Common Cases: Cornea

1. Dr. Riyad Banayot

3. Fleischer's ring (arrowed) around the cone Vogt's striae

4. Enlarged corneal nerves Corneal scar from previous hydrops

5.  Early cases:
 Thinning of the corneal apex with striation of the
stroma and descemet's membrane (Vogt's striaes).
 There is Fleischer's ring surronding the cone (blue light
on the slit-lamp can improve its visualization).
 Corneal nerves are prominent.
 Advanced cases:
 Corneal scars from previous hydrops.
 Other examination:
 Examine the fellow eye which may show a different
stage of progression or corneal graft
 Munson's sign (distortion of the lower lid on down-
gaze)
 Rizzuti's sign (conical reflection on the nasal cornea
when a penlight is shone from the temporal side).
 Associated features:
 Down's syndrome, atopic eczema, connective tissue
disorders (Marfan's syndrome)

6.  Acute hydrops develops from a break in
the descemet's membrane leading to the
entry of aqueous into the stroma causing
stromal edema. The condition is painful
and the cornea appears opaque.
 It is not an indication for urgent corneal
graft because the edema tends to resolve
over weeks and months when the
endothelium cells enlarge and grow over
the descemet's break.
 Cycloplegic and topical steroid are often
used because of the pain and
photophobia. Sodium chloride 5% can be
used to reduce the edema.

7.  There is fragmentation of Bowman's layer
 Thinning of the stroma and overlying
epithelium.
 The descemet's membrane shows folds
and breaks.
 Diffuse scarring is often present in the
stroma.

8.  The main problem is high astimgatism
which is irregular and often progressive.
 Glasses can initially correct the
astigmatism and when these become
ineffective hard contact lens is used.
 When the vision can not be corrected
with the contact lens or when the cornea
becomes too steep for the contact lens,
corneal graft is indicated.
 New Technologies?
 Cross linking!
 Kerra rings!

9. keratoconus
The topography shows the typical
inferior steepening of the cornea.
Pellucid marginal degeneration
The topography shows inferior
steepening of the cornea, but also
shows the typical flattening of the
central cornea.

10. Increased right corneal diameter

11. There are multiple horizontal lines in the descemet's
membrane (Haab's striae).
The cornea may be edematous if there are insufficient
endothelium.

12.  Look for secondary congenital glaucoma such as
aniridia, anterior chamber cleavage syndrome.
 Measure the corneal diameter (the normal corneal
diameter is between 10 and 12 mm) with the slit-lamp.
 Check the corneal edge on the temporal side for
any goniotomy scars.
 Also look for trabeculectomy scar at the superior
limbus
 Examine the optic disc for glaucomatous
changes
 Check the patient's glasses, the affected side
invariably has a myopic prescription.

13.  Primary congenital glaucoma
 Anterior cleave syndrome (Axenfeld,
Reiger and Peter's syndrome)
 Rubella
 Phakomatosis (Sturge-Weber's syndrome
and neurofibromatosis)
 Aniridia
 Persistent hyperplastic primary vitreous

14.  Myopia
 Lens subluxation
 Corneal decompensation
 Retinal detachment

15. There is a band of sub-epithlial whitish depositions in the inter-palpebral
zone. It is separated from the limbus by a clear zone. There are multiple
round clear holes (due to nerve channels).

16.  Look for any coexisting corneal lesions
such as interstitial keratitis
 If the patient is young (especially female)
look for pseudophakia and aqueous haze
and signs of arthritis
 A unilateral band keratopathy may be
caused by a phthisical eye
 If the center of the band is clear with
well-defined edges, the calcium may
have been removed with EDTA
or excimer laser.

17.  Ocular diseases:
 Chronic ocular disease; usually inflammatory (juvenile
chronic arthritis)
 Phthisis bulbi
 Interstitial keratitis
 Silicon oil keratopathy
 Systemic hypercalcaemia:
 Hyperparathryodism
 Vitamin D toxicity
 Sarcoidosis
 Chronic renal failure
 Idiopathic

18.  Cosmesis
 Visual disturbance
 Pain from the breakdown of epithelium

19. Band keratopathy treated with
excimer laser

20. The cornea has diffused stromal haze.
High magnification shows ghost (empty) vessels in the deep and
mid-stroma.
The cornea may be thin. The condition is usually bilateral.

21. Look for
 Signs of congenital syphilis
 Saddle-nose
 Deafness
 Under-developed dentition.

22.  It occurs in congenital syphilis and
usually develops in the first or second
decades of life.
 It is divided into three stages:
 Early stage: characterized by ocular pain,
photophobia. There is deep stromal and
endothelial edema. KPs may occur.
 Florid stage: when the inflammation is at its
peak with iridocyclitis. There is deep stromal
vessels which gives a pink color to the cornea
(salmon pink)
 Regression: vascular regression with clearing
of the stromal infiltrates leaving behind
corneal scarring. This may take up to 1 or 2
years to resolve unless topical steroid is used.

23.  Saddle-nose
 Interstitial keratitis
 Hutchinson's teeth
 widely spaced peg-shaped upper incisors
 Crescentric notch at the cutting edge

24. Corneal deposits (basal layer of the epithelium) distributed in whorl's-like pattern
Cornea verticillata

25. Look for signs of:
 Amiodarone intake (for example photosensitivity of
the skin, irregular pulse rate). There may be BRAO or
CRAO from emboli)
 Indomethacin intake (look for signs of rheumatoid
arthritis)
 Fabry's disease (renal failure with arterio-venous
fistula, angiokeratomas, dilated and tortuous
conjunctival vessels with or without aneurysms)

26.  It is an X-linked disorder due to a
deficiency of alpha-galactosidase.
 It causes deposition of glycosphingolipid
in the body tissue.
 Renal involvement causes renal failure.
 Skin lesion termed angiokeratoma
corporis discusum consists of clusters of
superficial cutaneous dark-red
angiokeratomas.
 Apart from vortex keratopathy, there
may be tortuosity and aneurysmal
dilatations of the conjunctival and retinal
vessels.

27. Angiokeratoma corporis discusum

28. There are multiple sub-epithelial ring-shaped opacities (so called
honeycomb appearance) in the center of the cornea
Opacities may extend towards the periphery in severe cases. The corneal
surface is irregular with ferritin deposition.
Look at the opposite eye for: Similar changes, corneal graft

29.  Autosomal dominant
 Appear in the first few years of life and mainly
affects the Bowman's membrane
 Symptoms begin in the first or second decade
with painful, recurrent epithelial erosion.
 Vision is affected by both anterior scarring with
surface irregularity and anterior stromal oedema
(from recurrent corneal erosion)
 Histologically, there is disruption and absence of
Bowman's layer, with replacement by fibrocellular tissue
that corresponds clinically to areas of subepithelial
opacification.
 Keratoplasty is the treatment of choice (either
penetrating or lamellar) and recurrence in the
graft is common.

30. There are refractile, branching lines (in the stroma) The periphery of the cornea is
spared. The lines may be double contoured in advanced cases.
Has the highest tendency for recurrence in the graft.
Look for systemic amyloidosis (rare) such as macroglossia and periorbital papules

32.  Type I:
 Classic type, type AA amyloid
 No systemic amyloid deposition
 Type II:
 Lattice dystrophy with coexistent systemic amyloidosis
 lines are less numerous and located more peripherally
 Known as Meretoja's syndrome. The patient may have
cranial and peripheral neuropathy.
 Type III:
 Mid-stromal and larger deposit than those in type I
 Deposits are of type AP amyloid

33. The cornea is stained with Congo red,
and contains multiple reddish deposits representing amyloid deposits.

34. There are multiple greyish white opacities involving the stroma and the corneal
periphery (in granular and lattice dystrophy, the periphery is typically spared). The stroma
between the opacities is diffusely cloudy
Right picture: recipient cornea showing changes of macular dystrophy
(because the dystrophy extends to the periphery).

35.  It is a autosomal recessive condition
 Characterized by an accumulation of
glycosaminoglycan both intra and
extracellularly.
 It is thought to be caused by defective
sulfo-transferase.
 It is thought to be a type of localized
mucopolysaccharidosis.

36.  It is an inborn error of metabolism
characterized by glycosaminoglycan
accumulation.
 The condition is usually autosomal
recessive except for Hunter's Syndrome
which is X-linked.
 Most of these patients have ocular
involvement which include corneal
cloudiness, retinal pigmentary
degeneration and optic atrophy.

37.  Most patients develop significant visual
impairment by the age of 30
 Corneal graft is usually necessary to
improve vision
 Recurrence has been reported in grafted
cornea and re-graft is sometimes
necessary

38. Macular dystrophy is best shown with alcian blue and colloidal iron.
Colloidal iron is used in this case.

39. There are multiple small, well-demarcated, grayish white opacities(in the anterior
stroma).
The intervening stroma is clear and the peripheral cornea is not involved.
Classically described ‘bread crumbs’, some have crystalline like appearance
The opposite eye either has similar changes or has corneal graft

40.  It is an autosomal dominant condition.
 It usually presents in the teens with
photophobia or abnormal corneal
appearance.
 Recurrent erosion is uncommon.
 Vision is often normal or only become
impaired in the 40s.
 Full thickness graft is sometimes needed
but recurrence is unusual.

41. The cornea has been stained with Masson trichrome which shows up the
granular dystrophy as red

42.  There are multiple
small white crystals
in the center of the
cornea (just below
the Bowman's
membranes)
 The surface of the
cornea is uninvolved
and there may be
corneal arcus
 Autosomal dominant
inheritance
Schnyder central crystalline
dystrophy

43.  There are multiple
grayish nebulous
opacities
separated by crack
like clear zones
(changes may
resemble crocodile shagreen)
 The opacities are
denser in the
center and the
posterior aspect of
the cornea.
 Autosomal
dominant
inheritance
Central cloudy dystrophy of
Francõis

44.  There are multiple
vesicles in the
endothelium. There are
also multiple greyish
opacities which may be
curvilinear or has
scalloped edges.
 In some patients there may
be stromal oedema or
abnormal iris shape such as
corectopia
or irido-corneal adhesion
(the changes may resemble
ICE syndrome but the
condition is bilateral)
 Autosomal dominant
inheritance
Posterior polymorphous
endothelial dystrophy

45.  There are multiple
opacities in the
epithelium and has
shapes resembling
map lines,
dots/microcysts or
fingerprint lines
 Common condition
 Dominant
inheritance with
incomplete
penetrance
Map-dot-fingerprint
dystrophy (Cogan
microcystic dystrophy)

46.  They can all present with endothelial failure with
corneal edema.
 Fuchs' endothelial dystrophy: autosomal
dominant; usually present in the 5th-6th decade
with corneal edema
 Posterior polymorphous dystrophy: autosomal
dominant; most patients are asymptomatic but
in some there may be corneal edema. The
trabecular meshwork and the iris may be
involved by the abnormal endothelium giving
rise to glaucoma and abnormal iris shapes
 Congenital hereditary endothelial dystrophy:
may be autosomal dominant or recessive. The
recessive form is associated with severe corneal
edema at birth.

47. There are radial incisions (usually four or eight) in the cornea with a clear central
zone. There may be ferritin lines at or around the incisions.

48.  Radial incisions (up to a depth of 90%)
causes flattening of the cornea and
reduces the radius of curvature of the
cornea. This procedure weakens the cornea and
trauma to the eye can easily cause.
 The amount of correction (i.e. the
refractive effect) is determined by the
depth, length and number of cuts.

49. Typical facial feature in acne rosacea Cornea neovascularization in
rosacea keratitis

50. The face is red with telangiectasia, papules and pustules found mainly on the
nose, cheeks and chin. The nose may have irregular thickening of the skin with
large follicular orifices (rhinophyma).
Look for: Blepharitis, meibomian gland dysfunction, keratitis.

51.  Acne rosacea is a common skin condition of
unknown origin.
 Ocular involvement is common but most tend to
be mild.
 Ocular problems is more severe in males
 Eyelids show blepharo-conjunctivitis sometimes
with thick meibomian secretion (chalazion may
be present)
 There may be scars on the tarsal conjunctiva
suggesting previous recurrent chalazion.
 The cornea show pannus with or without
peripheral corneal thinning.
 There are subepithelial opacities especially
inferiorly

52.  The lid problems include blepharitis and chronic
meibomian inflammation.
 Lid hygiene and hot compresses are needed to keep the
lids comfortable.
 In severe cases, oral doxycycline is useful for a period
of 6 weeks but some patients may need the treatment
for much longer.
 The cornea problems are related to dry eyes,
neovascularization and peripheral ulceration.
 Dry eyes can be controlled with lid hygiene and artificial
tears.
 Neovascularization results from chronic corneal
inflammation, and low dose steroid is useful but should
be used with care due to the risk of perforation.
 Peripheral ulceration may result from Staphylococcal
hypersensitivity and the treatment involve lid hygiene
and low dose combination of steroid and antibiotic.

53.  Doxycycline/tetracycline
has the tendency for
binding to growing
structures that require
calcification (chiefly
teeth and bones). These
can result in unsightly
staining of the teeth,
dental hypoplasia and
bone mal-development.
 Doxycycline/tetracycline
should be avoided in
pregnant women and
young children. It is also
contraindicated in breast
feeding women as the
drug is secreted in
breast milk.
A 3-month pregnant
female develops
severe blepharitis and
keratitis secondary to
acne rosacea.
How would you treat
her ?

54. A penetrating corneal graft
with interrupted sutures
A tectonic graft
performed for peripheral corneal
perforation resulting from rheumatoid
arthritis-related corneal melt

55.  Penetrating or lamellar graft (look at the edge of
the graft for the thickness)
 The sutures are interrupted / continuous or a
combination of both
 Is the graft eccentric (it is likely to be tectonic)
 Signs of rejection which may be:
 Epithelial, stromal or endothelial.
 Rejection lines on the endothelium are called
Khodadoust's line.
 keratic precipitates, cells or flare in the anterior
chamber
 Security of the suture and any pannus around
the sutures

56.  Presence of IOL especially anterior
chamber lens suggest pseudophakic
bullous keratopathy
 Look at the recipient cornea for signs for
corneal dystrophy (this is especially obvious with
macular dystrophy which extend to the peripheral cornea)
 Examine the other eye for signs of
dystrophies or keratoconus.
 In tectonic graft observe the patient's
hands for rheumatoid arthritis which may
be the cause of peripheral corneal melt

57.  Problems related to surgery and sutures
 Non-immune corneal problems
 Graft rejection

58.  Wound leak:
 Resuturing, patching or contact lenses
 Flat chamber/iris incarceration in the wound:
 Poor integrity of the wound or high IOP
 Early surgical intervention is important
 Endophthalmitis:
 Paracentesis and vitreous tap for culture and sensitivity
 followed by intravitreous antibiotic with amikacin and
vancomycin
 Suture-related problems:
 Tightening, loosening, infectious abscesses and
vascularization along suture tracks etc.
 The sutures are removed and appropriate treatment
given

59.  Primary endothelial failure:
 From deficiency of donor endothelium or the cornea is
sutured with the endothelium side up by mistake.
 Re-graft is the only option
 Persistent epithelial defect:
 This usually heal by 14 days.
 Treat any dry eyes, exposure keratitis or trichiasis
 Recurrence of primary disease:
 Treat conditions such as herpes keratitis as appropriate
 Infectious keratitis:
 Intensive topical antibiotics are needed
 Late non-immune endothelial failure:
 Re-graft is needed
 Post-operative corneal astigmatism:
 Removal of the offending sutures and consider CL
 Refractive surgery may be needed

60.  Usually occurs after the first two weeks and
early recognition is important for good outcome
 Endothelial rejection is the most serious.
 Keratic precipitates and Khodadoust's lines may be
seen.
 Patient should be admitted for intensive topical
steroids.
 Subepithelial infiltrates
 May resemble adenoviral infection, may be directed at
the donor epithelium or keratocytes and may precede
more serious rejection
 Topical steroid is the treatment of choice.
 Donor epithelium may be rejected showing as a
rejection line.
 As the host cells replace lost donor epithelium, this
rejection is less serious than endothelium rejection but
it may precede endothelium rejection.

61.  The two main methods of storing are by Refrigeration
 4°C (USA) in Organ culture at 31-37°C (Europe).
longer storage time
Greater opportunity to detect infection
Reduced risk of Post-op endophthalmitis
(20-fold less)
 Eyes are first washed in several changes of sterile saline,
immersed in a dilute iodine solution, which is neutralized
with sodium thiosulphate, and then given a final wash in
saline.
 The cornea with a 3-4mm rim of sclera is then carefully
excised taking care to avoid damage to the endothelium.
 A suture is placed through the scleral rim and the cornea
is suspended in about 80-ml of tissue culture medium.
 The corneas are then stored in an incubator.
 After a week, a sample of medium is withdrawn to check
for bacterial and fungal infection. Up to 4% of corneas are
discarded for this reason.

62.  The quality of the tissue is assessed, primarily by
examination of the endothelium to estimate endothelial
cell density and to detect endothelial damage or other
abnormalities.
 A minimum cell density of 2200 cells/mm2 with no central
stromal opacity is considered acceptable for penetrating
grafts.
 This quality assessment means that no age limits to
corneal donation need be set.
 Because corneas become edematous and thickened during
organ culture, they are transferred to tissue culture
medium containing 5% dextran after assessment of the
endothelium. The osmotic effect of the dextran thins the
cornea and returns it towards normal thickness prior to
surgery. The corneas can remain in this medium for four
days by which time they would have been transported to
a recipient hospital and grafted.
 Corneas are tested for????

63. • There is are peripheral
thinning of the superior
cornea which has a
sloping peripherally and a
sharp central edge
• Usually bilateral but
asymmetrical
• The epithelium is intact
and contains superficial
vascularization.
• There may be pseudo-
pterygium in advanced
cases.
Terrien's marginal
degeneration

64.  Although usually asymptomatic,
advanced cases may affect the vision by
causing astigmatism which is usually
against the rule.
 There is also the risk of corneal
perforation with minor trauma

65. • There is thinning with
ulceration involving a
sector of the peripheral
cornea.
• Look for:
• Associated systemic signs
such as symmetrical
arthropathy in rheumatoid
arthritis or saddle nose in
Wegener's granulomatosis
• Associated eye signs such
as keratoconjunctiva sicca
or scleromalacia perforan
both of which may occur in
rheumatoid arthritis
Peripheral corneal ulceration
due to systemic diseases

66.  Rheumatoid arthritis
 Wegener's granulomatosis
 SLE
 Inflammatory bowel diseases
 Polyarteritis nodosa
 Acne rosacea
 Leukemia

67. • There is peripheral
ulcerative keratitis which
is contiguous with the
limbus without intervening
clear zone.
• The epithelium is
vascularized and there is
an overhanging
advancing edge.
• The whole corneal
circumference may be
involved.
• The sclera is not involved.
Mooren's ulcer

68.  The diagnosis is based on the clinical
history and characteristic appearance in
conjunction with the exclusion of other
causes of peripheral ulcerative keratitis.
 There are no histological or blood tests
for its diagnosis.

69.  There is a prominent
irregular ridge which lies
central to the limbus.
 It is caused by an
anteriorly
displaced Schwalbe's line.
(junction of the TM with
the termination of
Descemet's membrane
 Note: this finding is seen in
about 8 to 30% of normal
individual.
Look for other pathology !
Posterior embryotoxon

70.  There is posterior
embryotoxon with iris strands
attached to it. Peripheral
anterior synechiae and
hypoplasia of the
anterior stroma may be seen.
 Axenfeld's anomaly have
posterior embryotoxon plus
anterior synechiae
 Reiger's anomaly have
posterior embryotoxon,
anterior synechiae, iris atrophy
and pupil distortion
 Reiger's syndrome is Reiger's
anomaly plus dental,
craniofacial and skeletal
abnormalities.
Look for:
 Trabeculectomy
 Corectopia, ectopia uvea and
polycoria
 Abnormal denture and maxillary
hypoplasia
Axenfeld-Reiger's syndrome

71. Dental dysplasia in a patient with Axenfeld-Reiger's syndrome

72.  There is central corneal
opacity involving the
stroma with iris adhesion
at the edges of the
opacities.
Look for:
 Trabeculectomy
 Small eye
(microphthalmos)
 Cleft lip/palate,
craniofacial anomalies
 Skeletal abnormalities
 Evidence of congenital
cardiac defects
 (if any of these were
present, the condition is
called Peters plus syndrome)
Peters' anomaly

73.  Posterior embryotoxon and Axenfeld-
Reiger's syndrome are usually inherited
in an autosomal dominant fashion.
 Peters' anomaly may be sporadic,
recessive and sometimes dominant.

74.  Posterior embryotoxon alone is not
associated with glaucoma.
 50% of the patients with Axenfeld-
Reiger's syndrome and Peters' anomaly
will develop glaucoma.

75.  S Sclero-cornea
 T Trauma
 U Ulcers (e.g. herpes simplex)
 M Metabolic disorders (e.g. MPS)
 P Peters' anomaly
 E Endothelial dystrophy (e.g. CHED)
 D Dermoid