Keratoconus

Progressive,
Non inflammatory,
Bilateral (usually asymmetrical)
Cone like anterior protrusion of the
cornea involving the central and the
inferior paracentral areas
Incidence of 1 in 2000 of general
population.
DOS Times - Vol. 15, No. 10, April 2010

Usually seen after puberty

No gender predominance
No race predominance
The patient becomes myopic but the error
of refraction cannot be satisfactorily
corrected with ordinary glasses owing to
parabolic nature of the curvature which
leads to irregular astigmatism.

 Abortive/forme fruste /sub clinical

keratoconus is seen in family members or
the fellow eye.
No frank clinical sign
The cornea is at risk of developing
keratoconus at a later stage and can be
diagnosed only by videokeratography.
DOS Times - Vol.10, No. 7 January 2005

Etiology
Various theories: Thinning may be due to
Defective formation/destruction of extracellular
matrix
Abnormal collagenase activity.
Increased levels of proteases &catabolic
enzymes in the basal epithelial cells
Decreased levels of proteinase inhibitors alpha 1
proteinase inhibitor and alpha 2 macroglobulin

 Excessive eye rubbing or atopic disease induces keratoconus by inducing epithelial

damage-----epithelial stress----increased
keratocyte apoptosis through interluekin 1
causing changes in stomal matrix
Hard contact lens wear
6-15 % positive family history.

 The role of heredity :

not been clearly established. .
In some cases, however, a sex-linked
autosomal dominant mode of inheritance,
particularly because of the predominance
of familial females with keratoconus.

Systemic associations

Atopy
Down syndrome
Turner syndrome
Ehlers danlos syndrome
Marfans syndrome
Osteogenesis imperfecta
Floppy eyelid syndrome
Oculodentodigital syndrome
Rieger's syndrome
Focal dermal hypoplasia
Nail -patella syndrome
Apert's syndrome
craniofacial dysostosis (Crouzon's syndrome)

Atopic dermatitis

Marfan syndrome

Down syndrome Ehlers-Danlos

syndrome

Crouzon syndrome

Osteogenesis imperfecta

Ocular association

Vernal keratoconjunctivitis
RP
Lebers congenital amaurosis
Retinopathy of prematurity
Progressive cone dystrophy
Aniridia
Iridoschisis
Iris atrophy
Fuchs' dystrophy
Posterior polymorphous dystrophy
Granular and lattice dystrophies

Symptoms

Progressive visual blurring and/or distortion

Rapidly changing spectacle prescription
Eye rubbing
Photophobia
Glare
Monocular diplopia
Sudden onset of pain, redness, loss of
vision, and photophobia suggests hydrops

 The onset of keratoconus occurs

predominantly in the late teens.
Symptoms usually appear bilaterally, but
it is common to have an asymmetric
presentation.
During the first 5-7 years of onset, the
condition generally worsens with
intermittent periods of remissions

 Munsons sign is a V-shaped conformation of

the lower lid produced by the ectatic cornea in
downgaze.
Rizzutis sign is a sharply focused beam of light
near the nasal limbus, produced by lateral
illumination ofthe cornea in patients with
advanced keratoconus.

Slit lamp examination

Prominent corneal nerves

Slit lamp examination

Fleischer's Ring
The Fleischer ring is a yellow-brown to
olive-green ring of pigment which may or
may not completely surround the base of
the cone
Formed when hemosiderin (iron) pigment
is deposited deep in the epithelium
Fleischer's ring often becomes thinner
and more discrete with progression

 seen approximately 50% of all cases.

Locating this ring initially may be made
easier by using a cobalt filter and carefully
focusing on the superior half of the
cornea's epithelium.
Once located, the ring should be viewed in
white light to assess its extent.

 Lines of Vogt:
small and brushlike lines, generally vertical but they can
be oblique.
Found in the deep layers of the stroma and form along
the meridian of greatest curvature.
Disappear when gentle pressure is exerted on the globe
through the lid.

 Corneal Thinning:

Significant thinning (up to 1/5th cornea

thickness) in the advanced stages of the disease
and a diagnostic criterion based on comparison
of central and peripheral corneal thickness has
been proposed.
Additionally, as the disease progresses, the
cone is often displaced inferiorly. The steepest
part of the cornea (apex) is generally the
thinnest.

 Corneal Scarring:

Sub-epithelial corneal scarring, not

generally seen early, may occur as
keratoconus progresses because of
ruptures in Bowman's membrane which is
then filled with connective tissue
Deep opacity of the cornea are also
common in keratoconus.

Corneal Hydrops:

Corneal hydrops occurs in advanced cases, when Descemet's

membrane ruptures, aqueous flows into the cornea and reseals
Keratoconus patients who are having an acute episode of corneal
hydrops report a sudden loss of vision and a visible white spot on
the cornea.
Corneal hydrops causes edema and opacification.

 As Descemet's regenerates, edema and

opacification diminish. Occasionally,
hydrops can benefit keratoconus patients
who have extremely steep corneas. If the
cornea scars, a flatter cornea often
results, making it easier to fit with a
contact lens.
An increased incidence of hydrops has
also been reported in keratoconus
patients with Down's syndrome.

Diagnosis
Early keratoconus usually manifests as a small
island of irregular astigmatism in the inferior
paracentral cornea.
As the cornea bulges outward, the amount of
astigmatism increases due to the progressive
distortion of the corneal surface.
These changes can easily be seen as irregular
mires on keratometry readings and on
corneal topography, a test used to map the
topographical surface area of the cornea

 Many objective signs are present in

keratoconus.
Retinoscopy shows a scissoring reflex.
Direct ophthalmoscopy may show a
shadow If the pupil is dilated and a +6.00
D lens is in the ophthalmoscopic system,
the cone may appear as an oil or honey
droplet when the red reflex is observedCharleux oil droplet sign

 The photokeratoscope or topographer

placido disc can provide an overview of
the cornea and can show the relative
steepness of any corneal area.
The even separation of the rings in the
spherical cornea ".

Placido disc

 In astigmatic cornea uneven spacing of

the rings--especially inferiorly--in the
keratoconic cornea should be noted
. The central rings may show a tear-drop
configuration termed "keratokyphosis".

 With the handheldkeratoscopes, such as

the Klein keratoscope,early keratoconus is
characterized by a downward deviation of
the horizontal axis of the Placido disk
reflection

 The keratometer also aids diagnosis.

The initial keratometric sign of
keratoconus is absence of parallelism and
inclination of the mires. These can easily
be missed in mild or early cases.

Rabinovitz criteria for diagnosis

of keratoconus
1. Central corneal power >47.2D
2. Inferior superior dioptric assymetry over
1.2
3. Sim K astimatism >1.5D
4. Skewed radial axes more than 21
degrees

Corneal topography
Provides a color coded map of the corneal
surface.
The power in diopters of the steepest and
flattest meridians and their axes are
calculated and displayed
Steep curvatures are marked orange or
red
Flat curvature in blue or violet
Normal curvatures in green or yellow

Classification scheme of normal videokeratographs

in the absolute scale devised as a baseline to monitor
topographic progression to keratoconus A, round:
B, oval: C, superior steepening; D, inferior steepening; E,
irregular; F, symmetric bow tie; G, symmetric bow tie with
skewed radial axes; H, asymmetric bow tie with inferior
steepening (AB/IS); I, asymmetric bow tie with superior
steepening; J, asymmetric bow tie with skewed radial axes
(AB/SRAX

two figures are a schematic

illustration of how to determine
whether a pattern is AB/IS or AB/
SRAX.
A line is drawn to bisect the upper
and lower lobes of the asymmetric
bow tie (see solid lines), if there is
no significant deviation from the
vertical meridian (i.e., no skewing),
the pattern is designated as AB/IS
(as in A);
if the lines bisecting the two lobes
appear skewed by more than
30degree from the vertical
meridian (i.e., 150 deg from one
another), it is labeled as AB/SRAX
(as in Bottom B).

Management of Keratoconus
Spectacles
Mild keratoconus can be corrected with
spectacles.
Retinoscopy is difficult;
a normal subjective refraction is required.
Monocular keratoconus is usually best
dealt with using spectacle correction.
In this group of patients, motivation for
contact lens wear tends to be poor.