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Dr. Riyad Banayot
Fleischer's ring (arrowed) around the cone Vogt's striae
Enlarged corneal nerves Corneal scar from previous hydrops
 Early cases:
 Thinning of the corneal apex with striation of the
stroma and descemet's membrane (Vogt's striaes).
 The...
 Acute hydrops develops from a break in
the descemet's membrane leading to the
entry of aqueous into the stroma causing
s...
 There is fragmentation of Bowman's layer
 Thinning of the stroma and overlying
epithelium.
 The descemet's membrane sh...
 The main problem is high astimgatism
which is irregular and often progressive.
 Glasses can initially correct the
astig...
keratoconus
The topography shows the typical
inferior steepening of the cornea.
Pellucid marginal degeneration
The topogra...
Increased right corneal diameter
There are multiple horizontal lines in the descemet's
membrane (Haab's striae).
The cornea may be edematous if there are i...
 Look for secondary congenital glaucoma such as
aniridia, anterior chamber cleavage syndrome.
 Measure the corneal diame...
 Primary congenital glaucoma
 Anterior cleave syndrome (Axenfeld,
Reiger and Peter's syndrome)
 Rubella
 Phakomatosis ...
 Myopia
 Lens subluxation
 Corneal decompensation
 Retinal detachment
There is a band of sub-epithlial whitish depositions in the inter-palpebral
zone. It is separated from the limbus by a cle...
 Look for any coexisting corneal lesions
such as interstitial keratitis
 If the patient is young (especially female)
loo...
 Ocular diseases:
 Chronic ocular disease; usually inflammatory (juvenile
chronic arthritis)
 Phthisis bulbi
 Intersti...
 Cosmesis
 Visual disturbance
 Pain from the breakdown of epithelium
Band keratopathy treated with
excimer laser
The cornea has diffused stromal haze.
High magnification shows ghost (empty) vessels in the deep and
mid-stroma.
The corne...
Look for
 Signs of congenital syphilis
 Saddle-nose
 Deafness
 Under-developed dentition.
 It occurs in congenital syphilis and
usually develops in the first or second
decades of life.
 It is divided into three...
 Saddle-nose
 Interstitial keratitis
 Hutchinson's teeth
 widely spaced peg-shaped upper incisors
 Crescentric notch ...
Corneal deposits (basal layer of the epithelium) distributed in whorl's-like pattern
Cornea verticillata
Look for signs of:
 Amiodarone intake (for example photosensitivity of
the skin, irregular pulse rate). There may be BRAO...
 It is an X-linked disorder due to a
deficiency of alpha-galactosidase.
 It causes deposition of glycosphingolipid
in th...
Angiokeratoma corporis discusum
There are multiple sub-epithelial ring-shaped opacities (so called
honeycomb appearance) in the center of the cornea
Opaci...
 Autosomal dominant
 Appear in the first few years of life and mainly
affects the Bowman's membrane
 Symptoms begin in ...
There are refractile, branching lines (in the stroma) The periphery of the cornea is
spared. The lines may be double conto...
 Type I:
 Classic type, type AA amyloid
 No systemic amyloid deposition
 Type II:
 Lattice dystrophy with coexistent ...
The cornea is stained with Congo red,
and contains multiple reddish deposits representing amyloid deposits.
There are multiple greyish white opacities involving the stroma and the corneal
periphery (in granular and lattice dystrop...
 It is a autosomal recessive condition
 Characterized by an accumulation of
glycosaminoglycan both intra and
extracellul...
 It is an inborn error of metabolism
characterized by glycosaminoglycan
accumulation.
 The condition is usually autosoma...
 Most patients develop significant visual
impairment by the age of 30
 Corneal graft is usually necessary to
improve vis...
Macular dystrophy is best shown with alcian blue and colloidal iron.
Colloidal iron is used in this case.
There are multiple small, well-demarcated, grayish white opacities(in the anterior
stroma).
The intervening stroma is clea...
 It is an autosomal dominant condition.
 It usually presents in the teens with
photophobia or abnormal corneal
appearanc...
The cornea has been stained with Masson trichrome which shows up the
granular dystrophy as red
 There are multiple
small white crystals
in the center of the
cornea (just below
the Bowman's
membranes)
 The surface of...
 There are multiple
grayish nebulous
opacities
separated by crack
like clear zones
(changes may
resemble crocodile shagre...
 There are multiple
vesicles in the
endothelium. There are
also multiple greyish
opacities which may be
curvilinear or ha...
 There are multiple
opacities in the
epithelium and has
shapes resembling
map lines,
dots/microcysts or
fingerprint lines...
 They can all present with endothelial failure with
corneal edema.
 Fuchs' endothelial dystrophy: autosomal
dominant; us...
There are radial incisions (usually four or eight) in the cornea with a clear central
zone. There may be ferritin lines at...
 Radial incisions (up to a depth of 90%)
causes flattening of the cornea and
reduces the radius of curvature of the
corne...
Typical facial feature in acne rosacea Cornea neovascularization in
rosacea keratitis
The face is red with telangiectasia, papules and pustules found mainly on the
nose, cheeks and chin. The nose may have irr...
 Acne rosacea is a common skin condition of
unknown origin.
 Ocular involvement is common but most tend to
be mild.
 Oc...
 The lid problems include blepharitis and chronic
meibomian inflammation.
 Lid hygiene and hot compresses are needed to ...
 Doxycycline/tetracycline
has the tendency for
binding to growing
structures that require
calcification (chiefly
teeth an...
A penetrating corneal graft
with interrupted sutures
A tectonic graft
performed for peripheral corneal
perforation resulti...
 Penetrating or lamellar graft (look at the edge of
the graft for the thickness)
 The sutures are interrupted / continuo...
 Presence of IOL especially anterior
chamber lens suggest pseudophakic
bullous keratopathy
 Look at the recipient cornea...
 Problems related to surgery and sutures
 Non-immune corneal problems
 Graft rejection
 Wound leak:
 Resuturing, patching or contact lenses
 Flat chamber/iris incarceration in the wound:
 Poor integrity of...
 Primary endothelial failure:
 From deficiency of donor endothelium or the cornea is
sutured with the endothelium side u...
 Usually occurs after the first two weeks and
early recognition is important for good outcome
 Endothelial rejection is ...
 The two main methods of storing are by Refrigeration
 4°C (USA) in Organ culture at 31-37°C (Europe).
longer storage ti...
 The quality of the tissue is assessed, primarily by
examination of the endothelium to estimate endothelial
cell density ...
• There is are peripheral
thinning of the superior
cornea which has a
sloping peripherally and a
sharp central edge
• Usua...
 Although usually asymptomatic,
advanced cases may affect the vision by
causing astigmatism which is usually
against the ...
• There is thinning with
ulceration involving a
sector of the peripheral
cornea.
• Look for:
• Associated systemic signs
s...
 Rheumatoid arthritis
 Wegener's granulomatosis
 SLE
 Inflammatory bowel diseases
 Polyarteritis nodosa
 Acne rosace...
• There is peripheral
ulcerative keratitis which
is contiguous with the
limbus without intervening
clear zone.
• The epith...
 The diagnosis is based on the clinical
history and characteristic appearance in
conjunction with the exclusion of other
...
 There is a prominent
irregular ridge which lies
central to the limbus.
 It is caused by an
anteriorly
displaced Schwalb...
 There is posterior
embryotoxon with iris strands
attached to it. Peripheral
anterior synechiae and
hypoplasia of the
ant...
Dental dysplasia in a patient with Axenfeld-Reiger's syndrome
 There is central corneal
opacity involving the
stroma with iris adhesion
at the edges of the
opacities.
Look for:
 Trab...
 Posterior embryotoxon and Axenfeld-
Reiger's syndrome are usually inherited
in an autosomal dominant fashion.
 Peters' ...
 Posterior embryotoxon alone is not
associated with glaucoma.
 50% of the patients with Axenfeld-
Reiger's syndrome and ...
 S Sclero-cornea
 T Trauma
 U Ulcers (e.g. herpes simplex)
 M Metabolic disorders (e.g. MPS)
 P Peters' anomaly
 E E...
Common Cases: Cornea
Common Cases: Cornea
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Common Cases: Cornea

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Common Cases: Cornea

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Common Cases: Cornea

  1. 1. Dr. Riyad Banayot
  2. 2. Fleischer's ring (arrowed) around the cone Vogt's striae
  3. 3. Enlarged corneal nerves Corneal scar from previous hydrops
  4. 4.  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)
  5. 5.  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.
  6. 6.  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.
  7. 7.  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!
  8. 8. 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.
  9. 9. Increased right corneal diameter
  10. 10. There are multiple horizontal lines in the descemet's membrane (Haab's striae). The cornea may be edematous if there are insufficient endothelium.
  11. 11.  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.
  12. 12.  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
  13. 13.  Myopia  Lens subluxation  Corneal decompensation  Retinal detachment
  14. 14. 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).
  15. 15.  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.
  16. 16.  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
  17. 17.  Cosmesis  Visual disturbance  Pain from the breakdown of epithelium
  18. 18. Band keratopathy treated with excimer laser
  19. 19. 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.
  20. 20. Look for  Signs of congenital syphilis  Saddle-nose  Deafness  Under-developed dentition.
  21. 21.  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.
  22. 22.  Saddle-nose  Interstitial keratitis  Hutchinson's teeth  widely spaced peg-shaped upper incisors  Crescentric notch at the cutting edge
  23. 23. Corneal deposits (basal layer of the epithelium) distributed in whorl's-like pattern Cornea verticillata
  24. 24. 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)
  25. 25.  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.
  26. 26. Angiokeratoma corporis discusum
  27. 27. 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
  28. 28.  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.
  29. 29. 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
  30. 30.  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
  31. 31. The cornea is stained with Congo red, and contains multiple reddish deposits representing amyloid deposits.
  32. 32. 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).
  33. 33.  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.
  34. 34.  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.
  35. 35.  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
  36. 36. Macular dystrophy is best shown with alcian blue and colloidal iron. Colloidal iron is used in this case.
  37. 37. 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
  38. 38.  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.
  39. 39. The cornea has been stained with Masson trichrome which shows up the granular dystrophy as red
  40. 40.  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
  41. 41.  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
  42. 42.  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
  43. 43.  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)
  44. 44.  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.
  45. 45. 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.
  46. 46.  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.
  47. 47. Typical facial feature in acne rosacea Cornea neovascularization in rosacea keratitis
  48. 48. 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.
  49. 49.  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
  50. 50.  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.
  51. 51.  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 ?
  52. 52. A penetrating corneal graft with interrupted sutures A tectonic graft performed for peripheral corneal perforation resulting from rheumatoid arthritis-related corneal melt
  53. 53.  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
  54. 54.  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
  55. 55.  Problems related to surgery and sutures  Non-immune corneal problems  Graft rejection
  56. 56.  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
  57. 57.  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
  58. 58.  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.
  59. 59.  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.
  60. 60.  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????
  61. 61. • 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
  62. 62.  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
  63. 63. • 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
  64. 64.  Rheumatoid arthritis  Wegener's granulomatosis  SLE  Inflammatory bowel diseases  Polyarteritis nodosa  Acne rosacea  Leukemia
  65. 65. • 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
  66. 66.  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.
  67. 67.  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
  68. 68.  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
  69. 69. Dental dysplasia in a patient with Axenfeld-Reiger's syndrome
  70. 70.  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
  71. 71.  Posterior embryotoxon and Axenfeld- Reiger's syndrome are usually inherited in an autosomal dominant fashion.  Peters' anomaly may be sporadic, recessive and sometimes dominant.
  72. 72.  Posterior embryotoxon alone is not associated with glaucoma.  50% of the patients with Axenfeld- Reiger's syndrome and Peters' anomaly will develop glaucoma.
  73. 73.  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

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