Keratoconus

1) Name a few corneal ectatic conditions?

Keratoconus, keratoglobus, pellucid marginal degeneration, Terrien’s marginal
degeration.
2) What are the signs of keratoconus?

External signs
Munson’s sign
Rizzuti phenomenon
Slit-lamp findings
Stromal thinning
Posterior stress lines (Vogt’s striae)
Iron ring (Fleischer ring)
Scarring—epithelial or subepithelial
Retroillumination signs
Scissoring on retinoscopy
Oil droplet sign (“Charleaux”)
Photokeratoscopy signs
Compression of mires inferotemporally
(“egg-shaped” mires)
Compression of mires inferiorly or centrally
Videokeratography signs
Localized increased surface power
Inferior superior dioptric asymmetry
Relative skewing of the steepest radial axes above and
below the horizontal meridian

3) Which sex is commonly affected by keratoconus?

Keratoconus has been seen a bit more commonly in males than in females (various
authors including H.M.Leibowitz)

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4) What are the biochemical alternations seen in corneas with keratoconus?

Various abnormalities suggested include,

1. Decreased levels of G6PD.

2. Relative decrease in hydroxylation of lysine and glycosylation of

hydroxylysine.

3. Decrease in total collagen and a relative increase in structural

glycoprotein.

4. In patients with keratoconus, keratin sulphate is decreased and its structure

is modified.

5. The ratio of dermatin sulphate to keratin sulphate is increased in

keratoconus.

5) What is Munson’s sign?

Munson’s sign is a V-shaped conformation of the lower lid produced by the ectatic
cornea in down gaze.
6) What is Rizzutti’s sign?

Rizzuti’s sign is a sharply focused beam of light near the nasal limbus, produced by
lateral illumination of the cornea in patients with advanced keratoconus.

7) What are Vogt’s striae?

Vogt’s striare are fine vertical lines in the deep stroma and Descemet’s membrane that
parallel the axis of the cone and disappear transiently on gentle digital pressure.

8) What are Fleisher’s rings?

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. The deposition occurs at the level
of basal epithelium. Locating this ring initially may be made easier by using a cobalt
filter and carefully focusing on the superior half of the corneal epithelium. Once
located, the ring should be viewed in white light to assess its extent.

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9) What is the clinical significance of Fleisher’s ring?
It delineates the extent of the base of the cone of the keratoconus, which helps during
the penetrating keratoplasty

10) What is acute hydrops?

The acute hydrops is caused by breaks in Descemet’s membrane with stromal
imbibition of aqueous through these breaks .The edema may persist for weeks or
months, usually diminishing gradually, with relief of pain and resolution of the
redness and corneal edema ultimately being replaced by scarring.

11) How is acute hydrops treated?

Acute hydrops is not an ophthalmic emergency and is treated conservatively with
topical hypertonic agents, patching or soft contact lens and mild cycloplegics. The
edema usually resolves within a few months.

12) What is the visual prognosis following healing of acute hydrops?

Acute hydrops is usually heals by scarring. The scarring can flatten the cornea and
decrease the astigmatism. The flattened cornea can be fitted with a contact lens easier.

13) Describe scissoring reflex on retinoscopy?

Clear cut central illumination and shadow move against the mirror. A less clearly
defined peripheral illumination and shadow move against the mirror. The two zones
of illumination being separated by a shaded ring.

14) Describe oil droplet sign.

It is seen with a dilated fundus examination. It is an annular dark shadow separating
the bright reflex of the central and peripheral areas. It occurs due to complete internal
reflection of the light.

15) What is swirl staining of cornea?

Swirl staining may occur in patients who have never worn contact lenses because
basal epithelial cells drop out and the epithelium slides from the periphery as the
cornea regenerates. Thus, a hurricane, vortex, or swirl stain may occur.

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16) What is forme fruste keratoconus?
Forme fruste keratoconus or Sub-clinical keratoconus is a clinical entity in which
there is no frank clinical sign of keratoconus, however, the cornea is at risk of
developing keratoconus at a later stage and can be diagnosed only by
videokeratography. Cornea is considered suspicious when

a) The central keratometry is more than 47.0 D

b) There is presence of an oblique astigmatism of > 1.5 D and
c) Superior-inferior curvature disparity of > 1.4 D on videokeratography.
The Massachusetts Eye and Ear Infirmary Keratoconus (KC) classification is
currently used to detect cases of forme-fruste keratoconus and variable grades of
clinical keratoconus.

17) Systemic disorders associated with keratoconus?

Crouzon’s syndrome
Down’s syndrome
Laurence –Moon-Bardet-Biedl syndrome
Marfan’s syndrome
Nail patella syndrome
Neurofibromatosis
Osteogenisis imperfecta
Pseudoxanthoma exasticum
Turner’s syndrome
Xeroderma pigmentosa

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18) Ocular associations of keratoconus?
Can be classified into corneal disorders and non corneal disorders
Corneal disorders Non corneal disorders
Atopic keratoconjunctivitis Retinitis pigmentosa
Axenfeld’s anomaly Aniridia
Corneal amyloidosis Congenital cataracts
Essential iris atrophy Leber’s congenital amaurosis
Fuchs’ corneal dystrophy Gyrate atrophy
Micro cornea Posterior lenticonus
Lattice dystrophy Vernal conjunctivitis

19) Why is keratoconus commonly associated with leber’s congenital amaurosis and
Down’s syndrome?
These two disorders are associated with increased incidence of eye rubbing. This is
due to increased incidence of Blepharitis in Down’s syndrome and oculo-digital sign
in leber’s congenital amaurosis. Recent study by Elder suggests that the association
might be due to genetic factors rather than eye rubbing.

20) What is the role of Contact lens wear in causing Keratoconus?

Contact lenses are suggested as a source of mechanical trauma to the cornea. It is
extremely difficult to determine which came first, the contact lens wear or the
keratoconus. It is possible that mechanical rubbing and hard contact lens wear can act
as environmental factors that enhance the progression of the disorder in genetically
predisposed individuals.
21) Describe the three types of cones seen in keratoconus?
Nipple cone – small in size (< 5 mm)
Steep curvature
Apical centre usually lies central or paracentral
Easiest to fit with contact lens
Oval cone – larger (5 -6 mm) ellipsoid displaced inferotemproally.
Globus cone – larger (>6mm) may involve more than 75% of the cornea.
Most difficult to fit with contact lenses

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22) What is posterior keratoconus?
It’s a congenital corneal anomaly unrelated to keratoconus which is characterised by
protrusion of posterior corneal surface into the stroma and is usually sporadic,
unilateral and non progressive.

23) What are Rabinowitz’s criteria for diagnosis of keratoconus?

1) Keratometry value >47.2 D
2) Steepening of inferior cornea compared with the superior cornea of >1.2D
3) Skewing of the radial axis of astigmatism by greater than21
4) Difference in central power of more than 1D between the fellow eye

24) Describe the histopathology of corneas in keratoconus?

Thinning of corneal stroma, breaks in Bowman’s layer and deposition of iron in the
basal layer of the corneal epithelium comprise a triad of the classic histopathologic
features found in keratoconus.
Epithelium – Degeneration of basal cells.
Breaks accompanied by downgrowth of epithelium into
Bowman’s layer.
Accumulation of ferritin particles within and between basal
epithelial cells.
Bowman’s layer - Breaks filled with eruptions of underlying stromal collagen.
Reticular scarring.
Stroma - Compactions and loss of arrangement of fibrils in the anterior
stroma.
Decrease in number of collagen lamellae.
Decemet’s membrane - Rarely affected except in cases with acute hydrops.
Endothelium - Usually normal.

25) Describe the biochemical abnormalities noted in corneas with keratoconus?

The underlying biochemical process and etiologic basis of keratoconus is poorly
understood. The loss of corneal stroma is due to degradatation process aberrant in
keratoconus.

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 The increased degereration of corneal stroma is due to
1. There is decreased levels of α 1 proteinase inhibitor, TIMP -1 proteinase
inhibitor and α2 macroglobin levels in keratoconus cornea.
2. The loss of anterior stromal keratinocytes is due to apoptotic cell death.
3. Keratinocytes have fourfold increased expression of interleukein 1
receptors. Interleukein 1 is released from epithelial and endothelial cells
and IL 1 can cause loss of keratinocytes through apoptosis and loss of
corneal stroma over a period of time.

26) Classify keratoconus based on keratometry?

Mild <45 D in both meridians
Moderate 45-52 D in both meridians
Advanced >52 D in both meridians
Severe >62 D in both meridians

27) How is a case of keratoconus managed?

The treatment approach to keratoconus follows an orderly progression from glasses
to contact lenses to corneal transplantation. In the initial stages glasses can be
prescribed to provide good vision.
The mainstay of management remains use of rigid gas permeable contact lens. A gas
permeable lens covers the irregular protrusion on the cornea and makes a new smooth
surface for the light to bend through.
Different types of contact lens designs are available to correct keratoconus.
When astigmatism progresses and is difficult to correct with contact lens or patient is
intolerant to contact lens, surgical options like PKP or lamellar keratoplasty is done.

28) What are the various types of contact lenses used in the management of
keratoconus?
Rigid gas permeable contact lens
Tricurve flex lens for nipple cone
Soper lens system
McGuire lens system
Rose k design
NiCone design

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 Bausch and Lomb C series
Double posterior curve lenses
One central curve to fit the corneal apex
Another flatter curve peripheral to the central apical zone to fit the mid corneal
periphery
Piggy back lenses – gas permeable firm lens is fitted upon a soft lens or flex lens
system of a hard lens fitted in to the groove of a soft lens.
Hard lens with a soft peripheral skirt.

30) What is ‘three point touch’ techinique of contact lens fitting?

The lens lightly touches the peak of the cone, then a very low vault over the edges of
the cone, and lastly a thin band of touching near the edge of the lens. The name
“three-point touch” refers to the edge-peak-edge pattern of the lens touching the
cornea. The lens is kept as small as is optically possible. Since the lens will center
itself over the peak of the cone, an off-center cone needs a bigger lens than a centered
cone.

31) What is the fluorescein pattern of a well – fit lens in a keratoconic patient?
¾ Slight central bearing
¾ Intermediate pooling of tears
¾ Peripheral bearing or touch over some portion of the lens circumference and
perhaps slight peripheral lift at the steepest site of the cone.

32) What are soft Perm lens?

The SoftPerm lens (from WJ/PBH, San Diego, CA) is a hybrid lens with a rigid, gas-
permeable center surrounded by a soft, hydrophilic skirt. This lens may be indicated
for patients with displaced corneal apexes or for patients who cannot tolerate rigid
lenses. However, in advanced keratoconus, in which a lens of larger diameter is
useful, the lack of steep base curves in the SoftPerm lens (its steepest base curve is 6.5
mm) limits performance. In addition, the lens material has a low DK value (rigid lens,
14 DK; soft portion, 5.5 DK).

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33) What is Soper lens system?
The objective of the Soper lens system is based on sagittal depth. The principle is that
a constant base curve with an increased diameter results in increased sagittal depth
and a steeper lens. The lenses included in the fitting set are categorized as mild (7.5-
mm diameter, 6.0-mm optic zone diameter), moderate (8.5-mm diameter, 7.0-mm
optic zone diameter), and advanced (9.5-mm diameter, 8.0-mm optic zone diameter).
The initial trial lens is selected on the basis of degree of advancement of the cone. The
more advanced the cone, the larger the diameter of the recommended lens; the smaller
and more centrally located the apex, the smaller the diameter of the lens.

34) What is Thermo- keratoplasty?

Thermokeratoplasty is corneal flattening by heat application, which may regularize
the corneal surface. It is often used to flatten the cornea at the time of keratoplasy to
make trephining easier.

35) What is epi keratoplasty?

A corneal lenticule is sewnover the keratoconus area, flattening the cone, reducing the
myopic astigmatism and improving contact lens fit. It is preferred in conditions like
Down’s syndrome because of its non invasive nature and decreased potential for
corneal graft rejection.

36) What is excimer laser phototheraputic keratectomy?

It is useful in management of patients with keratoconus who have nodular sub
epithelial corneal scars who are contact lens intolerant.

37) Why is prognosis of PKP good in cases of keratoconus?

I. Absence of vascularisation in the lesion
II. Keratoconus is noninflamatory ectatic condition.

38) What is collagen cross linkage?

Corneal collagen cross-linking riboflavin is a noninvasive procedure which
strengthens the weak corneal structure in keratoconus.

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 This technique works by increasing collagen cross-linking, which are the natural
“anchors” within the cornea. riboflavin eye drops are applied to the cornea, which is
then activated by a UVA light. This increases the amount of collagen cross-linking in
the cornea and strengthens the cornea. The technique uses riboflavin to create new
bonds between the adjacent collagen molecules so that the cornea is about one-and-a-
half times thicker and less malleable.

39) Explain the mechanism of action of collagen cross linkage.

Application of riboflavin on the cornea along with penetration for approximately 200
ìm and irradiation of the riboflavin molecules through UVA leads to loss of the
internal chemical balance of the riboflavin molecules, producing oxygen free radicals.
The riboflavin molecule becomes unstable and stabilizes only when it is linked to two
collagen fibrils. A cross bridge is created between the collagen fibrils (ie, cross-
linking) to produce a general strengthening of the cornea.

40) Compare and contrast the clinical features of keratoconus, keratoglobus and
terrien’s marginal degeneration?
Keratoconus Keratoglobus Terrien’s Pellucid
Age group Progress during Presents at Presents at 4 to Presents
adolescence birth 5th decade between 2 – 3rd
decade
Appearance Progressive Globular Begins Causes inferior
thinning of deformation superiorly and thinning of
central/paracentral of entire spreads cornea
cornea cornea circumferentially
Vascularisation - - Forms pannus -
Familial inheritance Most cases are Dominant - -
sporadic inheritance
with
incomplete
penetrance
Laterality Bilateral. usually Bilateral. Unilateral or Bilateral
asymmetrical Usually asymmetrically
asymmetrical bilateral
Prognosis after pkp Very favourable - Good prognosis -
prognosis after lamellar
keratoplasty

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