement membrane SJS/TEN Conjunctival biopsy: Absence of goblet cells TEN: Skin biopsy (Referral to plastics/dermatology) Bloods OCCP: Anti-BP180, ELISA. SJS/TEN: Raised inflammatory markers Trachoma Seroversion A-C (chlamydia trachomatis)
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What is the management of a patient with ocular mucous membrane pemphigoid? SJS/TEN?
OCP Conservative: Treat sicca syndrome: Artificial tears, ointments, punctal occlusion (if Schirmer < 5 mins), treat blepharitis Medical: Blepharitis management: oral doxycycline 50-100mg for 6 weeks on, 6 weeks off Immunomodulation: Active and progressive cases: Mild - Dapsone + Oral prednisolone Moderate - MTX, AZT or MMF + oral prednisolone Severe: IV/PO Cyclophosphamide + oral pred Surgical Keratoprosthesis for stage 4 disease SJS/TEN MAIN: Refer to intensive burns unit for management Acute management Conservative: Frequent conjunctival irritation, PF lubricants Medical: topical steroids, placement of symblepharon ring Surgical: - Amniotic membrane graft/tarrsorrphaghy for persistent epithelial defect - Stem cell transplantation for LECS - Gunderson flap for impending perforation - Lamellar keratoplasty/PK if perf impending - Boston keratoprothesis for end stage Trachoma - Azithromycin 1g PO
235
What is the management of a patient with limbal stem cell deficiency?
Conservative: Treat underlying cause: MGD - warm compression lubricants Medical: trea underlying chronic inflammation, conjunctival scarring, symblepharon formation, trichiasis Surgical Partial: AMG + Sector conjunctival epitheliectomy Total: Conjunctival limbal autograft from contralateral eye, if bilateral involvement, then keratolimbal allograft
236
What are the differentials of band keratopathy?
1. Spheroidal Degeneration: Golden-yellow deposits in interpalpebral area 2. Salzmann nodular degeneration 3. Vortex keratopathy 4. Corneal dystrophy
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What are the investigations of a patient with band keratopathy?
History: Chronic JIA, uveitis, previous RD requiring silicone oil. Hx of gout, CKD, hypercalcaemia symptoms (stones, groans, moans), calcium supplements Ophthalmic: Anterior segment OCT: Astigmatism Bloods - U&Es, Calcium, Phosphorous, Uric Acid - ACE, PTH levels, endocrine referral - CXR: sarcoidosis
238
What are the treatment options for a patient with a pterygium?
Conservative: Monitor Medical: Lubricants, topical steroids or NSAIDs if inflammed Surgical Indications: recurrent episodes, cosmesis, decreased vision due to astigmatism/pterygium encroaching axis Simple - Excision with conjunctival autograft Recurrent: Conj autograft +/- AMG +/- mitomycin C Large: Lamellar keratoplasty
239
What are the differentials of a patient with a pterygium?
1. Pseudopterygium: secondary to ulcer or Terrien's marginal degeneration 2. Non-pigmented conjunctival lesion - CIN/OSSN 3. Phlyectenular keratitis 4. Pingueculitis
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What is the management of band keratopathy?
Conservative: - Treat underlying cause - Lubricants Surgical 1. EDTA chelation 2. PTK - residual opacity 3. Superficial keratectomy: If associated with fibrous pannus
241
What are the risk factors of developing diffuse lamellar keratitis? (Sands of Sahara)
Patient factors: Pre-existing dry eye or ocular rosacea Surgical factors: Flap creation, intraoperative epithelial defeects Environmental factors: Contaminants (powder from gloves)
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What are the clinical features of a patient with diffuse lamellar keratitis / Sands of Sahara?
sterile inflammatory reaction in the LASIK flap interface, typically occurring within the first few days after surgery, characterised by fine granular white infiltrates - Interface granular infiltrates - No epithelial defect - Peripheral then central - No AC activity DLK staging Stage 1: Peripheral interface only Stage 2: Central interface involvement Stage 3: Dense central interface with VA reduction Stage 4: Stromal melting
243
What are the differentials of a patient with diffuse lamellar keratitis / Sands of Sahara?
1. Microbial keratitis post Lasik/interface keratitis 2. Epithelial ingrowth post-LASIK 3. Interface-fluid syndrome
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What is the management of a patient with diffuse lamellar keratitis?
Medical: High dose topical steroids (pred acetate HOURLY), consider oral steroids Surgical 1. Flap lifting and irrigation 2. Keratoplasty in advanced cases
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What are the complications of LASIK?
Pre-operative: Poor patient selection Intraoperative - Flap related issues: Incomplete/irregular flap, button-hole, corneal perforation - Photoablation issues: Treatment decentration, interface debris Post-operative Early: 1. Dislodged flaps: Emergency repositioning +/- sutures + BCL to prevent epithelial ingrwoth 2. Diffuse lamellar keartitis: White sand like granular deposits without epithelial defect/AC activity --> treat aggresively with steroids 3. Epithelial ingrowth: Hazy around flap --> Flap lift and scrape if encroaching visual axis Late 1. Dry eye 2. Post-LASIK ectasia
246
What are the differentials of a patient with conjunctival intraepithelial neoplasia (CIN) ?
Benign: Conjunctival papillopa Pre-malignant: Limbal dermoid Malignant: OSSN, SCC, Lymphoma Other: Limbal stem cell deficiency
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What are the investigations of a patient with conjunctival intraepithelial neoplasia?
History: Risk factors - HPV, HIV, immunosuppresion, smoking, pale, UV Ophthalmic - Anterior segment: photos and OCT - Biopsy: Dysplastic changes (increased nuclear-to-cytoplasmic ratio, atypia) confined to epithelium - Impression cytology if biopsy not available
248
What is the management of a patient with conjunctival intra-epithelial neoplasia?
Conservative: Observe Medical: Topical chemotherapy with MMC, 5-FU considered if MMC intolerance Surgical: Excisional biopsy with wide margins and cryotherapy --> especially if leukoplakic variant
249
What are the clinical features of ocular rosacea / keratitis?
Eyelids: Telangiectasia, MGD, blepharitis, chalazion/stye Conjunctiva: Chronic hyperaemia Cornea: Punctate epithelial erosions, peripheral neovascularisation, peripheral infiltrates/thinning
250
What is the management of a patient with rosacea keratitis ?
Conservative: Warm compresses and lid hygeine Medical: - Management of dry eye - Oral tetracyclines: 50-100mg doxycycline daily - Short course of topical steroids (FML/loteprednol) for severe inflammation - Topical antibiotics (azithromycin) Surgical - Superficial keratectomy for vascularised/scarred corneas - Fine needle diathermy/Argon laser photocoagulation/anti-VEGF for corneal vascularisation
251
What are the clinical features of a patient with chemical injury?
- Conjunctival injection - Subconj haemorrhage - Corneal opacification, conjunctivalisation - Limbal ischaemia - Cicatricial changes --> sympblepharon, ankyloblepharon
252
What are the classifications used in grading ocular chemical injuries?
Roper Hall Classification: Cornea clarity, limbal ischaemia, prognosis Grade I: Clear, No LI, Excellent Grade II: Hazy iris visible, <1/3 LI, Good Grade III: Opaque iris obscured, <1/2 LI, Guarded Grade IV: Opaque: > 1/2 LI, Poor Dua Classification: Clock hours limbal involvement and conjunctival involvement
253
What are the differentials of a patient with an ocular chemical injury?
1. Limbal Stem Cell Deficiency 2. Other injuries: Thermal, UV radiation 3. Inflammatory: OMMP, SJS, PUK, Mooren's Ulcer
254
What is the management of a patient with a chemical injury?
1. Neutralisation of pH with irrigation (can use Morgan contact lens) 2. Evert lids and fornices --> remove retained matter Mild chemical injury: Topical antibiotics PF, Cycloplegia PF, BCL, lubricants PF, oral analgesia Severe chemical injury: Add topical steroid PF, Topical ascorbic acid, oral ascorbic acid and doxycycline. Manage IOP. Surgical 1. AMT: persistent epithelial defect, moderate/severe 2. Limbal stem cell transplant 3. Keratoplasty: Late stage only
255
What are the differentials of a patient with interstitial keratitis?
1. Microbial keratitis 2. Chemical injury / burns 3. Cicatricial: OCP/SJS/TEN/Trachoma, LSCD 4. Atopic: VKC, Ocular rosacea 5. Herpes simplex
256
What are the clinical findings of a patient with interstitial keratitis?
1. Salmon patch stromal haze with fluffy margins 2. Deep stromal vascularisation 3. Late: Lipid keratopathy and ghost vessels 4. Corneal scarring
257
What are the investigations of a patient with interstitial keratitis?
History: HSV cold sores, immunosuppression, contact lens wear (acanthomoeba), TB symptoms, hearing loss (syphilis and Cogan's syndrome), sexual history Ophthalmology - Acanthomoeba: scrape with non-nutrient agar (e coli) - HSV PCR - Anterior segment OCT Bloods - Syphilis: VDRL, FTA-ABS - HIV - TB: IGRA test, T spot/Quantiferon, AFB sputum culture - Lyme serology - Autoimmune screen - Cogans syndrome: FBC (eosinophilia), ESR (raised), hearing test Hutchinsons triad: Interstital keratitis, notched incisors, sensorineural deafness: Congenital syphilis
258
How would you manage a patient with interstitial keratitis?
If syphilis: IM benzathine penicillin G (2.4 million units IM weekly x 3 doses) If neurosyphilis: IV penicillin G 18-24 million units/day for 10-14 days Conservative - Glasses/contact lenses to improve astigmatism Medical - Inflammation: Steroids - Ciliary spasm: Cyclopegia - Underlying treatment: eg antivirals Surgical 1. Fine needle diathermy/mitomycin C for active corneal vessels 2. PK/DALK for improvement from scarring when inactive.
259
What are the clinical features of epithelial ingrowth post LASIK?
1. Discrete grey-white crescenteric opacity across LASIK flap margin
260
What are the differentials of a patient with epithelial ingrowth post LASIK?
1. Diffuse lamellar keratitis: Presents day 1-day 6 - more granular/diffuse 2. Microbial keratitis along flap edge 3. Interface Fluid syndrome (Pressure-Induced Stromal Keratopathy PISK): Interface fluid due to high IOP (steroid response) 4. Interface debris: Lint or surgical drape fibers
261
What are the risk factors for epithelial ingrowth post-LASIK?
1. Epithelial defect at the time of LASIK 2. Flap-related issues - Flap dislocation/trauma - Re-lifting the flap for enhancement procedures 3. Patient factors - Older age - History of EBMD - Prior corneal surgery Presents usually 1 month post surgery
262
What is the management of a patient with epithelial ingrowth post LASIK?
Observation 1. If < 2mm from flap edge and stable, no visual axis involvement 2. Contacts/glasses for irregular astigmatism Surgical 1. Flap lift and epithelial removal (mechanical debridement 2. If recurrence: 20% ethanol to stromal bed, PTK, sutures to secure flap, consideration of mitomycin C 0.02%.
263
What are the clinical features of infectious crystalline keratopathy?
1. Branching needle like opacities with crystalline/snowflake appearance in anterior or mid-stroma in a grafted eye 2. No associated corneal inflammation 3. Associated with chronic epithelial defect/suture tract Streptococcus viridans / staphylococcus / pseudomonas / fungi (Candida)
264
What are the main investigations of a patient with infectious crystalline keratopathy?
History: Ask about chronic topical steroid use, ask about topical cyclopsorine/immunosuppresion, previous graft surgery Investigations 1. Corneal scrape: Insufficient --> needs corneal stash biopsy or suture-track culture 2. Gram stain and culture 3. In vivo confocal microscopy: Highly reflective branching needle-like structures
265
What is the management of a patient with infectious crystalline keratopathy?
Medical management: Longstanding 1. Intensive antibiotics: Fortified vancomycin (50mg/ml) or Fluoroquinolones 2. Intrastromal antibiotics: Vancomycin into crystalline area 3. Reduce/stop steroids Surgical management 1. Laser (PTK/YAG) YAG laser can disrupt bacterial biofilm 2. Repeat PK once infection is refractory
266
What is