A comprehensive of all peripheral corneal diseases

1. PERIPHERAL
CORNEAL
DISEASES
P R E S E N T E R – D R S A D H W I N I M H
M O D E R ATO R – D R S H I VAY O G I R K U S A G U R

2. PERIPHERAL CORNEA
• Peripheral cornea - that portion located between the central 50% of the cornea
and the limbus
• Thickest region (upto 0.7mm)of the cornea
• Avascular, a vascular arcade originating from the anterior ciliary arteries extends
approx. 0.5 mm into clear cornea
• Presence of more Langerhans’ cells
• Higher concentrations of IgM and C1 (important in the activation of the classic
complement pathway)
• Its proximity to the limbus and conjunctiva results in a unique collection of
abnormalities.
• Thus, vascular inflammatory disorders, limbal infections, collagen vascular disorders,
neoplastic disease, and local degenerations may affect the peripheral cornea in a
distinctive way.

3. PERIPHERAL CORNEAL DISEASES
CONGENITAL
/DEVELOPMENTAL
/INHERITED
Lattice dystrophy type II
Wilson’s disease
Conea plana
Sclerocornea
Posterior embryotoxon
Axenfeld-Rieger
anomaly
INFLAMMATORY
/AUTOIMMUNE
Rheumatoid Arthritis
Polyarteritis Nodosa
Wegener’s
Granulomatosis
Marginal keratitis
Phlyctenular
keratoconjunctivitis
Mooren’s ulcer
Superior limbic
keratoconjunctivitis
Contact lens induced
DEGENERATIVE
DISORDERS WITHOUT
CORNEAL THINNING
Corneal Arcus
Lipid keratopathy
Band keratopathy
Spheroidal
degeneration
Limbal Girdle of VOGT
Hassall Henle bodies
DEGENERATIVE
DISRDERS WITH
CORNEAL THINNING
Furrow
degeneration
Terrien’s Marginal
degeneration
Pellucid Marginal
degeneration
Dellen
NEOPLASTIC
Dermoid
Benign squamous
neoplasms
Conjunctival
Intraepithelial
Neoplasia
Invasive Squamous
call carcinoma
Conjunctival
Melanoma

4. C O N G E N I TA L / D E V E L O P M E N TA L /
I N H E R I T E D C O N D I T I O N S

5. LATTICE DYSTROPHY TYPE II
• Meretoja’s syndrome or Familial Amyloid Polyneuropathy type IV
• Associated with Systemic amyloidosis
• Not a true corneal dystrophy
• Corneal changes are later in onset
• Favourable visual outcome
• Mutations in the Gelsolin (GSN) gene, on Chr 9 (9q34)
• OCULAR
– CORNEA – fewer lattice line, radially oriented and involve primarily the peripheral
cornea, with relative central sparing.
– Reduced or absent corneal sensitivity.
– Glaucoma and pseudoexfoliation with or without glaucoma are common

6. • Systemic manifestations
– Systemic amyloidosis – dermatochalasis, facial drooping along with
protruding lips – typical facies.
– Cranial and peripheral neuropathies such as facial paresis
– Orthostatic hypotension, dysfunction of perspiration
– Cardiac conduction abnormalities, carpal tunnel syndrome
– Histopathology - mutated gelsolin deposited in arteries, skin,
peripheral nerves, sclera, etc. A regular amyloid layer lies beneath a
normal-appearing Bowman’s membrane.
• Confocal microscopy
– Stromal haze with highly reflective deposits - amyloid.
– Pleomorphic basal epithelial cells, deposits seen just posterior to these
cells.

7. • MANAGEMENT
• Depends on the patient’s symptoms
• Recurrent erosions - patching, hypertonic agents, artificial tears, or a therapeutic contact lens
• Excimer laser phototherapeutic keratectomy - optional treatment for recurrent erosions and
superficial opacities

8. WILSONS DISEASE
• AR – Ch 13q14.3
• Accumulation of copper in most body tissues
• Kayser-Fleischer ring is present in approximately 95% of patients
– Yellow-brown or green ring located at the level of Descemet’s membrane in the
peripheral cornea with no clear interval
• Begins peripherally at Schwalbe’s line and progresses centrally.
• Gonioscopy - to locate the ring in its early stage
• Wilson’s disease - systemic D-penicillamine - prevents disease progression and
reduces the amount of copper already deposited in tissues.

9. CORNEA PLANA
• AR or AD. Gene locus – KERA gene (12q22)
• Cornea is flat - curvature <43D, typically 30-35D
• Alteration in tertiary structure of keratan sulfate proteoglycans
• Developmental arrest of corneal curvature relative to sclera
• Associated with sclerocornea or microcornea
• Angle closure glaucoma - 20%
• Other ocular - Arcus juvenilis, nonspecific corneal opacities, anterior segment
dysgenesis, uveal & retinal coloboma, aniridia, congenital cataract, ectopia lentis
• Systemic – osteogenesis imperfecta, Ehler Danlos syndrome type IV
• Management – correction of refractive error, monitor for associated findings

10. SCLEROCORNEA
• 50% -sporadic mutation, 50% - AD/AR/XLD
• During embryonic development from 2nd mesenchymal wave forming tissue that
resembles sclera instead of clear corneal stroma
• May occur in peripheral cornea of one or both eyes, may be complete with an
absence of clear cornea.
• Commonly associated with cornea plana (80%)
• Other ocular – Angle closure glaucoma, high refractive errors, blue sclera, Peter’s
anomaly, uveal & retinal coloboma, cataract and microphthalmos.
• Systemic – anomalies of skull & facial bones, external ear deformities, polydactyly,
testicular abnormalities, osteogenesis imperfecta.
• Management – few therapeutic options available – limited to control raised IOP,
subsequent keratopalsty.

11. POSTERIOR EMBRYTOXON
• AD
• Clear to white line in peripheral cornea – thickened & centrally displaced anterior
border ring of Schwalbe
• seen with gonioscopy as a fine ground-glass-like membrane
• When posterior embryotoxon exists without additional pathology it does not
require treatment

12. AXENFELD – RIEGER ANOMALY
• AXENFELD’S ANOMALY - AD
– Corneal clouding, posterior embryotoxon, prominent iris processes (iridocorneal adhesions to or
beyond schwalbe’s line)
– Glaucoma - 50%
– Axenfeld’s syndrome – when associated with systemic anomalies – hypertelorism, facial asymmetry,
hypoplastic shoulder
• RIEGER’S ANOMALY – AD/sporadic
– Atrophy of iris stroma and corneal abnormalities
– PAS, corectopia, ectropion uveae, pseudopolycoria
– Glaucoma - >50%
– Rieger’s syndrome – when associated with skeletal, cranial & dental abnormalities

13. • AXENFELD-RIEGER’S ANOMALY & SYNDROME
• ANOMALY
– Findings of both conditions – spectrum of anterior segment disorders
• SYNDROME
– When associated with systemic developmental defects
• M Bruce Shields – primordial endothelium is retained over parts of iris and angle late in
gestation. These undifferentiated cells contract – iris stromal thinning & hole formation.
• MANAGEMENT – aqueous suppressants, goniotomy, trabeculectomy with antimetabolites

14. I N F L A M M AT O R Y / A U T O I M M U N E
C O N D I T I O N S

15. RHEUMATOID ARTHRITIS
• A chronic inflammatory disease of unknown etiology, affecting primarily synovial
joints, and less frequently extra-articular tissues, such as the eye, pleura, pericardium,
and nerves.
• F>M
• Between 4th and 6th decades
• Presents as a symmetrical polyarthritis, involving small joints of the hands and feet, as
well as larger joints
• Presentation with ophthalmic disease - with severe, long-standing RA and high titers
RF
• Ocular manifestations – keratoconjunctivitis sicca, scleritis, sclerosing keratitis,
keratolysis

16. • Pathogenesis
• The etiopathogenesis of RA is unknown
Immunoglob
ulin &
Complement
deposition
within blood
vessel wall
Focal
areas of
wall
necrosis
Rheumat
oid
Vasculitis
Increase
in
permea
bility
causing
leakage
Chemotactic
factors,
Inflammatory
cytokines (IL-
1, TNFα)
Neutrophils recruited
at peripheral cornea
liberate proteolytic
and collagenolytic
enzymes
Corneal
destruction
Immune
mediated
Hypersensit
ivity
Cell
mediated
Hypersensi
tivity
scleritis
• High titers of rheumatoid factor -
higher incidence of extra articular
manifestations, more severe joint
destruction, and worse prognosis

17. • KERATOCONJUNCTIVITIS SICCA
• most common associated (15–25% of RA patients)
• gritty or sandy FB sensation, burning, irritation, mucus discharge,
and photophobia
• diminished tear meniscus, Schirmer test - decreased tear production
• Punctate epithelial keratopathy, mucus stranding, and filamentary
keratitis
• SCLERITIS
• 20% of RA patients, isolated episcleritis in up to 11%
• severe boring ocular pain and photophobia
• Anterior scleritis - diffuse, nodular, necrotizing scleritis with
inflammation and necrotizing scleritis without inflammation, and
posterior scleritis.
• exhibit zones of intense scleral inflammation, edema, and necrosis
with overlying epithelial defects
• Also referred to as scleromalacia perforans - Severe scleral thinning
diffuse anterior scleritis

18. necrotizing scleritis post cataract
suregery
filamentary keratitis
diffuse scleromalacia
perforans in a 70-year-old
patient with long-standing
RA
acute marginal stromal infiltrates
PUK with scleritis, epithelial
defect
limbal guttering, absence of
stromal infiltrate
PUK

19. • KERATITIS
• PUK – crescent shaped peripheral corneal thinning, a leading edge of stromal WBC infiltration,
epithelial defect overlying the ulceration, with scleral inflammation
• SCLEROSING KERATITIS – adjacent to an area of active scleritis.
• Peripheral cornea becomes thickened and opacified and may become secondarily vascularized
• ACUTE STROMAL - as stromal opacities with edema. The opacities may coalesce if left
untreated. Overlying epithelium may break down – PUK
• LIMBAL GUTTERING - epithelium remains intact and the extent of peripheral thinning may be
variable
• KERATOLYSIS
• an acute severe melting of the corneal stroma which can proceed to perforation.
• most commonly seen in patients with rheumatoid-associated necrotizing scleritis
• Loss of stroma occurs in regions of clear cornea and may progress to descemetocele formation.
keratolysis

20. • MANAGEMENT : therapy directed toward control of the systemic vasculitis
• System disease : NSAIDs, corticosteroids and DMARDs
• KCS : ocular surface lubrication
– Moderate doses of topical steroids - underlying inflammatory component.
– Topical ciclosporin A drops BD for management of dry eye have
– Nutritional supplementation such as omega-3, omega-6, linoleic acid, linolenic acid, and
other essential fatty
– Punctal occlusion, Medial canthoplasty and/or lateral tarsorrhaphy can be performed
• KERATITIS : Oral corticosteroid and topical ciclosporin A play a major role
– Topical steroids - with great caution in furrowing with epithelial defects and sterile keratolysis
– suture or chemical tarsorrhaphy - persistent epithelial defects
– Keratolysis - a patch graft
• NON NECROTIZING SCLERITIS : mild to moderate - include oral NSAIDs
– Indometacin (25 mg) or ibuprofen (600 mg) TID or naproxen (500 mg) BD
– Topical steroids can be used initially in combination with oral NSAID
– Anterior scleritis - subconjunctival corticosteroid injections

22. • NECROTIZING SCLERITIS : Infectious etiologies must be initially ruled out
– Topical ciclosporin A, Protease inhibitors such as tetracyclines may be considered
– Acute inflammation - oral prednisolone at an initial dose of 1 mg/kg/day, with a subsequent slower
tapering
– High-dose IV pulse methylprednisolone - 1 g per day for 3 consecutive days followed by oral therapy
– Scleromalacia perforans - observation, eye protection, and therapeutic modulation of systemic disease
• IMMUNOSUPPRESSIVES : in recalcitrant cases of scleritis or keratitis, including PUK, and
keratolysis
– Immunomodulators - antimetabolites, alkylating agents, T-cell inhibitors, and biologic agents.
• SURGICAL MANAGEMENT :
– Cyanoacrylate glue application with oversized BCL, donor scleral grafting, AMT, lamellar or penetrating
keratoplasty, aggressive lubrication and tarsorrhaphy may enhance surgical success.

24. POLYARTERITIS NODOSA
• Periarteritis Nodosa - vasculitis of small and
medium-sized arteries leading to multiple organ
system disease
• middle age, M>F
• etiology is unknown
• Ocular (10– 20%) - secondary to HTN or vasculitis
of retinal vessels.
– Direct nerve involvement - EOM palsies and optic
nerve infarction
– Orbital involvement - pseudotumor-like lesion
– Conjunctival nodules - edema and necrosis of
conjunctival vessels & uveitis
• Anterior segment - scleritis, sclerokeratitis, and
PUK
weig
ht
loss
≥4
kg
testic
ular
pain
livedo
reticul
aris
Myalg
ia
PMNs in
artery
walls in
biopsy
Mon/p
olyneur
opathy
High
BUN or
Creatini
ne
HTN
with
DBP>90
mmHg
HBSAg
or Ab
in
serum
• Treatment - systemic disease
• Corticosteroids, Immunosuppressives,
which have markedly improved the
outcome
ANY 3 OF,

25. WEGENER’S GRANULOMATOSIS
• Systemic vasculitis characterized by necrotizing and
granulomatous inflammation of the vessels of the
upper and lower respiratory tract, glomerulonephritis,
and other organ involvement with small vessel
inflammation.
• Etiology is unknown
• Mid-40s, M:F=2:1
• Can present in children, with ocular involvement as
the initial manifestation
• Ocular (58%) :
– Proptosis with orbital pseudotumor or orbital
inflammation, nasolacrimal duct obstruction
– Retinal vasculitis and ischemic optic neuropathy
Upper airway
symptoms of
sinusitis,
Pulmonary
infiltrates on CXR
normocytic
normochrom
ic anemia
Elevated
ESR, positive
RF
Circulating
immune
complexes,
ANCA
Non
specific
complaint
s
Any 2 of 4

26. Focal necrotizing scleritis with
peripheral keratitis
Residual grainy anterior stromal opacities
Peripheral anterior stromal and
subepithelial infiltrates
necrotizing scleritis

27. • Anterior segment - conjunctivitis, episcleritis, scleritis, keratitis, and uveitis, dry eye,
• Focal necrotizing scleritis with peripheral keratitis, Peripheral corneal subepithelial
infiltrates may be associated and may develop into grainy anterior stromal opacities
• corneal guttering may occur
• MANAGEMENT
• Systemic - oral Prednisone (1 mg/kg) and cyclophosphamide (2 mg/kg) orally are
used for initial therapy, with the prednisone tapered after 2 to 4 weeks but continued
over many months while the cyclophosphamide is continued for at least 1year
• Alternatively, ‘pulsed’ IV methylprednisolone (500 mg to 1 g) may be used
• Artificial tears for dry eyes and topical corticosteroids for mild conjunctivitis and
episcleritis

28. MARGINAL KERATITIS
• Catarrhal infiltrates or ulcers
• Peripheral corneal disorder characterized
by inflammatory infiltration that may lead
to ulceration
• Represent an antibody response to toxins
rather than direct bacterial invasion
• Associated with Staphylococcal infection,
Wegener’s granulomatosis, soft contact
lens, phthiriasis, topical antimicrobials,
herpes simplex keratitis, Moraxella,
Haemophilus, and other streptococcal
species
• Gram and Giemsa staining of corneal
scrapings show neutrophils but no
organisms
• Pain, photophobia, FB sensation,

29. • Localized peripheral stromal infiltrates, (2, 4, 8, 10 o’clock positions)
• Parallel to the limbus and separated from the limbus by 1–2 mm of clear cornea
Infiltrates can
coalesce
Broader lesions
Epithelial break
down
Ulceration
• Spontaneous resolution in 2 to 3 weeks
• Recurrences are common
• MANAGEMENT
• Topical corticosteroids (i.e. fluorometholone, loteprednol, and prednisolone acetate) are
the mainstay in acute cases.
• Ulceration - a broad-spectrum topical antibiotic should be used briefly prior to starting
topical corticosteroids
• Treatment of blepharitis, if present

30. HSV Marginal Keratitis STAPH Marginal Keratitis
Etiology Infective Immunologic
Epithelial defect Present Generally absent
Progression Centrally Circumferentially
Limbal Injection More Minimal
Lucid interval Absent Present
Neovascularization Present Absent
Blepharitis Absent Present
Location Any meridian 2, 4, 8, 10 O’clock

31. PHLYCTENULAR KERATOCONJUNCTIVITIS
• Represent a bacterial allergy to a number of antigens - tuberculoprotein and
staphylococcal.
• Greek word phlyctena, which means ‘blister’
• 85% - positive to the tuberculin skin test
• Other - Chlamydia, Coccidioides, Candida, HSV and parasites
• In teenage years with a preference towards females
• Type IV hypersensitivity reaction to a specific antigen and thus requires,
– sensitization of the cornea or conjunctiva to antigen
– repeated exposure
• In TB-induced PKC, sensitization – bacteremia with a past pulmonary infection. Attack
of PKC is - when TB antigens are presented to eye either from an
endogenous/exogenous source
• In Staph-induced PKC, sensitization - via many routes, the same bacterial antigens
will be lingering locally to trigger a hypersensitivity attack.

32. • Subsequent corneal phlyctenules can arise at the central edge of a pannus from prior
attacks. Multiple times - phlyctenule can appear to ‘wander’ across the cornea.
• Multiple small phlyctenular lesions may be distributed diffusely over the entire corneal
surface (i.e. miliary phlyctenulosis).
• Histological - monocyte-derived cells with a moderate amount of T lymphocytes
CONJUNCTIVAL LESION CORNEAL LESION
Symptoms Tearing, FB sensation Same symptoms but with more
intensity and photophobia
Phlycten near the limbus, as pink fleshy
nodule, 1-2mm diameter with
conjunctival injection
at the limbus, small white nodule
with conjunctival injection
Course of
phylcten
Central, superficial portion turns
yellow or gray & soft, sloughs off,
creating an ulcer, which then
reepithelializes
undergo necrosis, forming a
marginal ulcer
Resolution 1-2 weeks Triangular shaped anterior
stromal scarring with broad
pannus

33. • MANAGEMENT
• Prednisolone acetate 1% - six times daily can be used as a starting dose. Tapering
based on the clinical response.
• A topical antibiotic is recommended - if a corneal lesion has an overlying epithelial
defect
• Conjunctival lesions can be treated right away with corticosteroids.
• TB-induced PKC - treatment of the primary infection.
• Minimizing Staphy colonization of lid margins - removal of bacteria with lid scrubs and
the use of topical antibiotic ointments, Oral tetracyclines

34. MOOREN’S ULCER
• A painful, progressive, chronic ulcerative keratitis that begins peripherally and
progresses circumferentially and centrally.
• An idiopathic disease occurring in complete absence of any diagnosable systemic
disorder that could be responsible for the progressive destruction of the cornea.
• The disease is strictly a peripheral ulcerative keratitis (PUK), with no associated scleritis
• Between 40 and 70 years, M>F
• Unilateral involvement - more common overall
• Interpalpabral limbus – inferior - superior limbus
• Association with hepatitis c, hookworm infestation
Wood and Kaufman
LIMITED/BENIGN/TYPICAL ATYPICAL/MALIGNANT
Unilateral, Older patients Bilateral, Younger patients
Mild – moderate symptoms Intense symptoms
Responds well to medical and
surgical treatment
Poor response to therapy
Progresses leading to perforation

35. Autoantigen - ‘cornea-
associated antigen’,
Trauma/infection may
alter normal corneal
antigens
Taken up by
macrophages
move through the
conjunctival vessels
to the peripheral
LNs
T-cell activation,
differentiation, and
proliferation
lymphocytes return to
vessels and cause
ocular inflammation
corneal edema allows
easier diffusion of
immune components
into pericentral from
limbal vessels
cornea is damaged,
liberating more altered
corneal antigens
Aggravate the process
until corneal stroma is
completely destroyed
PATHOGENESIS

36. • Cellular population found in the conjunctiva adjacent to the ulcer is primarily plasma
cells, lymphocytes, and macrophages
• Upregulation of various adhesion and co-stimulatory molecules in epithelial cells in the
conjunctiva
• Systemically, Mooren’s ulcer patients have a decrease in suppressor T cells relative to
number of helper T cells, elevated IgA levels, and circulating IgG antibodies and
immune complexes.
• Molecular mimicry - infecting agent stimulating an autoimmune response to corneal
antigens through cross-reacting epitopes
• SYMPTOMS
• Redness, tearing, photophobia
• Pain – incapacitating, out of proportion to the inflammation.
• Decreased VA, which may be secondary to associated iritis, central corneal
involvement, or irregular astigmatism

37. • SIGNS
• Concentric to limbus
• Leading edges – undermined and
deepithelialized
• Conjunctiva, sclera - inflamed &
hyperemic.
• Spreads in 3 directions: peripherally,
centrally, and rarely into sclera
• Behind the advancing edge, healing
may take place in the form of corneal
epithelialization and vascularization
• Associated with this is scarring and
thinning
• Advanced case - most of the cornea is
lost, leaving behind a central island
surrounded by area of grossly thinned,
scarred, and vascularized tissue.
Crescent-shaped graywhite
infiltrate in peripheral cornea
Epithelial breakdown & stromal
melting
Characteristic chronic crescent-
shaped peripheral ulcer
Progresses circumferentially &
centrally, creating an overhanging
edge at its central border
Over time it may involve the
limbus

38. • It is a diagnosis of exclusion
• CBC, Platelet counts, ESR, RF, ANA, ANCA, CXR, LFT and fluorescent treponemal antibody
absorption test.
• TREATMENT :
• Aim : Stop the ulcerative process and allow re-epithelialization of cornea
• Topical corticosteroids - Aggressively on an hourly basis
• Topical prophylactic antibiotics
• Cycloplegic medications
• Signs of reepithelialization - steroid therapy is tapered gradually over months.
• Systemic Immunosuppression
• Oral Prednisolone is 1–1.5 mg/kg body weight/day, adjusted according to the severity
• Cyclophosphamide (2 mg/kg/ day), Methotrexate (7.5–15 mg once weekly), and
Azathioprine (2 mg/kg/day), Ciclosporin A (3–4 mg/kg/day)
• Topical Ciclosporin 0.5%, 4 to 6 times daily - penetrates corneal epithelium to reach the
stroma

39. • Treating Chronic Hepatitis C patients - subconjunctival injections of interferon alpha-
2b over a 6-month period
• SURGICAL PROCEDURES :
• Conjunctival resection - Removes involved conjunctiva, adjacent to ulcer, up to 2
clock hours on either side and extends 3–4 mm from the limbus.
• Keratoepithelioplasty : Ulcerated corneal tissue and adjacent conjunctiva are removed
and donor corneal lenticules with intact epithelium are sutured onto bare sclera
• Lamellar keratectomy : 4/5th of the corneal thickness is excised. Controls the
inflammatory process by removal of the corneal antigenic stimulus
• Lamellar keratoplasty - removal of necrotic ulcerative cornea and reconstruction of
anatomical structure using lamellar donor lenticule
• Tissue adhesive and BCL : In cases of perforation or impending perforation
• Tectonic grafts (patch graft or PK) : Large perforations.

40. PUK MOOREN’S ULCER
Pain Usually absent Intense Pain
Scleral inflammation Present Absent
Defect Leading edge with
stromal WBC infiltration
Undermined &
overhanging edges
Associated with systemic
conditions
Diagnosis of exclusion
Clear zone Exists Doesn’t exist

41. SUPERIOR LIMBIC KERATOCONJUNCTIVITIS
• An external ocular disorder characterized by a well-
defined pattern of superior bulbar conjunctival injection
• Between 20 and 60 years, Bilateral.
• M:F=2:1
• Associated with thyroid dysfunction
• The pathogenesis of SLK is unknown
• Abnormal friction between the upper eyelid and the
superior bulbar conjunctiva, combined with redundancy of
the superior conjunctiva
• Abnormal eyelid pressure on the globe, abnormal eyelid
closure
• SYMPTOMS - burning sensation, photophobia and FB
sensation, feeling of ocular dryness and some will
complain of redness

42. • SIGNS - Injection of the superior bulbar conjunctiva contiguous
to the limbus, injection of palpebral conjunctiva
• Fine punctate epitheliopathy – over superior 20% of cornea and
bulbar conjunctiva, stains with fluorescein, rose bengal or
lissamine green.
• Filaments
• Bulbar conjunctiva - folds, excessive movement &
conjunctivochalasis
• Micropannus involving the superior limbus, in conjunction with
the ‘proliferation’ or ridge
• Papillae, follicles, or scarring of the upper palpebral conjunctiva
• Tests for tear dysfunction - evidence of lacrimal insufficiency
• Histopathology – shows keratinization of epithelium with
acanthosis, dyskeratosis, leukocytic infiltration.

43. • MANAGEMENT :
• Swabbing with silver nitrate, Therapeutic contact lenses
• Topical Cyclosporine 0.5% and 0.05%, Autologous serum, Vitamin A, Cromolyn
sodium, lodoxamide tromethamine and Acetyl cysteine - beneficial.
• Supratarsal Triamcinolone injection
• Liquid nitrogen and Thermal cautery have been applied to both bulbar and palpebral
conjunctiva
• Simple scraping of palpebral conjunctiva was just as effective
• Conjunctival resection, with and without AMT, Conjunctival advancement and
Conjunctival fixation sutures – reduce redundancy and mobility of conjunctiva

44. CONTACT LENS INDUCED PERIPHERAL
CORNEAL ULCER
• RISK FACTORS :
– Lens usage – overnight use
– Acanthamoeba, Pseudomonas, Fusarium
– Lens solution – Thiomersal
– topping off of old solutions in the case
– infrequent replacement of the storage
case
– failure to wash hands before handling
lenses
– exposure of the lens or lens case to tap
water, including swimming or showering
while wearing it
– extended wear of any soft contact lens or
a tight lens – acute or chronic hypoxia
• SYMPTOMS :
– moderate to severe pain
– Sterile corneal infiltrates - mild discomfort
– Active infection - Increasing pain
• SIGNS :
– Distinguishing noninfectious infiltrates from
microbial keratitis
– AC reaction, mucous discharge, an
overlying epithelial defect - microbial

45. CORNEAL
INFILTRATES
INFECTIVE STERILE
Size Larger Smaller
Progression Rapid Slow
Epithelial defect Very common –
larger
Less common – small
if present
Pain Moderate to severe Mild
Discharge Purulent Mucopurulent
Number of lesions Single Multiple
AC Reaction Severe Mild
Location Central Typically peripheral

46. • Acute hypoxia - Stromal & epithelial edema, no epithelial defect, mild AC reaction, with
or without a hypopyon
• Chronic hypoxia - conjunctival injection, corneal neovascularization, scarring, lipid
keratopathy
• Multiple peripheral subepithelial sterile corneal infiltrates, chronic or recurrent follicular
conjunctivitis, superficial scarring
• DIAGNOSIS – smears and cultures to rule infectious etiology
• TREATMENT
• Discontinuing lens wear until symptoms and signs resolve, Replacing the contact
lenses, and switching to a hydrogen peroxide disinfection system
• Refitting tight lenses and avoiding extended-wear lenses - if hypoxia
• Microbial keratitis - intensive broad-spectrum antibiotic therapy - fluoroquinolones,
topical fortified tobramycin and cefazolin or vancomycin
• Sterile infiltrates - Low-dose antibiotics & steroids.

47. DEGENERATIVE
DISORDERS
WITHOUT CORNEAL
THINNING

48. CORNEAL ARCUS
• A degenerative change involving lipid deposition in
peripheral cornea
• ARCUS SENILIS :
• Bilaterally symmetric, progressive
• Prevalence increase with age
• M>F, Black males
• Gray to yellow arc, first in the inferior then the superior
cornea
• As the deposition progresses, the arcs meet - complete ring
• Peripheral border - Sharp, Central edge - more diffuse
• A lucent zone (lucid interval of Vogt) to the limbus, due to
vessel’s ability to reabsorb the lipid in this area
• Lipid is first deposited at Descemet’s membrane and
subsequently at Bowman’s layer.
• Gerontoxon
• Aged population
Arcus
Senilis
• Anterior Embryotoxon
• Young population
Arcus
Juvenilis

49. • Histopathologically – an hourglass appearance as the opacity extends into the
corneal stroma
• Contains – cholesterol, cholesterol esters, phospholipids, and neutral glycerides
• LDL cross the capillary wall endothelium of blood vessels, in the presence of elevated
circulating LDL
• Corneal arcus has no visual significance, and thus no necessary treatment
• ARCUS JUVENILIS :
• Premature corneal arcus formation
• Hyperlipoproteinemia types IIa and IIb
• Primary or secondary
• Xanthelasma may be present
• Nephrotic syndrome, hypothyroidism, increased cholesterol intake, obstructive
jaundice, and diabetic ketoacidosis

50. LIPID KERATOPATHY
• Primary form (rare) and secondary form (common)
• F : M = 70 : 30
• Secondary from occurs in vascularized cornea - associated with
interstitial keratitis, trauma, corneal hydrops, corneal ulceration
• Primary form - no prior history of - trauma, family history of similar
conditions, corneal vascularization, and no known disorders of lipid
metabolism
• Either central or peripheral
• Yellow or cream deposit with intermittent crystals, in superficial and deep stroma
• Fan out in association with the blood vessels
• Contains - Cholesterol, neutral fats, phospholipids, free fatty acids, fibrin, and
glycoprotein
• MANAGEMENT :
• Superficial argon laser
• Lipid will resolve with subconjunctival Bevacizumab.
• Progresses - penetrating keratoplasty

51. BAND KERATOPATHY
• Term band keratopathy - Calcific form of keratopathy
• Occurs in calcific and noncalcific forms
• A corneal degeneration with deposition fine basophilic
calcium hydroxyapatite or urate at the level of Bowman’s
layer
• Calcium deposits are white, while Urate deposits are
brown.
• Alteration of corneal metabolism - increased tissue pH and
precipitation of calcium
• Evaporation of the tears because of exposure - calcium
precipitation
• Early stages - asymptomatic. Later stages - decreasing
vision, FB sensation, tearing
• Begins at the periphery (3 and 9 o’clock)
• Centrally in cases of chronic ocular inflammation.

52. • Peripheral form - sharp peripheral edge separated from limbus by a lucent zone
• Central edge - fades into normal cornea
• Gray opacity – white and chalky with Lucent holes
• A complete band from limbus to limbus may form in later stages.
• A medical work-up should include serum calcium, phosphorus, uric acid, and RFT
• MANAGEMENT
• Symptomatic - Application of Ethylene Diamine Tetraacetic acid (EDTA 0.05 molar
concentration)
• Amniotic membrane has been used after the primary surgical removal of band
keratopathy
Topical Anesthetic
Epithelium
removed
EDTA applied
to calcific
areas
Diamond
burr or No.
15 blade is
used to
remove any
residual
calcium

53. SPHEROIDAL DEGENERATION
• may occur in the cornea or the conjunctiva
• Climatic droplet keratopathy, Hyaline degeneration, Labrador keratopathy
• Primary corneal form •Secondary corneal form • Conjunctival form
• Increases with age and varies with geographic location –high sunlight exposure and high
winds.
• M:F=2:1
• Drying of the cornea and repeated corneal trauma are also risk factors
• Secondary forms - corneal scars, lattice dystrophy and glaucoma
• Shows fine droplets, yellow or golden in color, beneath the epithelium
• Droplets appear oily, although they are not of lipid origin

54. • Protein material, of unknown source - action of ultraviolet light on proteins from
limbal vessels
• Patients with visual loss may - superficial keratectomy
• Lamellar or penetrating keratoplasty have also been described
PRIMARY FORM
GRADE 1 GRADE II GRADE III
Fine shiny droplets Large corneal nodules
Only peripherally Central cornea
No symptoms vision ≥ 20/100 (6/30) vision ≤ 20/200 (6/60)

55. LIMBAL GIRDLE OF VOGT
• A crescentic yellow-to-white band found in the interpalpebral limbus
• Usually symmetric, occurring in nasal limbus more frequently
• Incidence increases with age
• Asymptomatic - No treatment is required.
WHITE LIMBAL GIRDLE OF VOGT TYPE I
– b/l
WHITE LIMBAL GIRDLE OF VOGT TYPE
II
Narrow band of chalkywhite opacities
and clear areas (Swisscheese)
White fleck deposits - nasally and
temporally
Central border is relatively sharp Centrally - irregular linear extensions
Separated from the limbus by a narrow
lucent area
No clear space
Calcium deposits, superficially below
epithelium
In the limbal area peripheral to
Bowman’s layer

56. HASSALL HENLE BODIES
• excrescences of Descemet’s membrane found in peripheral cornea
• Aging changes
• Due to age related endothelial cell loss
• Nodular areas of thickening of DM in the periphery extend into the anterior chamber
• Best seen on specular reflection
• Dark, round holes in the specular reflection
• They are clinically insignificant – no treatment

57. DEGENERATIVE
DISORDERS WITH
CORNEAL THINNING

58. FURROW DEGENERATION
• Rare degeneration
• Elderly patients
• Corneal thinning (shallow) within the clear band of cornea between the
limbus and the corneal arcus
• In Peripheral cornea under intact epithelium.
• Not associated with vascularization, inflammation, progression or
perforation
• The thinning may be an illusion caused by the arcus senilis. True thinning
also can occur.
• Rarely, there is an astigmatic effect on vision that can be treated with
refraction.

59. TERRIEN’S MARGINAL DEGENERATION
• A rare peripheral corneal inflammatory condition with stromal
thinning with a steep central wall and peripheral sloping
• Unknown etiology
• At any age, commonly between 20 and 40 years
• M:F = 3 : 1
• Bilateral and symmetric
• Begins superonasally with fine punctate opacities in anterior
stroma
• Superficial vascularization from the limbal arcades leading to
lesion
• Lucent area to the limbus
• A gutter then forms between the opacity and limbus
• Peripheral edge of the gutter gently slopes, whereas the central
edge is often steeper
• A yellow-white zone of lipid- at the advancing edge of the

60. • Gutter - vascularized and wider
• Extend circumferentially or centrally
• Quiescent type - older patients, asymptomatic for a long time
• Inflammatory - younger age groups, recurrent episodes of inflammation, episcleritis,
or scleritis
• Astigmatism may be produced
• Histopath - fibrillar degeneration of collagen. Bowman’s layer is fragmented or absent.
Breaks in Descemet’s membrane may be seen in thinned areas
• Perforation - spontaneously or secondary to minor trauma
• MANAGEMENT
• Astigmatism may be corrected with refraction.
• Lamellar or eccentric penetrating grafts in cases of large astigmatism,
impending perforation, or perforation

61. PELLUCID MARGINAL DEGENERATION
• Peripheral corneal ectatic disorder characterized by a band of thinning 1–
2 mm in width, typically in the inferior cornea, extending from the 4 to 8
o’clock position
• Atypical cases - >4 clock hours, superior location
• 2nd – 5th decade, bilateral, M=F
• Association with VKC, atopy, and frequent rubbing
• Associated with keratoconus in the same or alternate eye.
• Maximal corneal protrusion typically occurs just superior to the area of
thinning - ‘beer belly’ contour
• Shift in the axis of astigmatism from against-the rule, superiorly, to with-
the-rule, near the point of maximal protrusion
• Clear and avascular cornea, with no infiltrate or deposition
• Stromal scars - at the level of Descemet’s extending into mid stroma

62. • Acute hydrops can occur - edema, scarring, and vascularization
• Topography - classic crab-claw or C configuration - flattening of the
central cornea with inferior peripheral steeping
• Videokeratograph - superior flattening with against-the-rule
astigmatism superiorly and with-the-rule astigmatism inferiorly
• MANAGEMENT
• Large diameter, rigid gas-permeable contact lenses can be tried
• Hybrid lenses, such as the SoftPerm lens, have been used successfully
• Poor candidates for surgery
• Large-diameter or eccentric PK - to encompass the area of peripheral
thinning
• 2-stage procedure - large-diameter lamellar keratoplasty is followed
by a smaller central PK
• Alternative - Thermokeratoplasty, crescentic lamellar keratoplasty, and
crescentic or wedge excision

65. DELLEN
• Fuchs’ dimples
• Age-related change or secondary to other ocular abnormalities
• May last only 24 to 48 hours
• Commonly in temporal periphery
• Tissue thinning due to inadequate tear coverage & drying adjacent
to a paralimbal elevation
• Saucer-like areas of thinning or excavation in the corneal surface
• Epithelium is usually intact, base can be gray or hazy
• If Epithelium – not intact - infection and perforation are critical
concerns.
• Histopathologically - thinning of the epithelium, Bowman’s layer,
and anterior stroma
• Treatment with ocular lubricants or pressure patching will accelerate
the healing process
• After cataract extraction
• Strabismus surgery
• Glaucoma filtering
surgey, filtering blebs
• Episcleritis
• Conjunctivitis
• Pterygium/pingecula
• Dermoids
• Anesthestics - Cocaine
• Lagophthalmos

66. NEOPLASTIC

67. DERMOID
• Solid benign congenital tumor that frequently arise at the inferotemporal
corneoscleral junction.
• They are classified as choristomas
• Contain ectodermal derivatives, such as hair follicles, sebaceous and sweat glands
embedded in connective tissue and covered by squamous epithelium
• Commonly as single lesions but may be multiple
• Unilateral or bilateral
• Yellowish-white, 2 to 15 mm in diameter, solid, vascularized, elevated nodules at the
corneal limbus
• Extend into deeper stroma without affecting Descemet’s & endothelium

69. • MANAGEMENT :
• usually a cosmetic rather than a visual problem
• Astigmatism - correction of refractive errors with spectacles
• Drying of surrounding cornea by lifting of the lid during blinking - tumor should be
cut flush with the corneal surface
• Central cornea involved - penetrating and lamellar keratoplasty

70. BENIGN SQUAMOUS NEOPLASMS
• Nonkeratinized stratified squamous epithelia of the cornea and
conjunctiva are contiguous; therefore tumors arising within
them are pathologically inseparable, but a transition occurs at
limbus
• Most conjunctival squamous epithelial tumors arise from limbus
& spread to adjacent conjunctival and cornea
• BENIGN HEREDITARY INTRAEPITHELIAL DYSKERATOSIS
• AD, bilateral, first decade of life
• Duplication of a gene on Chr 4
• V-shaped, hyperplastic, translucent, elevations that arise at
limbus. Dilated vessels may be present
• Biopsy - epithelial acanthosis, parakeratosis, and dyskeratosis,
inflammatory cells in stroma.
• Complete excision is the treatment of choice

71. • PSEUDOEPITHELIOMATOUS HYPERPLASIA
• Benign, rapidly growing proliferation of conjunctival or corneal
epithelium
• In response to some preexisting inflammation such as a
pterygium
• A white elevated mass with hyperkeratotic surface.
• A central umbilication may be present
• Lateral margins of the lesion show a smooth transition into
surrounding normal epithelium
• Can mimic ocular surface neoplasia
• Histopathologically – acanthosis which may be pushed
downward into substantia propria, forming lobules of
squamous cells with keratin whorls.
• Precarcinomatous
• Simple excision is usually curative

72. CONJUNCTIVAL INTRAEPITHELIAL
NEOPLASIA • Neoplastic proliferation of dysplastic squamous epithelium
that is noninvasive
• Etiology - multifactorial – UV exposure, heavy smoking, light
skin pigmentation, immunosuppression, HIV, HPV (6, 8,11-
benign, 16,18-malignant), xeroderma pigmentosum
• Mutation or deletions of the tumor suppressor gene p53
• 95% - at the limbus, nasal or temporal interpalpebral zone.
• Slow-growing
• 10% of lesions are leukoplakic
• Most are translucent or gelatinous thickenings of the
conjunctiva
• Fine vascular pattern that has a hairpin configuration
• Spreads to involve the adjacent corneal surface, anterior to
Bowman’s layer.

73. • The abnormal epithelium has a frosted appearance with a fimbriated margin, supported
by pannus
• Primary corneal dysplasia & primary corneal epithelial dysmaturation - to describe cases
in which corneal involvement is disproportionate
• Histologically - partial- to full-thickness conjunctival epithelial dysplasia. BM is not
penetrated
• MANAGEMENT
• Newly diagnosed lesions - excised with 1 to 2 mm of surrounding clinically uninvolved
margins. The sclera is left bare. Corneal debridement, cryotherapy should be applied
• Recurrent lesions - wide local excision but more aggressive cryotherapy
• Deep tissue invasion - Cutaneous cryogun - full-thickness transscleral ice ball
• Topical mitomycin-C - incompletely excised or recurrent lesion
• Interferon-alfa, 5FU

74. INVASIVE SQUAMOUS CELL
CARCINOMA • CIN – precursor
• Occurs when dysplastic epithelial cells penetrate the
underlying basement membrane & extend into
subconjunctival space.
• HPV is thought to play a role in pathogenesis
• Lesions resemble those of CIN but are more elevated
• Frequently at limbus - gelatinous, translucent,
leukoplakic, or papilliform
• Engorged feeder vessels - firmly fixed to underlying
episcleral or scleral tissues
• A frosted corneal epithelium and an extensive
leukoplakic limbal component
• Histopathologically – similar to severe CIN. BM -
evidence of penetration with subepithelial neoplastic
cells.

75. • MANAGEMENT :
• Minimally invasive - wide local excision and aggressive cryotherapy. Often, superficial
lamellar sclerectomy or keratectomy is required
• Preop and postop adjunctive topical mitomycin-C
• Large lesions – excision with the adjunctive use of AMT
• Alcohol or cocaine - complete removal of the affected corneal epithelium
• Intraocular invasion - enucleation, orbital invasion - exenteration

76. CONJUNCTIVAL MELANOMA
• May arise from PAM, from pre-existing nevi, or de novo
• Dark pigmented individuals
• Atypical melanocytes invade from the epithelium into the
substantia propria
• Limbal/ Bulbar/ forniceal/ palpebral
• Thickened, nodularity, dilator feeder vessels
• May extend to cornea, lids, orbits
• Cytologically - spindle-type cells or epithelioid-type cells
• Spindle-shaped melanoma cells - amelanotic, highly invasive, with involvement of
nerves and extension posteriorly into the orbit
• MANAGEMENT :
• Excision with clinically free margins & edges treated with double freeze thaw
cryotherapy
• Scleral adhesion – partial thickness sclerectomy
• Corneal extension – removed by absolute alcohol keratoepitheliectomy
• Intraocular – enucleation
• Orbit - exenteration

77. REFERENCES
• CORNEA – Fundamentals, Diagnosis, Management – Krachmer, Manns, Holland – 3rd
edition
• Copeland & Afshari’s Principles and Practice of Cornea
• OPHTHALMOLOGY – Yanoff and Duker, 5th edition
• Posr Graduate Ophthalmology – Zia Chaudhuri
• Kanski’s Clinical Ophthalmology – 8th edition