KERATOCONUS

KERATOCONUS (CONICAL CORNEA)

• Central or paracentral corneal stromal
thinning occurs accompanied by apical
protrusion and irregular astigmatism

• App.50% of normal fellow eye will progress to

KC within 16 years

• Both eyes are affected eventually

• Aetiology:
congenital weakness of cornea, manifests only
after puberty
secondarily following trauma- unilateral
most patients donot have family history
only 10% offspring develop KC
autosomal dominant transmission with
incomplete penetrance
• Graded by highest axis of corneal power on
keratometry :
mild ( <48D )
moderate ( 48 – 54D )
severe ( >54D )
 Presentation
• Commnly during the teens or twenties, with
features initially in only one eye
• Systemic associations
Downs syndrome
Ehlers – Danlos
Marfan syndrome
Osteogenesis imperfecta
• OCULAR ASSOCIATIONS:
Vernal keratoconjunctivitis
blue sclera
aniridia
leber congenital amaurosis
retinitis pigmentosa
persistant eye rubbing from any cause
 DIAGNOSIS
• SYMPTOMS:
unilateral impairment of vision due to
progressive myopia and astigmatism
occasionally, initial presentation is with
acute hydrops
 Signs
• Progressive corneal protrusion in a cone
configuration with thinning maximal at the
apex
• MUNSON SIGN- bulging of the lower lid in
down gaze
• Direct ophthalmoscopy from a distance of half
a metre shows ‘OIL DROPLET’ reflex. It is due
to a zone through which a few rays pass into
the observer’s eye, as the emergent rays in
the centre are convergent, while those on the
periphery are divergent.
• Retinoscopy - irregular scissoring reflex
• Slit lamp examination – VOGT STRIA ( very
fine, vertical, deep stromal lines) which
disappear with pressure on globe
• FLEISCHER RING ( epithelial iron deposits, best
seen with cobalt blue filter, may surround the
base of cornea)
• In less advanced cases, distorsion of corneal
reflex is the chief guide, a change best seen
with a placido disc or corneal topography.
• Keratometry – readings are steep, mires are
malformed, mal aligned and malfocussed
• Corneal thinness- measured with ultrasonic
pachymetry or the orbscan II corneal
topography system.
 Corneal topography
• Highly sensitive for detection
• Characteristically, astigmatism progresses
from a symmetrical “ bow – tie” pattern,
through an asymmetrical appearance to an
inferotemporally displaced steep- sided cone.
• Sometimes a central cone may develop.
 Later stage- ACUTE HYDROPS
• Caused by a rupture in the sretched descemet
membrane
• Sudden influx of aqueous into cornea
• Pain, photophobia, decreased vision.
• Break usually heals in 6 – 10 weeks → corneal
scarring.
• This sometimes gives improved vision by
flattening the cornea.
 Treatment of acute hydrops
• Cycloplegia
• Hypertonic (5% )saline ointment
• Patching
• Soft BCL
• Accelerated resolution has been reported with
intracameral gas injection in the acute stage.
 TREATMENT
• LASIK- Contraindicated
• Patients should be screened for KC prior to corneal
refractive Sx
• Eye rubbing to be avoided
• Spectacles or soft contact lenses – in early cases. If
thinning marked, consider wearing safety spectacles
over contact lenses
• Rigid contact lenses- sometimes scleral – for higher
degrees of astigmatism – to provide a regular
refracting surface
 CORNEAL COLLAGEN CROSS LINKING
• Epithelium is removed.
• Riboflavin 0.1% e/d instilled every 3 minutes
for 30 minutes.
• Once the cornea is adequately saturated with
riboflavin, it is exposed to a quantitative dose
(3 Mw/cm2) of UVA radiation.
• Followed by insertion of a bandage soft
contact lens to permit the epithelium to heal.
• Riboflavin acts as a photosensitizing agent that
triggers increased cross linking of the corneal
collagen fibrils bythe formation of intrafibrillar
and interfibrillar covalent bonds by
photosensitized oxidation to stabilize the
corneal stroma, delay progression and
improve contact lens tolerance.
INTRACORNEAL RING SEGMENT
IMPLANTATION
 KERATOPLASTY
• Penetrating or DALK.
• DALK is currently becoming the procedure of choice, if the
Descemet's membrane and endothelium remain involved
as it removes the entire corneal stroma sparing the host
Descemet's membrane and endothelium.

• H/O hydrops is a contraindication to DALK due to the

presence of a descemet membrane discontinuity

• Residual astigmatism and anisometropia→ contact lens

correction for optimal acuity.
 POSTERIOR KERATOCONUS
• A sporadic condition in which there is
unilateral non progressive increase in
curvature of the posterior corneal surface.
• Anterior surface – normal
• Visual acuity – relatively unimpaired because
of similar refractive indices of cornea and
aqueous humour
• TYPES:
• Generalised – (involvement of the entire
posterior corneal surface)
• Localised –( paracentral or central posterior
indentation)
 KERATOGLOBUS
• Congenital anomaly in which there is a
hemispherical protrusion of the whole cornea,
occuring bilaterally.
• It is familial and hereditary
• It differs from buphthalmos in that the IOP is
normal, cornea- clear, refractive errors are '
with the rule', the angle of AC normal and
there is no cupping of the optic disc.
THANK YOU