Corneal Dystrophies

CORNEAL DYSTROPHIES
Dr.Mohammad Dmour , MD

Case Presentation
• A 45 year old woman came with complaints of Gradual Progressive
Decreased ofVision in both eyes since 10 years of age.
• Patient was apparently alright before that with good visual acuity.
• No H/of Photophobia, Coloured halos,Watering and Pain .
• No H/ofTrauma,Viral infection.
• No relevant family history.
• No H/ of any systemic illness .
• No known drug allergy.

Introduction
•The corneal dystrophies are a group of inherited
corneal diseases that are typically bilateral,
symmetric, slowly progressive, usually bilateral,
many of which are associated with decreased vision
and discomfort and without relationship to
environmental or systemic factors.

Outlines
•Corneal Dystrophy by History
• Corneal Dystrophy Literature
•IC3D Classification
•Epithelial and Subepithelial Dystrophies
•Bowman Layer Dystrophies
•Stromal Dystrophies
•Descemet Membrane and Endothelial Dystrophies
•Take Home Message

HISTORY
The word dystrophy is derived from the Greek (dys =wrong,
difficult; trophe = nourishment) and was introduced into the
medical literature byWilhelm Erb (1840–1921) in 1884, in
describing a disease of the musculature.
In 1890, Arthur Groenouw (1862–1945) published his classic
paper describing 2 patients with ‘‘Noduli Corneae’’ with 1
patient having granular corneal dystrophy and the other,
macular corneal dystrophy.

CORNEAL DYSTROPHY LITERATURE
Bucklers, whose name was
later attached to Reis–
Bucklers corneal dystrophy
(RBCD), published the first
classification of the corneal
dystrophies when he
described the differences
between granular, lattice,
and macular corneal
dystrophies.
Although the dystrophies can
be classified according to
genetic pattern, severity,
histopathologic features, or
biochemical characteristics,
the most commonly used
classification system has
been anatomically based.
The dystrophies are typically
classified by level of the
cornea that is involved.

IC3D CLASSIFICATION
Category 1: A well-defined corneal dystrophy in which the gene has been mapped and
identified and specific mutations are known.
Category 2: A well-defined corneal dystrophy that has been mapped to 1 or more
specific chromosomal loci, but the gene(s) remains to be identified.
Category 3: A well-defined corneal dystrophy in which the disorder has not yet been
mapped to a chromosomal locus.
Category 4: This category is reserved for a suspected new, or previously documented,
corneal dystrophy, although the evidence for it, being a distinct entity, is not yet
convincing.

IC3D CLASSIFICATION
Corneal Dystrophies
Epithelial and
Subepithelial
Dystrophies
Bowman Layer
Dystrophies
Stromal Dystrophies Descemet Membrane&
Endothelial Dystrophies

Epithelial and Subepithelial Dystrophies
1. Epithelial basement
membrane dystrophy
(EBMD)— majority
degenerative, rarely C1
2. Epithelial recurrent
erosion dystrophy
(ERED) C4
3. Subepithelial
mucinous corneal
dystrophy (SMCD) C4
4. Mutation in keratin
genes: Meesmann
corneal dystrophy
(MECD) C1
5. Lisch epithelial
corneal dystrophy
(LECD) C2
6. Gelatinous drop-like
corneal dystrophy
(GDLD) C1

Bowman Layer Dystrophies
1. Reis–Bucklers corneal
dystrophy (RBCD)—
Granular corneal
dystrophy type 3- C1
2.Thiel–Behnke corneal
dystrophy (TBCD) C1,
potential variant C2
3. Grayson –Wilbrandt
corneal dystrophy
(GWCD) C4

Stromal Dystrophies
1.TGFBI corneal
dystrophies
A. Lattice corneal
dystrophy
a. Lattice corneal dystrophy,
TGFBI type (LCD): (LCD1) C1)
B. Granular corneal
dystrophyC1
a. Granular corneal dystrophy,
type 1 (classic) (GCD1) C1
b. Granular corneal dystrophy,
type 2 (granular-lattice) (GCD2)
C1
C. Granular corneal dystrophy,
type 3 (RBCD) = Reis– Bucklers
C1
b. Lattice corneal
dystrophy, gelsolin type
(LCD2) C1
2. Macular corneal dystrophy
(MCD) C1
3. Schnyder corneal
dystrophy (SCD) C1
4. Congenital stromal corneal
dystrophy (CSCD) C1
5. Fleck corneal dystrophy
(FCD) C1
6. Posterior amorphous
corneal dystrophy (PACD) C3
7. Central cloudy dystrophy of
Francxois (CCDF) C4
8. Pre-Descemet corneal
dystrophy (PDCD) C4

Descemet Membrane &Endothelial Dystrophies
1. Fuchs
endothelial
corneal
dystrophy
(FECD) C1, C2,
or C3
2. Posterior
polymorphous
corneal
dystrophy
(PPCD) C1 or
C2
3. Congenital
hereditary
endothelial
dystrophy 1
(CHED1) C2
4. Congenital
hereditary
endothelial
dystrophy 2
(CHED2) C1
5. X-linked
endothelial
corneal
dystrophy
(XECD) C2

Inheritance Pattern : Recessive and X linked

Epithelial Basement Membrane Dystrophy
Epithelial basement membrane is the most common corneal dystrophy.
• Map-dot-fingerprint dystrophy
• Cogan microcystic epithelial dystrophy
• Anterior basement membrane dystrophy
Alternative Names,
Eponyms :
Inheritance : The condition is usually sporadic, and these cases may be
degenerations or secondary to trauma rather than true dystrophies, in
contrast to the rare familial (autosomal dominant ) cases.

Histology shows thickening of the basement membrane with
deposition of fibrillary protein between the basement membrane
and the Bowman layer. Basal epithelial cell hemidesmosomes are
deficient.
Onset is in the second decade. About 10% of patients develop
recurrent corneal erosions in the third decade and the remainder
are asymptomatic throughout life.
The occurrence of bilateral recurrent erosions with no history of
trauma suggests basement membrane dystrophy.

• Dot-like and microcystic epithelial lesions .
• Subepithelial map-like patterns surrounded by
a faint haze.
• Whorled fingerprint-like lines.
• Bleb-like subepithelial pebbled glass pattern.
Signs: Lesions are
often best
visualized by
retroillumination or
scleral scatter.
Treatment is that of recurrent corneal erosions.

(A) Cogan – Dots and Microcysts; (B) Cogan – Map-like pattern.
(A) (B)

Epithelial basement membrane dystrophy. A, Map-like changes. B, Intraepithelial dot
opacities underlying map-like figures.C, Fingerprint lines viewed in retroillumination.

This high magnification photo shows gray
putty-like dots and scalloped map-like lesions.
Blebs are fine, bubble-like structures that appear clear
with retro-illumination.These blebs can coalesce to
form groups with a linear branching pattern (inset).

Meesmann epithelial dystrophy
Meesmann dystrophy is a rare non-progressive abnormality of corneal epithelial
metabolism, underlying which mutations in the genes encoding corneal epithelial
keratins have been reported.
Inheritance: AD.
Histology shows irregular thickening of the epithelial basement membrane and
intraepithelial cysts.
Onset : Early childhood.
Symptoms: Patients may be asymptomatic, or there may be recurrent erosions and
blurring (usually mild).

Meesmann epithelial dystrophy
• Tiny intraepithelial cysts of uniform size but
variable density are maximal centrally and extend
towards but do not reach the limbus .
• The cornea may be slightly thinned and sensation
reduced.
Signs:
Treatment other than lubrication is not normally required.

Meesmann corneal dystrophy. A, Multiple solitary microcysts that are most prominent in
the interpalpebral region are seen in retroillumination. B, Diffuse gray opacity with broad
oblique illumination, and multiple solitary microcysts in retroillumination.

Meesmann’s dystrophy.
This high-power view
shows the cysts of
Meesmann’s dystrophy.
The cysts vary in size.

Confocal microscopy of Meesmann’s dystrophy.There are numerous microcysts filled with hyper-reflective material at the
level of the basal epithelium in the photo on the right.The photo on the left shows a confocal image of normal epithelium.

Gelatinous Drop-like Corneal Dystrophy

Bowman Layer Dystrophies
1. Reis–Bucklers corneal
dystrophy (RBCD)—
Granular corneal
dystrophy type 3 C1
2.Thiel–Behnke corneal
dystrophy (TBCD) C1,
potential variant C2
3. Grayson –Wilbrandt
corneal dystrophy
(GWCD) C4

Reis–Bücklers Corneal Dystrophy
This may be categorized as
an anterior variant of
granular stromal dystrophy
(GCD type 3 ) and is also
known as corneal basement
dystrophy type I (CBD1).
Inheritance is AD; the
affected gene is TGFB1.
Histology: Replacement
of the Bowman layer by
connective tissue bands.
Symptoms: Severe
recurrent corneal
erosions in childhood.
Visual impairment may
occur.

Reis–Bücklers corneal dystrophy
• Grey–white geographic subepithelial opacities,
most dense centrally, increasing in density with
age to form a reticular pattern.
• Corneal sensation is reduced.
Signs:
Treatment is directed at the recurrent erosions.
Excimer keratectomy achieves satisfactory control
in some patients.

Reis–Bucklers dystrophy – typical moderately discrete geographical opacities
(Courtesy ofW Lisch)

Reis–Bucklers corneal dystrophy. A, Coarse geographic opacity of the
superficial cornea. B, Broad oblique illumination demonstrating dense,
reticular, superficial opacity. C, Slit lamp view demonstrating
irregularities in Bowman layer.

Reis-Bücklers’ dystrophy.
This autosomal dominant
dystrophy is characterized
by irregular gray-white
opacities beneath the
epithelium. It begins in
childhood and visual loss is
progressive. Recurrent
erosions occur periodically..

Thiel–Behnke corneal dystrophy
Honeycomb-shaped corneal dystrophy and corneal basement dystrophy type II (CBD2);
features are generally less severe than Reis–Bücklers.
Inheritance: AD; gene TGFB1 and at least one other.
Histology: Bowman layer ‘curly fibres’ on electron microscopy.
Symptoms: Recurrent erosions in childhood.
• Subepithelial opacities are less individually defined than the granular
dystrophy-type lesions seen in Reis–Bücklers dystrophy.
• They develop in a network of tiny rings or honeycomb-like morphology,
predominantly involving the central cornea.
Signs:
Treatment is not always necessary.

Thiel–Behnke corneal dystrophy. A, Reticular honeycomb pattern
ofThiel–Behnke. B, Superficial opacification in advanced disease.

Thiel-Behnke honeycomb
dystrophy. In Reis-
Bücklers’ dystrophy, rod-
like bodies are seen in the
region of Bowman’s layer
and inThiel-Behnke
dystrophy “curly” fibers are
seen in the region of
Bowman’s layer.

Stromal Dystrophies
1.TGFBI corneal
dystrophies
A. Lattice corneal dystrophy
a. Lattice corneal dystrophy,
TGFBI type (LCD): (LCD1) C1,
variants (III, IIIA, I/IIIA, and IV)
B. Granular corneal dystrophyC1
a. Granular corneal dystrophy,
type 1 (classic) (GCD1) C1
b. Granular corneal dystrophy,
type 2 (granular-lattice) (GCD2)
C1
C. Granular corneal dystrophy,
type 3 (RBCD) = Reis– Bucklers
C1
b. Lattice corneal dystrophy,
gelsolin type (LCD2) C1
2. Macular corneal dystrophy
(MCD) C1
3. Schnyder corneal
dystrophy (SCD) C1
4. Congenital stromal corneal
dystrophy (CSCD) C1
5. Fleck corneal dystrophy
(FCD) C1
6. Posterior amorphous
corneal dystrophy (PACD) C3
7. Central cloudy dystrophy of
Francxois (CCDF) C4
8. Pre-Descemet corneal
dystrophy (PDCD) C4

Lattice corneal dystrophy,TGFB1 type
This is usually regarded as the classic form of lattice dystrophy.
Clinical variants (e.g. IIIA ) associated with more than 25 heterozygous
mutations in TGFB1 have been described.
Inheritance. AD; gene TGFB1
Histology: Amyloid, staining with Congo red and exhibiting green
birefringence with a polarizing filter.

Advanced lattice dystrophy type I. Congo red stain
reveals extracellular fusiform deposits of congophilic
material (1) and elastoid degeneration (2).
(1)
(2)

Histopathology
of lattice
corneal
dystrophy type
I. Congo red
stain reveals
large fusiform
stromal lesion.

Lattice corneal dystrophy,TGFB1 type
Symptoms: Recurrent erosions occur at the end of the first
decade in the classic form, when typical stromal signs may not
yet be present. Blurring may occur later.
• Refractile anterior stromal dots , coalescing into a relatively fine
filamentous lattice that spreads gradually but spares the periphery .
• A generalized stromal haze may progressively impair vision.
• Corneal sensation is reduced.
Signs:
Treatment by penetrating or deep lamellar keratoplasty is
frequently required. Recurrence is not uncommon.

Lattice corneal dystrophy,TGFBI type (classic lattice).A, Early lattice corneal
dystrophy (LCD1) with dots and lattice lines in retroillumination. B, Magnified
view of lattice lines and dots in LCD1. C, Central opacification in advanced LCD1.

Lattice dystrophy type I
in an adult. There are
Refractile filamentary
lines with nodular
dilations.The deposits
are more common in the
anterior stroma. Usually,
there is a limbal clear
zone. A fine central
anterior stromal haze
may be present.

Lattice dystrophy type 1. (A) Histology shows amyloid staining with Congo red; (B) glassy dots in the anterior
stroma; (C) fine lattice lines; (D) early central stromal haze (Courtesy ofJ Harry – fig. A; C Barry – figsC and D)

Lattice corneal dystrophy, gelsolin type
Also known as LCD2 and Meretoja syndrome, this is a systemic condition
rather than a true corneal dystrophy.
Inheritance: AD; gene GSN.
Histology shows amyloid deposits in the corneal stroma.
Ocular symptoms. Ocular irritation and late impairment of vision;
erosions are rare.
• Sparse stromal lattice lines spread centrally from the periphery.
• Corneal sensation is impaired.
Ocular signs

Lattice corneal dystrophy, gelsolin type
Systemic features: Progressive cranial and peripheral
neuropathy, mask-like facies and autonomic features.
Homozygous disease is rare but severe.
Treatment: Keratoplasty may rarely be required in later
life.

Lattice dystrophy type II
(Meretoja’s syndrome). There are
refractile corneal deposits that
differ in several respects from
those seen in lattice dystrophy
type I.The deposits are fewer,
coarser, and most dense in the
corneal midperiphery and
generally extend to the limbus
with a more radial orientation.The
central cornea is usually spared,
and the cornea is relatively clear
between the lines.

Lattice corneal dystrophy, gelsolin type (Meretoja). A, Diffuse lattice
lines of the stroma. B,Typical facies of the Meretoja syndrome.

Lattice dystrophy
type IIIA. Coarse
lattice lines traverse
the cornea from limbus
to limbus (arrows).This
rare form of lattice
dystrophy is inherited
as an autosomal
dominant disorder, has
an adult onset, and
includes frequent
episodes of recurrent
corneal erosions.

Lattice dystrophy type
IV.Thick lattice lines are
seen coming in from
limbus in the mid and
deep stroma. In this form
of lattice dystrophy there
is a lack of epithelial
involvement and erosions
are not seen.The onset is
late in life. type IV is due
to mutations in (BigH3)
located on chromosome
5.

Granular corneal dystrophy, type 1 (classic)
Inheritance: AD; gene TGFB1. Homozygous disease gives
more severe features.
Histology: Amorphous hyaline deposits staining bright
red with Masson trichrome .
Symptoms: Glare and photophobia, with blurring as
progression occurs. Recurrent erosions are uncommon.

Granular corneal dystrophy, type 1 (classic)
• Discrete white central anterior stromal deposits resembling sugar
granules, bread crumbs or glass splinters separated by clear stroma
• Gradual increase in number and size of the deposits with deeper
and outward spread, sparing the limbus .
• Gradual confluence and diffuse haze leads to visual impairment.
• Corneal sensation is impaired.
Signs:
Treatment: by penetrating or deep lamellar keratoplasty is
usually required by the fifth decade. Superficial recurrences
may require repeated excimer laser keratectomy.

Granular dystrophy type 1. (A) Histology shows red-staining material with
Masson trichrome; (B) sharply demarcated crumb-like opacities.

Continued (C) increase in number and outward spread; (D)
confluence (Courtesy of J Harry – fig. A)

Granular corneal dystrophy, type 1. A, Discrete and confluent, axially distributed
anterior stromal deposits. B, Diffuse granular opacities in an adult. C, Early
subepithelial verticillate opacity in a 6-year old.

Granular dystrophy.
In this autosomal
dominant condition,
there are numerous
“bread crumb”
white deposits in the
corneal stroma.

Granular corneal dystrophy, type 2
Also known as Avellino and combined granular-lattice dystrophy.
Inheritance: AD; gene TGFB1.
Histology shows both hyaline and amyloid.
Symptoms: Recurrent erosions tend to be mild.Visual impairment is a later feature.
Signs: are usually present by the end of the first decade in heterozygotes. Fine superficial opacities progress
to form stellate or annular lesions, sometimes associated with deeper linear opacities.
Treatment is usually not required. Corneal trauma accelerates progression; refractive surgery is
contraindicated.

Histopathology of
granular dystrophy.
There are deposits of
extracellular hyaline
material in the
corneal stroma.These
deposits are primarily
in the anterior stroma
and have a bread
crumb appearance.
The hyaline material
stains red with
Masson’s trichrome
stain.

Granular
dystrophy type
2 (Avellino)
(Courtesy ofW
Lisch)

Avellino dystrophy.
This autosomal
dominant disorder has
features of both
granular and lattice
dystrophy clinically
and on
histopathologic
examination.The
lattice lesions develop
after the granular
deposits.

Macular corneal dystrophy
Inheritance: Autosomal recessive (AR); gene CHST6; the
condition is relatively common in Iceland.
Histology: Aggregations of glycosaminoglycans intra- and
extracellularly; stain with Alcian blue and colloidal iron.
Symptoms: Early (end of first decade) visual
deterioration; recurrent erosions are very common.

Macular corneal dystrophy
• Dense but poorly delineated greyish-white spots
centrally in the anterior stroma and peripherally in
the posterior stroma.
• There is eventual involvement of full-thickness
stroma, extending to the limbus with no clear zone.
• Thinning is a fairly early feature, with late thickening
from oedema due to endothelial dysfunction.
• Sensation is reduced.
Signs:
Treatment. Penetrating keratoplasty. Recurrence is
common.

Histopathology of
macular dystrophy.
Alcian blue staining
of extracellular and
intracellular
mucopolysaccharides
occurs in all layers of
the cornea, including
the epithelium,
endothelium, and
Decrement's
membrane.

Macular corneal dystrophy. A, Early macular corneal dystrophy with few central
opacities. B, Slit-lamp photograph of advanced macular dystrophy with stromal
opacities at multiple levels and diffuse stromal haze. C, More advanced macular
dystrophy at higher magnification revealing more numerous and diffuse corneal
opacities and stromal haze.

Macular dystrophy.
This autosomal
recessive disorder is
characterized by a
diffuse stromal haze
extending limbus to
limbus and
throughout the
corneal stroma.
Multiple, irregular,
gray-white nodular
lesions are found
within the diffuse
haze.

Schnyder (crystalline) corneal dystrophy
This is a disorder of corneal lipid metabolism, associated in some
patients with systemic dyslipidaemia.
Inheritance: AD; gene UBIAD1.
Histology: Phospholipid and cholesterol deposits.
Symptoms: Visual impairment and glare.

Schnyder (crystalline) corneal dystrophy
Signs
• Central haze is an early feature,
progressing to more widespread
full-thickness involvement over
time.
• Subepithelial crystalline
opacities are present in only
around 50%.
• Prominent corneal arcus is
typical, and gradually progresses
centrally leading to diffuse haze.
Treatment is by
excimer
keratectomy or
corneal
transplantation.

(SCD). A, Central stromal opacity in early SCD without crystals. B, Central subepithelial crystals in early
SCD with crystals.C, Central ring-like opacity, prominent peripheral arcus lipoides, and moderate mid-
peripheral haze in a middle-aged individual with non crystalline Schnyder. D, Central dense opacity,
peripheral arcus lipoides, and prominent mid-peripheral haze. E, Advanced SCD with dense corneal
opacification, subepithelial crystals, and peripheral arcus lipoides.

Schnyder’s crystalline
dystrophy. In this
autosomal dominant
disorder, there is central
anterior stromal corneal
opacity.The peripheral
edge is irregular and
crystalline (1).The
crystals are composed of
cholesterol, and patients
may have systemic
hyperlipidemia. Arcus (2)
is often present.
(1).
(2)

Schnyder’s
crystalline
dystrophy.
Multiple central
crystalline
deposits and
peripheral arcus
are seen.

Descemet Membrane &Endothelial Dystrophies
1. Fuchs
endothelial
corneal
dystrophy
(FECD)
2. Posterior
polymorphous
corneal
dystrophy
(PPCD)
3. Congenital
hereditary
endothelial
dystrophy 1
(CHED1)
4. Congenital
hereditary
endothelial
dystrophy 2
(CHED2)
5. X-linked
endothelial
corneal
dystrophy
(XECD)

Fuchs endothelial corneal dystrophy
This disorder is characterized by bilateral accelerated endothelial cell loss. It
is more common in women and is associated with a slightly increased
prevalence of open-angle glaucoma.
Inheritance: Most are sporadic, with occasional AD inheritance. Mutation in
COL8A2 has been identified in an early-onset variant.
Symptoms: Gradually worsening blurring, particularly in the morning, due
to corneal oedema. Onset is usually in middle age or later.

Cornea guttata: the presence of
irregular warts or ‘excrescences’
on Descemet membrane secreted
by abnormal endothelial cells .
Specular reflection shows tiny
dark spots caused by disruption of
the regular endothelial mosaic.
progression occurs to a ‘beaten
metal’ appearance.
Endothelial decompensation
gradually leads to central stromal
oedema and blurred vision, worse
in the morning.
Signs

Fuchs endothelial corneal dystrophy. A, Central guttae viewed in
retroillumination and in the slit beam. B, Cornea guttae as seen in specular
reflection. C, Advanced stromal edema. D, Advanced endothelial
decompensation with epithelial microcystic and bullous edema.

Fuchs’ dystrophy.
Confluent guttata
and thickening of
Descemet’s
membrane
produce a beaten
metal appearance .

• Conservative options include topical sodium chloride 5% drops or
ointment, reduction of intraocular pressure and use of a hair dryer for
corneal dehydration.
• Ruptured bullae can be made more comfortable by the use of bandage
contact lenses, cycloplegia, antibiotic ointment and lubricants.Anterior
stromal puncture may be helpful.
• Posterior lamellar (e.g. Descemet membrane-stripping endothelial
keratoplasty – DSAEK – or Descemet membrane endothelial keratoplasty
– DMEK) and penetrating keratoplasty have a high success rate.
Treatment

Options in eyes with poor visual potential include conjunctival flaps and amniotic membrane
transplantation.
A promising new treatment, topical Rho-kinase inhibitor with prior transcorneal endothelial
cryotherapy, seems to stimulate endothelial cell proliferation and improve function.
Cataract surgery may worsen the corneal status via significant endothelial cell loss, and
protective steps should be taken.
A ‘triple procedure’ (combined cataract surgery, lens implantation and keratoplasty) may be
considered in eyes with corneal oedema.

Posterior polymorphous corneal dystrophy
There are three forms of posterior polymorphous dystrophy, PPCD1–3.Associations include iris
abnormalities, glaucoma and Alport syndrome.
The pathological basis involves metaplasia of endothelial cells.
Inheritance is usuallyAD.
Symptoms: Typically absent, with incidental diagnosis.
Signs: Subtle vesicular, band-like or diffuse endothelial lesions.
Treatment is not required.

Posterior polymorphous
dystrophy. This autosomal
dominant disorder of the
corneal endothelium is
almost always bilateral,
although it can be
extremely asymmetric or
unilateral.This is an
example of posterior
corneal vesicles , the most
common finding in this
disorder.

Congenital hereditary endothelial dystrophy
CHED is a rare dystrophy in which there is focal or diffuse thickening of Descemet
membrane and endothelial degeneration.CHED2 is a more common, and more severe,
form than CHED1, and is occasionally associated with deafness (Harboyan syndrome).
• CHED1 is AD with the gene locus on chromosome 20.
• CHED1 may not be distinct from PPCD.
• CHED2 is AR; gene SLC4A11.
Inheritance
Symptoms: Photophobia and watering are common in CHED1, but not in CHED2.

Histopathology of
congenital hereditary
endothelial dystrophy. There
is an absence of endothelial
cells, and Descemet’s
membrane is thickened.There
is corneal edema, with loss of
the artifactual stromal clefting
and random orientation of
collagen lamellae. Epithelial
bullae are present.

Congenital hereditary endothelial dystrophy.A, CHED1— Milky appearance of cornea with
diffuse illumination. B, CHED2—Slit beam photograph demonstrating diffuse stromal
thickening in a homozygote individual with SLC4A11 mutations.

TAKE HOME MESSAGE
Corneal
dystrophy is a
wide spectrum
of diseases with
overlapping
clinical
presentations.
Morphologic
features may not
be able to
differentiate one
from another.
Patients may be
symptom free or
may have recurrent
corneal erosions or
diminution of
vision.
Treatment will be
tailored according
to the patients
requirement.

http://www.corneasociety.org/publications/ic3d

Corneal Dystrophies

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