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Dr. Mohd Najmussadiq Khan
Disease
 Keratoconus is a degenerative non-inflammatory disease of the
  cornea where the central or paracentral cornea undergoes
  progressive thinning and steepening causing irregular
  astigmatism.


Etiology
 Etiology is unknown.
 However, it is associated with atopy, Down’s Syndrome, Leber’s
  congenital amaurosis, and Ehler’s Danlos/connective disorders.
 The hereditary pattern is neither prominent nor predictable, but
  positive family histories have been reported.
 The most common presentation of keratoconus is as a sporadic
  disorder, in which only a significant minority of patients exhibit a
  family history with autosomal dominant or recessive transmission
 The incidence of keratoconus if often reported to be 1 in 2000
  people.


                                     Dr. Mohd Najmussadiq Khan   11/7/2012   2
Risk Factors
 Eye rubbing, associated with atopy
 Sleep apnea
 Floppy Lid Syndrome




                         Dr. Mohd Najmussadiq Khan   11/7/2012   3
   Based on severity of curvature
    ◦ Mild : less than 45.00D
    ◦ Moderate : 45.00 to 52.00D
    ◦ Advanced : 52.00 to 62.00D
    ◦ Severe : more than 62.00D

   Based on shape:
    ◦ Nipple cones (Small size 5mm )
    ◦ Oval cones (larger (5-6mm) ellipsoid)
    ◦ Globus cones (Largest >6mm ,may involve over 75%
      of cornea. )

                                Dr. Mohd Najmussadiq Khan   11/7/2012   4
   Retinitis Pigmentosa
   Leber’s Congenital Amaurosis
   Microcornea
   Aniridia
   Corneal Degeneration
   Congenital Cataract
   Ectopia lentis
   Lenticonus
   Macular coloboma
   Retinal dysplasia
   Floppy eyelid syndrome


                            Dr. Mohd Najmussadiq Khan   11/7/2012   5
Dr. Mohd Najmussadiq Khan   11/7/2012   6
   Down’s syndrome
   Ehlers-Danlos syndrome
   Osteogenesis Imperfecta
   Mitral valve prolapsed




                          Dr. Mohd Najmussadiq Khan   11/7/2012   7
General Pathology --Keratoconus can show the
  following pathologic findings
 Deposition of iron in the basal epithelial cells,
  forming the Fleischer ring—a pigmented ring
  that results from the accumulation of ferritin
  particles in the cytoplasm of epithelial cells
  and widened intercellular spaces.
 Breaks in Bowman's membrane, filled with cells,
  collagen, and PAS-positive material.




                          Dr. Mohd Najmussadiq Khan   11/7/2012   8
Dr. Mohd Najmussadiq Khan   11/7/2012   9
   thinning of stroma and overlying epithelium
   abnormal keratocyte morphology, and
    endothelial polymorphism
   folds or breaks in Descemet’s membrane
    (Vogts striae)
   variable amounts of diffuse corneal scarring.




                            Dr. Mohd Najmussadiq Khan   11/7/2012   10
Dr. Mohd Najmussadiq Khan   11/7/2012   11
Dr. Mohd Najmussadiq Khan   11/7/2012   12
Pathophysiology
 Histopathology studies demonstrate breaks in or
  complete absence of Bowman’s layer, collagen
  disorganization, scarring and thinning.
 The etiology of these changes is not known,
  though some suspect changes in enzymes that
  lead to breakdown of collagen in the cornea.
 While a genetic predisposition to keratoconus is
  suggested, a specific gene has not been
  identified.



                          Dr. Mohd Najmussadiq Khan   11/7/2012   13
Biochemical abnormalities:--
 Studies in patients with keratoconus have
  demonstrated an increased activity by
  proteases that breakdown the collagen cross-
  linkages in the corneal stroma.
 There is a simultaneous reduced expression
  of protease inhibitors.
 The ratio of keratin sulfate to dermatan
  sulfate is altered in the stromal matrix when
  compared to normal corneas.
 Patients with keratoconus have also shown
  enzymatic changes in the epithelium with
  increased expression of lysosomal and
  proteolytic enzymes.
                         Dr. Mohd Najmussadiq Khan   11/7/2012   14
Primary prevention
 No preventive strategy has been proven
  effective to date.
 Some feel that eye rubbing or pressure (e.g.
  sleeping with the hand against the eye) can
  cause and/or lead to progression of
  keratoconus, so patients should be informed
  not to rub the eyes.
 In some patients, avoidance of allergens may
  help decrease eye irritation and therefore
  decrease eye rubbing.


                         Dr. Mohd Najmussadiq Khan   11/7/2012   15
Diagnosis
 Diagnosis can be made by slit-lamp examination
  and observation of central or inferior corneal
  thinning.
 Computerized videokeratography is also useful in
  detecting early keratoconus and allows following its
  progression.
 Ultrasound pachymetry can also be used to measure
  the thinnest zone on the cornea.
 New        algorithms       using       computerized
  videokeratopgraphy have been devised which now
  allow the detection of subclinical or suspected
  keratoconus.
 These devices may allow better screening of
  patients for prospective refractive surgery.



                            Dr. Mohd Najmussadiq Khan   11/7/2012   16
Dr. Mohd Najmussadiq Khan   11/7/2012   17
Dr. Mohd Najmussadiq Khan   11/7/2012   18
History
 The majority of cases of keratoconus are bilateral,
  and often asymmetric.
 The less affected eye may show a high amount of
  astigmatism or mild steepening.
 Onset is typically in early adolescence         and
  progresses into the mid-20’s and 30’s. However,
  cases may begin much earlier or later in life.
 There is variable progression for each individual.
 There is often a history of frequent changes in eye
  glasses which do not adequately correct vision.
 Another    common progression is from soft
  contact lenses, to Toric or astigmatism correcting
  contact lens, to rigid gas permeable contact lenes.
                            Dr. Mohd Najmussadiq Khan   11/7/2012   19
Dr. Mohd Najmussadiq Khan   11/7/2012   20
Physical examination
 A thorough and complete eye exam should be
  performed on any patient suspected of having
  keratoconus.

   The general health of the eye should be assessed
    and appropriate ancillary tests should be done to
    assess corneal curvature, astigmatism and
    thickness.

   The best potential vision should also be evaluated.
    Many of the potential exam components are listed
    below:

   Proper history, including change in eye glass
    prescription, decreased vision, history of eye
    rubbing, medical problems, allergies, sleep patterns

                              Dr. Mohd Najmussadiq Khan   11/7/2012   21
   Assessment of relevant aspects of the patient’s mental and
    physical status.

   Measurement of best corrected visual acuity with spectacles and
    hard or gas permeaable contact lenses (with refraction when
    indicated)

   Measurement of pinhole visual acuity

   External examination (lids, lashes, lacrimal apparatus, orbit)

   Examination of ocular alignment and motility
   Assessment of pupillary function

   Measurement of intraocular pressure (IOP)

   Slit-lamp biomicroscopy of the anterior segment

   Dilated examination of the lens, macula, peripheral retina, optic
    nerve, and vitreous

   Keratomety/Computerized Topography//Ultrasound Pachymetry
                                     Dr. Mohd Najmussadiq Khan   11/7/2012   22
Symptoms
 Progressively poor vision not easily corrected
  with eye glasses.
Clinical diagnosis
 Diagnosis is made based on history of
  changing refraction, poor best spectacle
  corrected     vision,     abnormalities      in
  keratometry, corneal topography and corneal
                     thinning.



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Dr. Mohd Najmussadiq Khan   11/7/2012   24
   Asymmetric refractive error with high or
    progressive astigmatism
   Keratometry showing high astigmatism and
    irregularity (axis that do not add to 180 degrees)
   Scissoring of the red reflex on ophthalmoscopy
    or retinoscopy.
   Inferior steepening, skewed axis, or elevated
    keratometry      values    on    K   reading   and
    computerized corneal topography



                            Dr. Mohd Najmussadiq Khan   11/7/2012   25
   Corneal thinning, especially in inferior cornea.
    Maximum corneal thinning corresponds to the site of
    maximum steepening or prominence.
   Rizutti’s sign or a conical reflection on nasal cornea
    when a penlight is shone from the temporal side
   Fleischer ring, an iron deposit often present within
    the epithelium around the base of the cone. It is
    brown in color and best visualized with a cobalt blue
    filter
   Vogt’s striae, fine, roughly vertically parallel striations
    in the stroma. These generally disappear with firm
    pressure applied over the eyeball and re-appear
    when pressure is discontinued.


                                  Dr. Mohd Najmussadiq Khan   11/7/2012   26
   Munson’s sign, a protrusion of the lower eyelid in
    down gaze.
   Superficial scarring • Break’s in Bowman’s
    membrane

   Acute hydrop’s, a condition where a break in
    Descemet’s membrane allows aqueous into the
    stoma causing severe corneal thickening,
    decreased        vision      and        pain.

   Stromal scarring after resolution of acute
    hydrops, which paradoxically may improve vision
    in some cases by changing corneal curvature and
    reducing the irregular astigmatism.
                             Dr. Mohd Najmussadiq Khan   11/7/2012   27
Dr. Mohd Najmussadiq Khan   11/7/2012   28
Dr. Mohd Najmussadiq Khan   11/7/2012   29
   Differentiation is made between a “quiet” type
    of keratoconus (“forme fruste”) and
    progressive keratoconus.
   “Forme fruste” occurs 10 times more often
    than progressive keratoconus.
   Normally it produces no symptoms and has
    only to be observed.
   If the condition remains stable, there is no
    need for treatment.



                           Dr. Mohd Najmussadiq Khan   11/7/2012   30
   Progressive keratoconus is aggressive and can
    begin at a very early age.
   With progression of the disease, correction of
    visual acuity with glasses becomes more difficult
    because protrusion of the cornea develops
    unevenly.
   Hard contact lenses are a good solution because
    they put pressure on the cornea, thus correcting
    irregularities.



                            Dr. Mohd Najmussadiq Khan   11/7/2012   31
Diagnostic procedures
 • Ophthalmic history, including family eye history,
  heritable disease, history of allergies, etc

   • Slit-lamp examination

   • Hard or gas permeable contact lens trial because good
    vision with lenses eliminates other sources of poor vision,
    including amploypia

   • Measurement of K values

   • Ultrasound Pachymetry

   • Computerized corneal topography


                                   Dr. Mohd Najmussadiq Khan   11/7/2012   32
Dr. Mohd Najmussadiq Khan   11/7/2012   33
Dr. Mohd Najmussadiq Khan   11/7/2012   34
Dr. Mohd Najmussadiq Khan   11/7/2012   35
Dr. Mohd Najmussadiq Khan   11/7/2012   36
Dr. Mohd Najmussadiq Khan   11/7/2012   37
Dr. Mohd Najmussadiq Khan   11/7/2012   38
Laboratory test--Assessment of heritable diseases
  including
 • Down’s Syndrome
 • Leber’s congenital amaurosis
 • Ehlers Danlos/connective disorder.
 • Other medical conditions including sleep apnea


Differential diagnosis
 • Keratoconus
 . Pellucid marginal degeneration
 • Keratoglobus
 • Contact lens induced corneal warpage
 • Coneal Ectasia post excimer laser treatment




                             Dr. Mohd Najmussadiq Khan   11/7/2012   39
   In 1963, scientists found that the initial
    changes of KC were related to degradation
    (break down) of the cornea s basement
    membrane.
   They found that over time, a cascade of events
    occurred, leading to alterations in the layers of
    the cornea and resulting in the thinning of the
    stromal layer.
   This weakened the structural integrity of the
    cornea and in turn resulted in a bulging, cone-
    like distortion of the normally spherical cornea.



                            Dr. Mohd Najmussadiq Khan   11/7/2012   40
Dr. Mohd Najmussadiq Khan   11/7/2012   41
   Investigators found that the enzyme activities
    of the KC corneas were increased compared to
    normal corneas.
   In addition, there was a significant reduction of
    inhibitors for these enzymes.
   The cornea is responsible for approximately
    80% of the absorption of ultraviolet B (UVB)
    light that enters the eye.
   UV light generates free oxygen radicals, high
    energy molecules, which if unchecked, can
    damage tissue.
                              Dr. Mohd Najmussadiq Khan   11/7/2012   42
Typically, the free radicals are removed from
the cornea via antioxidants (such as superoxide
dismutase, catalase and glutathione reductase).

Those that are not removed undergo reactions
that form aldehydes, which can be destructive
to the tissue




                        Dr. Mohd Najmussadiq Khan   11/7/2012   43
   Normally, the cornea protects itself from these
    aldehydes with an enzyme called aldehyde
    dehydrogenase (ALDH) which detoxifies these
    aldehydes.
   ALDH is a major protein within the cornea.
    (Approximately 78% of the ALDH within the eye
    is found in the cornea.).
   In the KC cornea there is decreased activity of
    ALDH enzyme.


                           Dr. Mohd Najmussadiq Khan   11/7/2012   44
These peroxynitrites are also harmful to tissues and can
be identified by using antibodies to nitrotyrosine.

KC corneas have an increased amount of nitroytrosine
staining compared to normal corneas

This increased staining in KC corneas suggests that they
have additional destructive substances in the tissue.

                               Dr. Mohd Najmussadiq Khan   11/7/2012   45
   Normally, the human cornea has very little
    apoptosis occurring.
   Dr. Wilson demonstrated that KC corneas
    have apoptosis occurring in the anterior
    stroma and epithelium, especially in the areas
    of breaks in Bowman's layer.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   46
   KC corneas produce a unique enzyme that is not
    found in normal corneas or corneas with other
    diseases.
   This enzyme is called leukocyte common
    antigen related protein (LAR).
   LAR in KC corneas also supports the notion of
    apoptosis in KC corneas because other
    investigators have shown in other systems that
    LAR expression plays a role in apoptosis.


                           Dr. Mohd Najmussadiq Khan   11/7/2012   47
   The goal of future research studies is to
    understand the biochemical and molecular
    changes and possible genetic influences that
    occur in KC corneas.
   Intervention at one of these steps might be
    the key factor needed to block the
    progression of keratoconus.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   48
General treatment --The goal of treatment is
 primarily to provide functional visual acutiy.
 More recently attention has been directed to
 halting changes in the corrneal shape.

   Initially, spcetacles or soft toric contact lenses in
    mild cases can be used.


                               Dr. Mohd Najmussadiq Khan   11/7/2012   49
Dr. Mohd Najmussadiq Khan   11/7/2012   50
Dr. Mohd Najmussadiq Khan   11/7/2012   51
   Patients with very mild disease may initially
    be corrected with glasses or soft contact
    lenses.
   However the vast majority of patients need
    rigid contact lenses for adequate vision
    correction.
   The very latest contact lens for treating
    keratoconus is the “synergize hybrid contact
    lens” which is rigid in the middle and soft on
    the edges.
                            Dr. Mohd Najmussadiq Khan   11/7/2012   52
   Needed in the majority of cases to neutralize the
    irregular corneal astigmatism.
   It resurfaces the irregular cornea & the
    intervening fluid and corrects the irregular
    astigmatism to provide good quality of vision.
   However the disadvantage with these lenses is
    that they have standard lens design with fixed
    optical zones and do not give an ideal fit in
    patients with keratoconus
   The majority of patients that can wear hard or
    gas-permeable contact lenses have a dramatic
    improvement in their vision.
                            Dr. Mohd Najmussadiq Khan   11/7/2012   53
Dr. Mohd Najmussadiq Khan   11/7/2012   54
   Many patients find their contact lenses
    uncomfortable and can only tolerate their
    contact lenses for a short period of time.
   The reason is that the cornea steepens and
    rubs against the lens causing an abrasion and
    light sensitivity .

   Another reason is patients with keratoconus
    often have very dry eye and as the eye dries
    out there is no lubricating barrier between
    the lens and the cornea contributing to the
    patient being uncomfortable.
                           Dr. Mohd Najmussadiq Khan   11/7/2012   55
   RoseK-- The Rose-k lenses are made with
    complex geometry to fit any type of cone. The
    optical zone is reduced to snugly fit the cone &
    the peripheral curve is computer designed to fit
    the rest of the irregular cornea.




                             Dr. Mohd Najmussadiq Khan   11/7/2012   56
   piggy back lens-- they are known as piggy
    back because a RGP Lens is fitted on top of a
    soft contact lens. These lenses provide
    excellent comfort and good vision.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   57
   Scleral Contact Lenses: They are made of a
    special polymer called Itaflurocon. They
    characteristically vault over the cornea and
    limbus and are supported entirely by the
    sclera. The fluid lens smoothens the irregular
    cornea and provides good vision. It also
    prevents desiccation due to its very high
    oxygen permeability. The small pores on the
    lens provide adequate exchange of tears. The
    disadvantage with these lenses is that they
    are difficult to use. They are large lenses
    which are cumbersome to the patients.
                            Dr. Mohd Najmussadiq Khan   11/7/2012   58
   Boston Scleral Contact lenses (BSLP): BSLP
    lenses are made of Flurosilicone Acrylate
    Polmers with DK values of 87 and 130. Its
    diameter ranges from15.5mm to 20mm. The
    space created over the cornea is filled with
    non-preserved, buffered sterile saline. The
    limitation to the use of these lenses is that the
    fitting process is skill intensive and time
    consuming.




                              Dr. Mohd Najmussadiq Khan   11/7/2012   59
Those patients that become contact lens
intolerant or do not have acceptable vision,
typically from central scaring, can proceed to
surgical alternatives




                       Dr. Mohd Najmussadiq Khan   11/7/2012   60
Medical therapy—for acute corneal hydrops
  involves
 a cycloplegic agent
 sodium chloride 5% ointment or eye drop
 Lubricating eye drop
 Steroid drops can be used if the eye is
  congested
 a pressure patch
 After the pressure patch is removed patients
  may still need to continue sodium chloride
  drops or ointment for several weeks to months.
 Patients are advised to avoid vigorous eye
  rubbing
                          Dr. Mohd Najmussadiq Khan   11/7/2012   61
Dr. Mohd Najmussadiq Khan   11/7/2012   62
   It takes 3 - 4 months for the corneal edema to
    resolve, following which a standard full
    thickness keratoplasty is required to restore
    corneal clarity and visual improvement.
   It this situation lamellar surgery is not
    recommended
   Thermokeratoplasty has been tried and
    believed to shrinkage of the collagen with
    resultant flattening of the hydrops which helps
    in early healing of the ruptured DM.


                            Dr. Mohd Najmussadiq Khan   11/7/2012   63
   Patients are usually followed on a 6-month to
    yearly basis to monitor the progression of the
    corneal thinning, steepening, the resultant
    visual changes, and to re-evaluate contact
    lens fit and care.
   Patients with hydrops are seen more
    frequently until it resolves.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   64
   When patients become intolerant or no longer
    benefit from contact lenses, surgery is the next
    option. Surgical options can include: INTACS,
    Anterior lamellar keratoplasty, penetrating
    keratoplasty.
   Non- FDA approved treatments, which typically have
    less evidence based information available on safety
    and efficacy, include use of UV/riboflavin collagen
    cross-linking of the cornea to stiffen the cornea and
    possibly prevent progressive changes in shape. This
    treatment has also be experimentally combined with
    excimer laser treatment, conductive keratoplasty,
    and/or INTACS.
   Some surgeons will use phakic IOLs to address high
    myopia and some of the asitigmatism.

                               Dr. Mohd Najmussadiq Khan   11/7/2012   65
Dr. Mohd Najmussadiq Khan   11/7/2012   66
   The most promising technology for treating
    Keratoconus called collagen crosslinking
    (CXL) with UVA is currently being introduced
   It is undergoing Phase 1 FDA clinical trials in
    the United States.
   It has been demonstrated to be safe and
    effective if performed, with the epithelium
    removed, and has the potential to stop the
    progression of Keratoconus.




                             Dr. Mohd Najmussadiq Khan   11/7/2012   67
   This treatment is recommended for
    individuals with progressive Keratoconus or
    Ectasia following LASIK to stabilize the
    cornea.
   It can be performed with our without INTACS.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   68
CORNEAL COLLAGEN CROSS-LINKING (C3R/CXL):
 The basic problem in keratoconus is the weakness of
  the cross links which act as anchors between the
  collagen fibrils.
 UV-A irradiance of the cornea after sensitization with
  riboflavin, augments these cross-links, and hence
  recovers some of the corneal mechanical strength in
  keratoconus.
 This treatment modality stabilizes the collagen scaffold
  and increases the stiffness of the cornea against the
  action of proteolytic enzymes.
 Riboflavin/UVA light causes increase in the corneal
  stiffness of the anterior 200-250 μm.
 Deeper structures like the lens and endothelium do not
  get damaged.
 The progression of keratoconus or keratectasia can be
  stopped.
                              Dr. Mohd Najmussadiq Khan   11/7/2012   69
   This results in cross linking of the collagen
    fibres of the cornea, thereby increasing
    its physical strength by up to 300%.
   Thus further progression of keratoconus can
    be arrested, and in 15 -20% cases regression
    has also been noted.
   Longest follow-up duration following this
    treatment is for 7 years.




                           Dr. Mohd Najmussadiq Khan   11/7/2012   70
Dr. Mohd Najmussadiq Khan   11/7/2012   71
Dr. Mohd Najmussadiq Khan   11/7/2012   72
   The top layer of the cornea is removed under local
    anesthesia.
   Vitamin drops are soaked into the cornea until they
    penetrate the entire corneal and evidence of
    penetration into the anterior chamber of the eye is
    demonstrated by slit-lamp evaluation.
   Once this is confirmed the patient’s eye is put under a
    specialized lamp, which emits UV A light at a
    predetermined wavelength for approximately 30
    minutes.
   During this process the cross links, which link the
    fibers of the cornea, are increased thereby stiffening
    the whole cornea.
   A bandage contact lens is then put on the eye and
    patients are given antibiotics and anti-inflammatory
    drops and follow up on a regular basis with their
    physicians for several months.
                                 Dr. Mohd Najmussadiq Khan   11/7/2012   73
Dr. Mohd Najmussadiq Khan   11/7/2012   74
Dr. Mohd Najmussadiq Khan   11/7/2012   75
   During the post-operative period flurometholone
    and artificial tears are applied for a period of one
    month.
   In the pilot study done on 23 eyes and followed up
    to 4 years Wollensak et al found a mean reduction
    in steepening of 2.01 D.
   Other investigators such as caporossi et al and
    Krumeich et al reported a similar result.
   The improvement in vision after cross-linking is
    caused by a decrease in astigmatism and corneal
    curvature as well as topographical homogenization
    of the cornea as a result of the increased rigidity in
    the cross- linked cornea.
   In addition tolerance to contact lenses improved.
    Cross-linking is a practical outpatient treatment,
    which is minimally invasive and cost-effective.

                                Dr. Mohd Najmussadiq Khan   11/7/2012   76
Dr. Mohd Najmussadiq Khan   11/7/2012   77
   A method for flattening the cornea that is too
    steep and making a patient more contact lens
    tolerant is the insertion of INTACS into the
    cornea.
   This procedure is good for patients who are
    contact lens intolerant and who want to avoid
    a corneal transplant and whose K readings
    are not in excess of 58 Diopters.
   (INTACS ) have been approved for the
    treatment of mild to moderate keratoconus in
    patients who are contact lens intolerant.


                           Dr. Mohd Najmussadiq Khan   11/7/2012   78
   In these cases, patients must have a clear central
    cornea and a corneal thickness of > 450 microns
    where the segments are inserted, approximately
    at 7 mm optical zone.
   Intrastromal corneal ring segments (Intacs
    Addition Technology Inc) have crescent shaped
    two 150-degree PMMA segments that are
    surgically placed in the peripheral cornea at two-
    thirds corneal depth.
    Intacs comes in thickness ranging from 0.25 to
    0.45 mm in 0.05 mm increments.
   Intacs correct the myopia and irregular
    astigmatism by producing a mechanical flattening
    of the central cone.
                             Dr. Mohd Najmussadiq Khan   11/7/2012   79
   The advantage of INTACS is that they require no
    removal of corneal tissue, no intraocular
    incision, and leave the central cornea
    untouched.
   Most patients will need spectacles and/or
    contact lenses post-operatively for best vision,
    but will have flatter corneas and easier use of
    lenses.
   If a patient does not gain the expected results,
    the INTACS can be removed and then other
    surgical options can be considered



                             Dr. Mohd Najmussadiq Khan   11/7/2012   80
Dr. Mohd Najmussadiq Khan   11/7/2012   81
   Femtosecond laser technology allows
    realization with very high accuracy of
    individualized shape and size of corneal tunnel
    depending on the surgical need.
   Intrastromal cuts from the inner to the outer
    parts of the cornea can be performed easily.
   During surgery, stress to the cornea is
    minimal, because only moderate pressure is
    exerted on the eye.
   The risk of infection is significantly reduced.



                           Dr. Mohd Najmussadiq Khan   11/7/2012   82
   Kera rings are tiny plastic semicircular rings
    surgically implanted into the cornea to flatten
    the corneal surface and improve vision.
   Inserts can improve contact lens wear in most
    patients.
   They are not suitable for all patients with
    keratoconus.
   Implantation does not affect the central optic
    zone, does not involve the removal of any
    tissue, and can be reversed if vision changes.



                            Dr. Mohd Najmussadiq Khan   11/7/2012   83
Imagine your cornea as
a tent with a curved top.
If you push out the sides
of the tent, the top
flattens.
Similarly, when Kera
rings are placed in the
sides of the cornea, they
flatten it.


                      Dr. Mohd Najmussadiq Khan   11/7/2012   84
Combined KERARINGS insertion and CXL can be performed safely in one or
two sessions. However, the same-session procedure appears to be more
effective regarding the improvement in the corneal shape. Br J Ophthalmol.
2011 Jan;95(1):37-41. Epub 2010 Jun 28.




                                       Dr. Mohd Najmussadiq Khan   11/7/2012   85
   Laser may now be used to treat keratoconus.
   The technique is similar to the Lasek laser done for
    short sight etc, but uses a cornea topographer to
    guide the laser.
   The laser reduces the keratoconic protrusion,
    significantly improving vision.
   When controlled by a topographer only a few microns
    of cornea need be removed, greatly enhancing safety.
   The technique is effective for mild-to-moderate
    keratoconus.
   To stabilize the cornea after laser, collagen cross
    linking is done.
   Only the most sophisticated lasers, such as the Alcon
    Wave light EX500 excimer laser can be used
                              Dr. Mohd Najmussadiq Khan   11/7/2012   86
Dr. Mohd Najmussadiq Khan   11/7/2012   87
   It is the newest procedure under investigation for the
    treatment of keratoconus.
   It is non-incisional and reshapes the cornea without
    removing any tissue.
   It is currently under clinical investigation in Europe for
    treating myopia and keratoconus.
   It involves the delivery of a single low energy
    microwave pulse lasting less than one second to the
    cornea.
   Energy is applied to the cornea using a dielectrically
    shielded microwave emitter which contacts the
    epithelial surface.
   Through capacitive coupling, the single pulse raises the
    temperature of the selected region of corneal stroma to
    approximately 65°C, forming a doughnut-shaped area
    of collagen shrinkage in the upper 150 microns of the
    stroma.
                                 Dr. Mohd Najmussadiq Khan   11/7/2012   88
   The lesion created during Keraflex is intended
    to flatten the central cornea both to decrease
    the cone in keratoconus and to achieve myopic
    correction.
   In order to improve the stability of the cornea
    with Keraflex, Keraflex KXL includes focal
    corneal collagen crosslinking.
   After the actual Keraflex procedure, riboflavin
    drops are administered over the treatment
    area, a mask is applied to protect the central
    and peripheral areas of the cornea, and
    ultraviolet light is admistered to crosslink the
    collagen.
                            Dr. Mohd Najmussadiq Khan   11/7/2012   89
   Corneal Transplants are the only option for
    patients who have scarring in the center of
    the cornea or who are contact lens intolerant
    because their corneas are too steep.
   The results of corneal transplants are
    excellent in keratoconus patients with an over
    97% success rate.
   Patients can have LASIK or PRK on their
    transplants and become relatively
    independent of glasses or contact lenses.
   Recently the Femtosecond Laser was
    approved for performing Corneal Transplants


                           Dr. Mohd Najmussadiq Khan   11/7/2012   90
   It involves replacement of the central anterior
    cornea, leaving the patient’s endothelium
    intact.
   The advantages are that the risk of endothelial
    graft rejection is eliminated, and there is less
    risk of traumatic rupture of the globe in the
    incision, since the endothelium and
    Descemet’s and some stroma are left intact,
    and faster visual rehabilitation.



                             Dr. Mohd Najmussadiq Khan   11/7/2012   91
   There are several techniques utilized including,
    deep anterior lamellar keratoplasty (DALK) and
    big bubble keratoplasty(BBK) to remove the
    anterior stroma, while leaving Descemet’s layer
    and endothelium untouched.
   However, the procedures can be technically
    challenging requiring conversion to a penetrating
    keratoplasty
   Post-operatively there is the possibility of
    interface haze leading to a decrease in BCVA
   It is not clear if astigmatism is better treated with
    anterior vs penetrating keratoplasty.


                               Dr. Mohd Najmussadiq Khan   11/7/2012   92
Dr. Mohd Najmussadiq Khan   11/7/2012   93
Dr. Mohd Najmussadiq Khan   11/7/2012   94
   In DALK the bottom layer of the cornea is
    spared this results in less chance of rejection.
   It should be remembered however that in
    some instances the separation of the bottom
    layers of the cornea and the upper parts might
    be uneven which would result in poorer visual
    outcomes.




                             Dr. Mohd Najmussadiq Khan   11/7/2012   95
Dr. Mohd Najmussadiq Khan   11/7/2012   96
Descemets exposed




DALK(deepanteriorlamellarkeratoplasty




             Dr. Mohd Najmussadiq Khan   11/7/2012   97
   It has a high success rate and is the standard
    surgical treatment with a long track record of
    safety and efficacy.
   Risks of this procedure include infection and
    cornea rejection and risk of traumatic rupture at
    wound margin.
   Many patients after PK may still need hard or gas-
    permeable contact lenses due to residual irregular
    astigmatism
   Any type of refractive procedure is considered a
    contraindication in keratoconic patients due to the
    unpredictability of the outcome and risk of leading
    to increased and unstable irregular astigmatism.

                             Dr. Mohd Najmussadiq Khan   11/7/2012   98
Dr. Mohd Najmussadiq Khan   11/7/2012   99
10
Dr. Mohd Najmussadiq Khan   11/7/2012    0
10
Dr. Mohd Najmussadiq Khan   11/7/2012    1
   Following any corneal surgical procedure,
    patients need to be followed to complete visual
    rehabilitation.
   Most patients still require vision correction with
    spectacles or contact lenses, and often hard or
    gas permeable lenses are required if high levels
    of astigmatism are present.
   All surgical patients need to be followed to
    ensure wound healing, evaluation for infection,
    suture removal and other routine eye care, such
    as testing for glaucoma, cataracts and retinal
    disease.
   Graft rejection can occur after penetrating
    keratoplasty, requiring prompt diagnosis and
    treatment to ensure graft survival.
                                                                      10
                              Dr. Mohd Najmussadiq Khan   11/7/2012    2
Complications
 Infection
 poor wound healing
 cornea transplant rejection
 corneal neovascularization
 Glare
 irregular astigmatism
 high refractive error




                                                                 10
                         Dr. Mohd Najmussadiq Khan   11/7/2012    3
Prognosis
 The prognosis for penetrating keratoplasty in a
  keratoconic patient is excellent, with most patients
  able to return to an active lifestyle and the pursuit
  of personal goals.
 Intrastromal corneal ring segments (such as Intacs)
  can provide long-term success for patients with
  keratoconus, but this is typically in conjunction with
  contact lens use, and some may ultimately require
  corneal transplant to reach their goals of visual
  rehabilitation. "Progression" of keratoconus, even
  after corneal surgery, has been reported, but it is
  not clear how common or to what extent this can
  occur
                                                                      10
                              Dr. Mohd Najmussadiq Khan   11/7/2012    4
   The donor stromal lenticule was created
    using the femtosecond laser (Intralase).
   The diameter of the lamellar cut was 9 mm.
   The laser parameters were set to cut the first
    lamellar interface at a depth of 400 μm
    posterior to the epithelial surface.
   A side cut was then performed creating a
    tapered edge to the lenticule.
   Then, a second superficial lamellar cut was
    made at a depth of 150 μm posterior to the
    epithelial surface.
   This produced a 250-μm donor corneal
    lamellar disc.                                                 10
                           Dr. Mohd Najmussadiq Khan   11/7/2012    5
   Next, a stromal pocket was formed in the host
    cornea at a depth of 250 μm using the Intralase.
   The diameter of the pocket was 9.5 mm, with a
    3.0-mm exit incision at the 12 o’clock position
   The patient was then taken to the operating
    room where prepared with povidone-iodine 10%
    solution
   The donor lamellar lenticule was folded and
    inserted into the host stromal pocket through the
    anterior incision.
   The donor lenticule was centered and smoothed
    with a cyclodialysis spatula.
   The wound was closed with a single 10-0 nylon
    suture.
                                                                     10
                             Dr. Mohd Najmussadiq Khan   11/7/2012    6

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Keratoconus and management

  • 2. Disease  Keratoconus is a degenerative non-inflammatory disease of the cornea where the central or paracentral cornea undergoes progressive thinning and steepening causing irregular astigmatism. Etiology  Etiology is unknown.  However, it is associated with atopy, Down’s Syndrome, Leber’s congenital amaurosis, and Ehler’s Danlos/connective disorders.  The hereditary pattern is neither prominent nor predictable, but positive family histories have been reported.  The most common presentation of keratoconus is as a sporadic disorder, in which only a significant minority of patients exhibit a family history with autosomal dominant or recessive transmission  The incidence of keratoconus if often reported to be 1 in 2000 people. Dr. Mohd Najmussadiq Khan 11/7/2012 2
  • 3. Risk Factors  Eye rubbing, associated with atopy  Sleep apnea  Floppy Lid Syndrome Dr. Mohd Najmussadiq Khan 11/7/2012 3
  • 4. Based on severity of curvature ◦ Mild : less than 45.00D ◦ Moderate : 45.00 to 52.00D ◦ Advanced : 52.00 to 62.00D ◦ Severe : more than 62.00D  Based on shape: ◦ Nipple cones (Small size 5mm ) ◦ Oval cones (larger (5-6mm) ellipsoid) ◦ Globus cones (Largest >6mm ,may involve over 75% of cornea. ) Dr. Mohd Najmussadiq Khan 11/7/2012 4
  • 5. Retinitis Pigmentosa  Leber’s Congenital Amaurosis  Microcornea  Aniridia  Corneal Degeneration  Congenital Cataract  Ectopia lentis  Lenticonus  Macular coloboma  Retinal dysplasia  Floppy eyelid syndrome Dr. Mohd Najmussadiq Khan 11/7/2012 5
  • 6. Dr. Mohd Najmussadiq Khan 11/7/2012 6
  • 7. Down’s syndrome  Ehlers-Danlos syndrome  Osteogenesis Imperfecta  Mitral valve prolapsed Dr. Mohd Najmussadiq Khan 11/7/2012 7
  • 8. General Pathology --Keratoconus can show the following pathologic findings  Deposition of iron in the basal epithelial cells, forming the Fleischer ring—a pigmented ring that results from the accumulation of ferritin particles in the cytoplasm of epithelial cells and widened intercellular spaces.  Breaks in Bowman's membrane, filled with cells, collagen, and PAS-positive material. Dr. Mohd Najmussadiq Khan 11/7/2012 8
  • 9. Dr. Mohd Najmussadiq Khan 11/7/2012 9
  • 10. thinning of stroma and overlying epithelium  abnormal keratocyte morphology, and endothelial polymorphism  folds or breaks in Descemet’s membrane (Vogts striae)  variable amounts of diffuse corneal scarring. Dr. Mohd Najmussadiq Khan 11/7/2012 10
  • 11. Dr. Mohd Najmussadiq Khan 11/7/2012 11
  • 12. Dr. Mohd Najmussadiq Khan 11/7/2012 12
  • 13. Pathophysiology  Histopathology studies demonstrate breaks in or complete absence of Bowman’s layer, collagen disorganization, scarring and thinning.  The etiology of these changes is not known, though some suspect changes in enzymes that lead to breakdown of collagen in the cornea.  While a genetic predisposition to keratoconus is suggested, a specific gene has not been identified. Dr. Mohd Najmussadiq Khan 11/7/2012 13
  • 14. Biochemical abnormalities:--  Studies in patients with keratoconus have demonstrated an increased activity by proteases that breakdown the collagen cross- linkages in the corneal stroma.  There is a simultaneous reduced expression of protease inhibitors.  The ratio of keratin sulfate to dermatan sulfate is altered in the stromal matrix when compared to normal corneas.  Patients with keratoconus have also shown enzymatic changes in the epithelium with increased expression of lysosomal and proteolytic enzymes. Dr. Mohd Najmussadiq Khan 11/7/2012 14
  • 15. Primary prevention  No preventive strategy has been proven effective to date.  Some feel that eye rubbing or pressure (e.g. sleeping with the hand against the eye) can cause and/or lead to progression of keratoconus, so patients should be informed not to rub the eyes.  In some patients, avoidance of allergens may help decrease eye irritation and therefore decrease eye rubbing. Dr. Mohd Najmussadiq Khan 11/7/2012 15
  • 16. Diagnosis  Diagnosis can be made by slit-lamp examination and observation of central or inferior corneal thinning.  Computerized videokeratography is also useful in detecting early keratoconus and allows following its progression.  Ultrasound pachymetry can also be used to measure the thinnest zone on the cornea.  New algorithms using computerized videokeratopgraphy have been devised which now allow the detection of subclinical or suspected keratoconus.  These devices may allow better screening of patients for prospective refractive surgery. Dr. Mohd Najmussadiq Khan 11/7/2012 16
  • 17. Dr. Mohd Najmussadiq Khan 11/7/2012 17
  • 18. Dr. Mohd Najmussadiq Khan 11/7/2012 18
  • 19. History  The majority of cases of keratoconus are bilateral, and often asymmetric.  The less affected eye may show a high amount of astigmatism or mild steepening.  Onset is typically in early adolescence and progresses into the mid-20’s and 30’s. However, cases may begin much earlier or later in life.  There is variable progression for each individual.  There is often a history of frequent changes in eye glasses which do not adequately correct vision.  Another common progression is from soft contact lenses, to Toric or astigmatism correcting contact lens, to rigid gas permeable contact lenes. Dr. Mohd Najmussadiq Khan 11/7/2012 19
  • 20. Dr. Mohd Najmussadiq Khan 11/7/2012 20
  • 21. Physical examination  A thorough and complete eye exam should be performed on any patient suspected of having keratoconus.  The general health of the eye should be assessed and appropriate ancillary tests should be done to assess corneal curvature, astigmatism and thickness.  The best potential vision should also be evaluated. Many of the potential exam components are listed below:  Proper history, including change in eye glass prescription, decreased vision, history of eye rubbing, medical problems, allergies, sleep patterns Dr. Mohd Najmussadiq Khan 11/7/2012 21
  • 22. Assessment of relevant aspects of the patient’s mental and physical status.  Measurement of best corrected visual acuity with spectacles and hard or gas permeaable contact lenses (with refraction when indicated)  Measurement of pinhole visual acuity  External examination (lids, lashes, lacrimal apparatus, orbit)  Examination of ocular alignment and motility  Assessment of pupillary function  Measurement of intraocular pressure (IOP)  Slit-lamp biomicroscopy of the anterior segment  Dilated examination of the lens, macula, peripheral retina, optic nerve, and vitreous  Keratomety/Computerized Topography//Ultrasound Pachymetry Dr. Mohd Najmussadiq Khan 11/7/2012 22
  • 23. Symptoms  Progressively poor vision not easily corrected with eye glasses. Clinical diagnosis  Diagnosis is made based on history of changing refraction, poor best spectacle corrected vision, abnormalities in keratometry, corneal topography and corneal thinning. Dr. Mohd Najmussadiq Khan 11/7/2012 23
  • 24. Dr. Mohd Najmussadiq Khan 11/7/2012 24
  • 25. Asymmetric refractive error with high or progressive astigmatism  Keratometry showing high astigmatism and irregularity (axis that do not add to 180 degrees)  Scissoring of the red reflex on ophthalmoscopy or retinoscopy.  Inferior steepening, skewed axis, or elevated keratometry values on K reading and computerized corneal topography Dr. Mohd Najmussadiq Khan 11/7/2012 25
  • 26. Corneal thinning, especially in inferior cornea. Maximum corneal thinning corresponds to the site of maximum steepening or prominence.  Rizutti’s sign or a conical reflection on nasal cornea when a penlight is shone from the temporal side  Fleischer ring, an iron deposit often present within the epithelium around the base of the cone. It is brown in color and best visualized with a cobalt blue filter  Vogt’s striae, fine, roughly vertically parallel striations in the stroma. These generally disappear with firm pressure applied over the eyeball and re-appear when pressure is discontinued. Dr. Mohd Najmussadiq Khan 11/7/2012 26
  • 27. Munson’s sign, a protrusion of the lower eyelid in down gaze.  Superficial scarring • Break’s in Bowman’s membrane  Acute hydrop’s, a condition where a break in Descemet’s membrane allows aqueous into the stoma causing severe corneal thickening, decreased vision and pain.  Stromal scarring after resolution of acute hydrops, which paradoxically may improve vision in some cases by changing corneal curvature and reducing the irregular astigmatism. Dr. Mohd Najmussadiq Khan 11/7/2012 27
  • 28. Dr. Mohd Najmussadiq Khan 11/7/2012 28
  • 29. Dr. Mohd Najmussadiq Khan 11/7/2012 29
  • 30. Differentiation is made between a “quiet” type of keratoconus (“forme fruste”) and progressive keratoconus.  “Forme fruste” occurs 10 times more often than progressive keratoconus.  Normally it produces no symptoms and has only to be observed.  If the condition remains stable, there is no need for treatment. Dr. Mohd Najmussadiq Khan 11/7/2012 30
  • 31. Progressive keratoconus is aggressive and can begin at a very early age.  With progression of the disease, correction of visual acuity with glasses becomes more difficult because protrusion of the cornea develops unevenly.  Hard contact lenses are a good solution because they put pressure on the cornea, thus correcting irregularities. Dr. Mohd Najmussadiq Khan 11/7/2012 31
  • 32. Diagnostic procedures  • Ophthalmic history, including family eye history, heritable disease, history of allergies, etc  • Slit-lamp examination  • Hard or gas permeable contact lens trial because good vision with lenses eliminates other sources of poor vision, including amploypia  • Measurement of K values  • Ultrasound Pachymetry  • Computerized corneal topography Dr. Mohd Najmussadiq Khan 11/7/2012 32
  • 33. Dr. Mohd Najmussadiq Khan 11/7/2012 33
  • 34. Dr. Mohd Najmussadiq Khan 11/7/2012 34
  • 35. Dr. Mohd Najmussadiq Khan 11/7/2012 35
  • 36. Dr. Mohd Najmussadiq Khan 11/7/2012 36
  • 37. Dr. Mohd Najmussadiq Khan 11/7/2012 37
  • 38. Dr. Mohd Najmussadiq Khan 11/7/2012 38
  • 39. Laboratory test--Assessment of heritable diseases including  • Down’s Syndrome  • Leber’s congenital amaurosis  • Ehlers Danlos/connective disorder.  • Other medical conditions including sleep apnea Differential diagnosis  • Keratoconus  . Pellucid marginal degeneration  • Keratoglobus  • Contact lens induced corneal warpage  • Coneal Ectasia post excimer laser treatment Dr. Mohd Najmussadiq Khan 11/7/2012 39
  • 40. In 1963, scientists found that the initial changes of KC were related to degradation (break down) of the cornea s basement membrane.  They found that over time, a cascade of events occurred, leading to alterations in the layers of the cornea and resulting in the thinning of the stromal layer.  This weakened the structural integrity of the cornea and in turn resulted in a bulging, cone- like distortion of the normally spherical cornea. Dr. Mohd Najmussadiq Khan 11/7/2012 40
  • 41. Dr. Mohd Najmussadiq Khan 11/7/2012 41
  • 42. Investigators found that the enzyme activities of the KC corneas were increased compared to normal corneas.  In addition, there was a significant reduction of inhibitors for these enzymes.  The cornea is responsible for approximately 80% of the absorption of ultraviolet B (UVB) light that enters the eye.  UV light generates free oxygen radicals, high energy molecules, which if unchecked, can damage tissue. Dr. Mohd Najmussadiq Khan 11/7/2012 42
  • 43. Typically, the free radicals are removed from the cornea via antioxidants (such as superoxide dismutase, catalase and glutathione reductase). Those that are not removed undergo reactions that form aldehydes, which can be destructive to the tissue Dr. Mohd Najmussadiq Khan 11/7/2012 43
  • 44. Normally, the cornea protects itself from these aldehydes with an enzyme called aldehyde dehydrogenase (ALDH) which detoxifies these aldehydes.  ALDH is a major protein within the cornea. (Approximately 78% of the ALDH within the eye is found in the cornea.).  In the KC cornea there is decreased activity of ALDH enzyme. Dr. Mohd Najmussadiq Khan 11/7/2012 44
  • 45. These peroxynitrites are also harmful to tissues and can be identified by using antibodies to nitrotyrosine. KC corneas have an increased amount of nitroytrosine staining compared to normal corneas This increased staining in KC corneas suggests that they have additional destructive substances in the tissue. Dr. Mohd Najmussadiq Khan 11/7/2012 45
  • 46. Normally, the human cornea has very little apoptosis occurring.  Dr. Wilson demonstrated that KC corneas have apoptosis occurring in the anterior stroma and epithelium, especially in the areas of breaks in Bowman's layer. Dr. Mohd Najmussadiq Khan 11/7/2012 46
  • 47. KC corneas produce a unique enzyme that is not found in normal corneas or corneas with other diseases.  This enzyme is called leukocyte common antigen related protein (LAR).  LAR in KC corneas also supports the notion of apoptosis in KC corneas because other investigators have shown in other systems that LAR expression plays a role in apoptosis. Dr. Mohd Najmussadiq Khan 11/7/2012 47
  • 48. The goal of future research studies is to understand the biochemical and molecular changes and possible genetic influences that occur in KC corneas.  Intervention at one of these steps might be the key factor needed to block the progression of keratoconus. Dr. Mohd Najmussadiq Khan 11/7/2012 48
  • 49. General treatment --The goal of treatment is primarily to provide functional visual acutiy. More recently attention has been directed to halting changes in the corrneal shape.  Initially, spcetacles or soft toric contact lenses in mild cases can be used. Dr. Mohd Najmussadiq Khan 11/7/2012 49
  • 50. Dr. Mohd Najmussadiq Khan 11/7/2012 50
  • 51. Dr. Mohd Najmussadiq Khan 11/7/2012 51
  • 52. Patients with very mild disease may initially be corrected with glasses or soft contact lenses.  However the vast majority of patients need rigid contact lenses for adequate vision correction.  The very latest contact lens for treating keratoconus is the “synergize hybrid contact lens” which is rigid in the middle and soft on the edges. Dr. Mohd Najmussadiq Khan 11/7/2012 52
  • 53. Needed in the majority of cases to neutralize the irregular corneal astigmatism.  It resurfaces the irregular cornea & the intervening fluid and corrects the irregular astigmatism to provide good quality of vision.  However the disadvantage with these lenses is that they have standard lens design with fixed optical zones and do not give an ideal fit in patients with keratoconus  The majority of patients that can wear hard or gas-permeable contact lenses have a dramatic improvement in their vision. Dr. Mohd Najmussadiq Khan 11/7/2012 53
  • 54. Dr. Mohd Najmussadiq Khan 11/7/2012 54
  • 55. Many patients find their contact lenses uncomfortable and can only tolerate their contact lenses for a short period of time.  The reason is that the cornea steepens and rubs against the lens causing an abrasion and light sensitivity .
  Another reason is patients with keratoconus often have very dry eye and as the eye dries out there is no lubricating barrier between the lens and the cornea contributing to the patient being uncomfortable. Dr. Mohd Najmussadiq Khan 11/7/2012 55
  • 56. RoseK-- The Rose-k lenses are made with complex geometry to fit any type of cone. The optical zone is reduced to snugly fit the cone & the peripheral curve is computer designed to fit the rest of the irregular cornea. Dr. Mohd Najmussadiq Khan 11/7/2012 56
  • 57. piggy back lens-- they are known as piggy back because a RGP Lens is fitted on top of a soft contact lens. These lenses provide excellent comfort and good vision. Dr. Mohd Najmussadiq Khan 11/7/2012 57
  • 58. Scleral Contact Lenses: They are made of a special polymer called Itaflurocon. They characteristically vault over the cornea and limbus and are supported entirely by the sclera. The fluid lens smoothens the irregular cornea and provides good vision. It also prevents desiccation due to its very high oxygen permeability. The small pores on the lens provide adequate exchange of tears. The disadvantage with these lenses is that they are difficult to use. They are large lenses which are cumbersome to the patients. Dr. Mohd Najmussadiq Khan 11/7/2012 58
  • 59. Boston Scleral Contact lenses (BSLP): BSLP lenses are made of Flurosilicone Acrylate Polmers with DK values of 87 and 130. Its diameter ranges from15.5mm to 20mm. The space created over the cornea is filled with non-preserved, buffered sterile saline. The limitation to the use of these lenses is that the fitting process is skill intensive and time consuming. Dr. Mohd Najmussadiq Khan 11/7/2012 59
  • 60. Those patients that become contact lens intolerant or do not have acceptable vision, typically from central scaring, can proceed to surgical alternatives Dr. Mohd Najmussadiq Khan 11/7/2012 60
  • 61. Medical therapy—for acute corneal hydrops involves  a cycloplegic agent  sodium chloride 5% ointment or eye drop  Lubricating eye drop  Steroid drops can be used if the eye is congested  a pressure patch  After the pressure patch is removed patients may still need to continue sodium chloride drops or ointment for several weeks to months.  Patients are advised to avoid vigorous eye rubbing Dr. Mohd Najmussadiq Khan 11/7/2012 61
  • 62. Dr. Mohd Najmussadiq Khan 11/7/2012 62
  • 63. It takes 3 - 4 months for the corneal edema to resolve, following which a standard full thickness keratoplasty is required to restore corneal clarity and visual improvement.  It this situation lamellar surgery is not recommended  Thermokeratoplasty has been tried and believed to shrinkage of the collagen with resultant flattening of the hydrops which helps in early healing of the ruptured DM. Dr. Mohd Najmussadiq Khan 11/7/2012 63
  • 64. Patients are usually followed on a 6-month to yearly basis to monitor the progression of the corneal thinning, steepening, the resultant visual changes, and to re-evaluate contact lens fit and care.  Patients with hydrops are seen more frequently until it resolves. Dr. Mohd Najmussadiq Khan 11/7/2012 64
  • 65. When patients become intolerant or no longer benefit from contact lenses, surgery is the next option. Surgical options can include: INTACS, Anterior lamellar keratoplasty, penetrating keratoplasty.  Non- FDA approved treatments, which typically have less evidence based information available on safety and efficacy, include use of UV/riboflavin collagen cross-linking of the cornea to stiffen the cornea and possibly prevent progressive changes in shape. This treatment has also be experimentally combined with excimer laser treatment, conductive keratoplasty, and/or INTACS.  Some surgeons will use phakic IOLs to address high myopia and some of the asitigmatism. Dr. Mohd Najmussadiq Khan 11/7/2012 65
  • 66. Dr. Mohd Najmussadiq Khan 11/7/2012 66
  • 67. The most promising technology for treating Keratoconus called collagen crosslinking (CXL) with UVA is currently being introduced  It is undergoing Phase 1 FDA clinical trials in the United States.  It has been demonstrated to be safe and effective if performed, with the epithelium removed, and has the potential to stop the progression of Keratoconus. Dr. Mohd Najmussadiq Khan 11/7/2012 67
  • 68. This treatment is recommended for individuals with progressive Keratoconus or Ectasia following LASIK to stabilize the cornea.  It can be performed with our without INTACS. Dr. Mohd Najmussadiq Khan 11/7/2012 68
  • 69. CORNEAL COLLAGEN CROSS-LINKING (C3R/CXL):  The basic problem in keratoconus is the weakness of the cross links which act as anchors between the collagen fibrils.  UV-A irradiance of the cornea after sensitization with riboflavin, augments these cross-links, and hence recovers some of the corneal mechanical strength in keratoconus.  This treatment modality stabilizes the collagen scaffold and increases the stiffness of the cornea against the action of proteolytic enzymes.  Riboflavin/UVA light causes increase in the corneal stiffness of the anterior 200-250 μm.  Deeper structures like the lens and endothelium do not get damaged.  The progression of keratoconus or keratectasia can be stopped. Dr. Mohd Najmussadiq Khan 11/7/2012 69
  • 70. This results in cross linking of the collagen fibres of the cornea, thereby increasing its physical strength by up to 300%.  Thus further progression of keratoconus can be arrested, and in 15 -20% cases regression has also been noted.  Longest follow-up duration following this treatment is for 7 years. Dr. Mohd Najmussadiq Khan 11/7/2012 70
  • 71. Dr. Mohd Najmussadiq Khan 11/7/2012 71
  • 72. Dr. Mohd Najmussadiq Khan 11/7/2012 72
  • 73. The top layer of the cornea is removed under local anesthesia.  Vitamin drops are soaked into the cornea until they penetrate the entire corneal and evidence of penetration into the anterior chamber of the eye is demonstrated by slit-lamp evaluation.  Once this is confirmed the patient’s eye is put under a specialized lamp, which emits UV A light at a predetermined wavelength for approximately 30 minutes.  During this process the cross links, which link the fibers of the cornea, are increased thereby stiffening the whole cornea.  A bandage contact lens is then put on the eye and patients are given antibiotics and anti-inflammatory drops and follow up on a regular basis with their physicians for several months. Dr. Mohd Najmussadiq Khan 11/7/2012 73
  • 74. Dr. Mohd Najmussadiq Khan 11/7/2012 74
  • 75. Dr. Mohd Najmussadiq Khan 11/7/2012 75
  • 76. During the post-operative period flurometholone and artificial tears are applied for a period of one month.  In the pilot study done on 23 eyes and followed up to 4 years Wollensak et al found a mean reduction in steepening of 2.01 D.  Other investigators such as caporossi et al and Krumeich et al reported a similar result.  The improvement in vision after cross-linking is caused by a decrease in astigmatism and corneal curvature as well as topographical homogenization of the cornea as a result of the increased rigidity in the cross- linked cornea.  In addition tolerance to contact lenses improved.  Cross-linking is a practical outpatient treatment, which is minimally invasive and cost-effective. Dr. Mohd Najmussadiq Khan 11/7/2012 76
  • 77. Dr. Mohd Najmussadiq Khan 11/7/2012 77
  • 78. A method for flattening the cornea that is too steep and making a patient more contact lens tolerant is the insertion of INTACS into the cornea.  This procedure is good for patients who are contact lens intolerant and who want to avoid a corneal transplant and whose K readings are not in excess of 58 Diopters.  (INTACS ) have been approved for the treatment of mild to moderate keratoconus in patients who are contact lens intolerant. Dr. Mohd Najmussadiq Khan 11/7/2012 78
  • 79. In these cases, patients must have a clear central cornea and a corneal thickness of > 450 microns where the segments are inserted, approximately at 7 mm optical zone.  Intrastromal corneal ring segments (Intacs Addition Technology Inc) have crescent shaped two 150-degree PMMA segments that are surgically placed in the peripheral cornea at two- thirds corneal depth.  Intacs comes in thickness ranging from 0.25 to 0.45 mm in 0.05 mm increments.  Intacs correct the myopia and irregular astigmatism by producing a mechanical flattening of the central cone. Dr. Mohd Najmussadiq Khan 11/7/2012 79
  • 80. The advantage of INTACS is that they require no removal of corneal tissue, no intraocular incision, and leave the central cornea untouched.  Most patients will need spectacles and/or contact lenses post-operatively for best vision, but will have flatter corneas and easier use of lenses.  If a patient does not gain the expected results, the INTACS can be removed and then other surgical options can be considered Dr. Mohd Najmussadiq Khan 11/7/2012 80
  • 81. Dr. Mohd Najmussadiq Khan 11/7/2012 81
  • 82. Femtosecond laser technology allows realization with very high accuracy of individualized shape and size of corneal tunnel depending on the surgical need.  Intrastromal cuts from the inner to the outer parts of the cornea can be performed easily.  During surgery, stress to the cornea is minimal, because only moderate pressure is exerted on the eye.  The risk of infection is significantly reduced. Dr. Mohd Najmussadiq Khan 11/7/2012 82
  • 83. Kera rings are tiny plastic semicircular rings surgically implanted into the cornea to flatten the corneal surface and improve vision.  Inserts can improve contact lens wear in most patients.  They are not suitable for all patients with keratoconus.  Implantation does not affect the central optic zone, does not involve the removal of any tissue, and can be reversed if vision changes. Dr. Mohd Najmussadiq Khan 11/7/2012 83
  • 84. Imagine your cornea as a tent with a curved top. If you push out the sides of the tent, the top flattens. Similarly, when Kera rings are placed in the sides of the cornea, they flatten it. Dr. Mohd Najmussadiq Khan 11/7/2012 84
  • 85. Combined KERARINGS insertion and CXL can be performed safely in one or two sessions. However, the same-session procedure appears to be more effective regarding the improvement in the corneal shape. Br J Ophthalmol. 2011 Jan;95(1):37-41. Epub 2010 Jun 28. Dr. Mohd Najmussadiq Khan 11/7/2012 85
  • 86. Laser may now be used to treat keratoconus.  The technique is similar to the Lasek laser done for short sight etc, but uses a cornea topographer to guide the laser.  The laser reduces the keratoconic protrusion, significantly improving vision.  When controlled by a topographer only a few microns of cornea need be removed, greatly enhancing safety.  The technique is effective for mild-to-moderate keratoconus.  To stabilize the cornea after laser, collagen cross linking is done.  Only the most sophisticated lasers, such as the Alcon Wave light EX500 excimer laser can be used Dr. Mohd Najmussadiq Khan 11/7/2012 86
  • 87. Dr. Mohd Najmussadiq Khan 11/7/2012 87
  • 88. It is the newest procedure under investigation for the treatment of keratoconus.  It is non-incisional and reshapes the cornea without removing any tissue.  It is currently under clinical investigation in Europe for treating myopia and keratoconus.  It involves the delivery of a single low energy microwave pulse lasting less than one second to the cornea.  Energy is applied to the cornea using a dielectrically shielded microwave emitter which contacts the epithelial surface.  Through capacitive coupling, the single pulse raises the temperature of the selected region of corneal stroma to approximately 65°C, forming a doughnut-shaped area of collagen shrinkage in the upper 150 microns of the stroma. Dr. Mohd Najmussadiq Khan 11/7/2012 88
  • 89. The lesion created during Keraflex is intended to flatten the central cornea both to decrease the cone in keratoconus and to achieve myopic correction.  In order to improve the stability of the cornea with Keraflex, Keraflex KXL includes focal corneal collagen crosslinking.  After the actual Keraflex procedure, riboflavin drops are administered over the treatment area, a mask is applied to protect the central and peripheral areas of the cornea, and ultraviolet light is admistered to crosslink the collagen. Dr. Mohd Najmussadiq Khan 11/7/2012 89
  • 90. Corneal Transplants are the only option for patients who have scarring in the center of the cornea or who are contact lens intolerant because their corneas are too steep.  The results of corneal transplants are excellent in keratoconus patients with an over 97% success rate.  Patients can have LASIK or PRK on their transplants and become relatively independent of glasses or contact lenses.  Recently the Femtosecond Laser was approved for performing Corneal Transplants Dr. Mohd Najmussadiq Khan 11/7/2012 90
  • 91. It involves replacement of the central anterior cornea, leaving the patient’s endothelium intact.  The advantages are that the risk of endothelial graft rejection is eliminated, and there is less risk of traumatic rupture of the globe in the incision, since the endothelium and Descemet’s and some stroma are left intact, and faster visual rehabilitation. Dr. Mohd Najmussadiq Khan 11/7/2012 91
  • 92. There are several techniques utilized including, deep anterior lamellar keratoplasty (DALK) and big bubble keratoplasty(BBK) to remove the anterior stroma, while leaving Descemet’s layer and endothelium untouched.  However, the procedures can be technically challenging requiring conversion to a penetrating keratoplasty  Post-operatively there is the possibility of interface haze leading to a decrease in BCVA  It is not clear if astigmatism is better treated with anterior vs penetrating keratoplasty. Dr. Mohd Najmussadiq Khan 11/7/2012 92
  • 93. Dr. Mohd Najmussadiq Khan 11/7/2012 93
  • 94. Dr. Mohd Najmussadiq Khan 11/7/2012 94
  • 95. In DALK the bottom layer of the cornea is spared this results in less chance of rejection.  It should be remembered however that in some instances the separation of the bottom layers of the cornea and the upper parts might be uneven which would result in poorer visual outcomes. Dr. Mohd Najmussadiq Khan 11/7/2012 95
  • 96. Dr. Mohd Najmussadiq Khan 11/7/2012 96
  • 97. Descemets exposed DALK(deepanteriorlamellarkeratoplasty Dr. Mohd Najmussadiq Khan 11/7/2012 97
  • 98. It has a high success rate and is the standard surgical treatment with a long track record of safety and efficacy.  Risks of this procedure include infection and cornea rejection and risk of traumatic rupture at wound margin.  Many patients after PK may still need hard or gas- permeable contact lenses due to residual irregular astigmatism  Any type of refractive procedure is considered a contraindication in keratoconic patients due to the unpredictability of the outcome and risk of leading to increased and unstable irregular astigmatism. Dr. Mohd Najmussadiq Khan 11/7/2012 98
  • 99. Dr. Mohd Najmussadiq Khan 11/7/2012 99
  • 100. 10 Dr. Mohd Najmussadiq Khan 11/7/2012 0
  • 101. 10 Dr. Mohd Najmussadiq Khan 11/7/2012 1
  • 102. Following any corneal surgical procedure, patients need to be followed to complete visual rehabilitation.  Most patients still require vision correction with spectacles or contact lenses, and often hard or gas permeable lenses are required if high levels of astigmatism are present.  All surgical patients need to be followed to ensure wound healing, evaluation for infection, suture removal and other routine eye care, such as testing for glaucoma, cataracts and retinal disease.  Graft rejection can occur after penetrating keratoplasty, requiring prompt diagnosis and treatment to ensure graft survival. 10 Dr. Mohd Najmussadiq Khan 11/7/2012 2
  • 103. Complications  Infection  poor wound healing  cornea transplant rejection  corneal neovascularization  Glare  irregular astigmatism  high refractive error 10 Dr. Mohd Najmussadiq Khan 11/7/2012 3
  • 104. Prognosis  The prognosis for penetrating keratoplasty in a keratoconic patient is excellent, with most patients able to return to an active lifestyle and the pursuit of personal goals.  Intrastromal corneal ring segments (such as Intacs) can provide long-term success for patients with keratoconus, but this is typically in conjunction with contact lens use, and some may ultimately require corneal transplant to reach their goals of visual rehabilitation. "Progression" of keratoconus, even after corneal surgery, has been reported, but it is not clear how common or to what extent this can occur 10 Dr. Mohd Najmussadiq Khan 11/7/2012 4
  • 105. The donor stromal lenticule was created using the femtosecond laser (Intralase).  The diameter of the lamellar cut was 9 mm.  The laser parameters were set to cut the first lamellar interface at a depth of 400 μm posterior to the epithelial surface.  A side cut was then performed creating a tapered edge to the lenticule.  Then, a second superficial lamellar cut was made at a depth of 150 μm posterior to the epithelial surface.  This produced a 250-μm donor corneal lamellar disc. 10 Dr. Mohd Najmussadiq Khan 11/7/2012 5
  • 106. Next, a stromal pocket was formed in the host cornea at a depth of 250 μm using the Intralase.  The diameter of the pocket was 9.5 mm, with a 3.0-mm exit incision at the 12 o’clock position  The patient was then taken to the operating room where prepared with povidone-iodine 10% solution  The donor lamellar lenticule was folded and inserted into the host stromal pocket through the anterior incision.  The donor lenticule was centered and smoothed with a cyclodialysis spatula.  The wound was closed with a single 10-0 nylon suture. 10 Dr. Mohd Najmussadiq Khan 11/7/2012 6