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FocalPoints

Clinical Modules for Ophthalmologists


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Advances in the
Surgical Treatment of
Keratoconus
Aylin Kılıç, MD
Joseph Colin, MD

Reviewers and Contributing Editor Consultants

George A. Stern, MD, Editor for Cornea and External Disease James J. Reidy, MD, FACS
Elmer Y. Tu, MD, Basic and Clinical Science Course Faculty, Section 8 David J. Schanzlin, MD
Edward K. Isbey III, MD, Practicing Ophthalmologists Advisory Committee for Education

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Claiming CME Credit Focal Points Editorial Review Board
The American Medical Association requires that all learners George A. Stern, MD, Missoula, MT
participating in activities involving enduring medical materi- Editor in Chief; Cornea and External Disease
als complete a formal assessment before claiming continuing
D. Michael Colvard, MD, FACS, Encino, CA
medical education (CME) credit. To assess your achievement in
this activity and ensure that a specified level of knowledge has Cataract Surgery
been reached, a post test for this module is provided. A mini- Bradley S. Foster, MD, Springfield, MA
mum score of 80% must be obtained to pass the test and claim Retina and Vitreous
CME credit.
Syndee J. Givre, MD, PhD, Raleigh, NC
To claim Focal Points CME credits, visit the Academy
Neuro-Ophthalmology
web site and access CME Central (www.aao.org/CME) to
report CME credit you have earned. You can claim up to Ramana S. Moorthy, MD, FACS, Indianapolis, IN
2 AMA PRA Category 1 Credits™ per module. CME credit may Ocular Inflammation and Tumors
be claimed for up to three (3) years from date of issue.
Eric P. Purdy, MD, Fort Wayne, IN
Oculoplastic, Lacrimal, and Orbital Surgery

Focal Points (ISSN 0891-­8260) is published quarterly by the American Acad- Steven I. Rosenfeld, MD, FACS, Delray Beach, FL
emy of Ophthalmology at 655 Beach St., San Francisco, CA 94109-­1336. For Refractive Surgery; Optics and Refraction
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Points, P.O. Box 7424, San Francisco, CA 94120-­7424.
The American Academy of Ophthalmology is accredited by the Accredita- Focal Points Staff
tion Council for Continuing Medical Education to provide continuing medical
education for physicians. Susan R. Keller, Acquisitions Editor
The American Academy of Ophthalmology designates this enduring mate-
Kim Torgerson, Publications Editor
rial for a maximum of 2 AMA PRA Category 1 Credits™. Physicians should claim
only the credit commensurate with the extent of their participation in the activity.
The Academy provides this material for educational purposes only. It is not
intended to represent the only or best method or procedure in every case, nor
Clinical Education Secretaries and Staff
to replace a physician’s own judgment or give specific advice for case manage-
Gregory L. Skuta, MD, Senior Secretary for Clinical Education,
ment. Including all indications, contraindications, side effects, and alternative
agents for each drug or treatment is beyond the scope of this material. All Oklahoma City, OK
information and recommendations should be verified, prior to use, with current Louis B. Cantor, MD, Secretary for Ophthalmic Knowledge,
information included in the manufacturers’ package inserts or other indepen-
Indianapolis, IN
dent sources and considered in light of the patient’s condition and history.
Reference to certain drugs, instruments, and other products in this publica- Richard A. Zorab, Vice President, Ophthalmic Knowledge
tion is made for illustrative purposes only and is not intended to constitute
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tions that are not considered community standard, that reflect indications not
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and all claims that may arise out of the use of any recommendations or other
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©2012 American Academy of Ophthalmology®. All rights reserved.

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ii Focal Points : Module 2, 2012

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Learning Objectives
At the completion of this module,
Contents
the reader should be able to:
Introduction 1
• Discuss the indications, surgical techniques, results,
and complications of the use of intracorneal ring Intracorneal Ring Segments 3
segments in the treatment of keratoconus • Surgical Technique 5
• Discuss the indications, techniques, results, and • Results 6
complications of collagen crosslinking, with and • Complications 6
without photorefractive keratectomy, in the
treatment of keratoconus
Collagen Crosslinking 6
• Surgical Technique 7
• Describe the use of long-­acting intracameral gases
• Results 8
to treat corneal hydrops
• Complications 8
• Compare the relative merits of deep anterior
lamellar keratoplasty and penetrating keratoplasty Combined Collagen Crosslinking
in the treatment of keratoconus and Intrastromal Rings 8

Combined Collagen Crosslinking


Financial Disclosures and Photorefractive Keratectomy 9
The authors, reviewers, and consultants disclose the follow-
ing financial relationships. Joseph Colin, MD: (C) Abbott Medical
Treatment of Corneal Hydrops 9
Optics, Addition Technology, Alcon Laboratories. D. Michael
Lamellar Keratoplasty 9
Colvard, MD, FACS: (C) Abbott Medical Optics, Bausch & Lomb;
(P) OASIS Medical. Steven I. Rosenfeld, MD, FACS: (C) Inspire Conclusion 10
Pharmaceuticals; (L) Allergan. David J. Schanzlin, MD: (C) Aaren
Scientific, OASIS Medical. C. Gail Summers, MD: (L) BioMarin Clinicians’ Corner 12
Pharmaceutical, (S) NOAH (The National Organization for Albinism
and Hypopigmentation). Elmer Y. Tu, MD: (L) Alcon Laboratories.

The following contributors state that they have no significant financial


interest or other relationship with the manufacturer of any commer- Introduction
cial product discussed in their contributions to this module or with
Keratoconus is a progressive ectatic disorder of the cor-
the manufacturer of any competing commercial product: Bradley S.
Foster, MD; Syndee J. Givre, MD; Daniel Mummert; Edward K.
nea that generally affects younger individuals. As the
Isbey III, MD; Susan R. Keller; Aylin Kılıç, MD; Ramana S. Moorthy, disease progresses, the central cornea thins and the
MD; Eric P. Purdy, MD; James J. Reidy, MD, FACS; George A. Stern, cornea assumes a conical, rather than spherical, cross-­
MD; Kim Torgerson. sectional shape. Physical findings include a steep cor-
neal curvature, apical thinning, and striae at the level of
C = Consultant fee, paid advisory boards or fees for attending a Descemet’s membrane (Figure 1, Figure 2). A V‑shaped
meeting curvature of the lower lid margin on downgaze is termed
L = Lecture fees (honoraria), travel fees or reimbursements when Munson’s sign (Figure 3). The apex of the cone is usually
speaking at the invitation of a commercial sponsor decentered inferiorly (Figure  4). This leads to progres-
P = Patents and/or royalties that might be viewed as creating a
sive myopia and astigmatism, usually irregular, leading
potential conflict of interest
to a loss of best spectacle-­corrected visual acuity (BSCVA).
S = Grant support for the past year (all sources) and all sources used
Later in the disease, scarring at the level of Bowman’s
for this project if this form is an update for a specific talk or manu-
script with no time limitation
membrane often occurs. Tears in Descement’s mem-
brane cause edema (hydrops), with subsequent scarring,
that further decreases visual acuity (Figure 5). Corneal
topographic analysis, using either a Placido-­based or
Scheimpf lug camera system, is often helpful in making
the diagnosis and following progression of the disease

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Figure 3 ​A V‑shaped curvature of the lower lid margin on
Figure 1 ​Slit-lamp photograph of a patient with keratoconus,
downgaze is termed Munson’s sign. (Reprinted, with permis-
demonstrating increased corneal curvature, apical thinning,
sion from Colin J, Ertan (Kılıç) A, Intracorneal Ring Segments
and striae at the level of Descemet’s membrane. (Reprinted,
and Alternative Treatments for Corneal Ectatic Diseases.
with permission from Colin J, Ertan (Kılıç) A, Intracorneal
Ankara: Kudret Eye Hospital, 2007:14.)
Ring Segments and Alternative Treatments for Corneal Ec-
tatic Diseases. Ankara: Kudret Eye Hospital, 2007:13.)

Figure 4 ​Oblique slit-beam view of a patient with keratoco-


nus shows inferiorly decentered protrusion of the cornea.

Figure 2 ​Optical distortion created by the cone is easily vis-


ible in the red reflex.

(Figure 6). Keratoconus diagnostic software is available


with most topographic systems and generally utilizes a
significant difference between superior and inferior cur-
vatures to suggest the diagnosis.
Treatment of early keratoconus includes the use of
glasses or soft contact lenses to improve visual acuity.
When irregular astigmatism precludes adequate cor-
rection with glasses or soft lenses, rigid gas-­permeable
Figure 5 ​Slit-lamp photograph of a patient with hydrops, a
contact lenses, which mask the surface irregularities,
central opacity due to edema created by a tear in Des-
often restore vision. Some patients, however, cannot cemet’s membrane. (Reprinted, with permission from
be comfortably fit with a rigid lens, and as the disease Colin J, Ertan (Kılıç) A, Intracorneal Ring Segments and
progresses, the cornea may become too steep to main- Alternative Treatments for Corneal Ectatic Diseases. Ankara:
tain a contact lens on the eye. In some cases, this can be Kudret Eye Hospital, 2007:14.)

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rejections, however, are common, and threaten graft sur-
vival. Recent advances in lamellar surgery techniques
that enable near-­equivalent visual outcomes to PKP have
made deep anterior lamellar keratoplasty (DALK) a viable
option to PKP, sparing the risk of endothelial graft rejec-
tion. In this module, these new surgical techniques for
the treatment of keratoconus are discussed.

Intracorneal Ring
Segments
Intracorneal rings affect the keratoconic cornea in two
ways. First, the ring effectively adds tissue and elevates
the anterior surface of the cornea overlying the ring. Since
the central cornea f lattens in the direction of an elevated
area, the 360° placement of the rings causes a generalized
f lattening of the central cornea, reducing the amount
of myopia. The f lattening effect of the ring segments is
Figure 6 ​Typical topographic picture of keratoconus,
proportional to the thickness of the ring and inversely
demonstrating greater than normal central curvature, with
steeper curvature inferiorly. proportional to the diameter of the ring segments. Asym-
metric placement of rings of different thicknesses can
reduce astigmatism and centralize an eccentric cone.
managed with the use of newer, ultra-large scleral, rigid Secondly, in keratoconus, the corneal elastic modulus
gas-­permeable contact lenses, but cost and need to travel is reduced due to pathology in the corneal stroma. This
to be fit with these lenses can be prohibitive. In addition, causes the cornea to be more deformable in response
central corneal scarring can limit vision despite the use to stress, leading to thinning and protrusion (increased
of any optical device. Traditionally, when patients could curvature), which are the hallmarks of keratoconus
not get adequate vision with either glasses or contact (Figure 7). By shortening the path length of the collagen
lenses, the only surgical option has been penetrating ker- lamellae central to the segments, there is a redistribu-
atoplasty (PKP). tion of corneal stress, interrupting the biomechanical
Recent advances have added to the surgical options cycle of keratoconus disease progression (Figure 8).
for keratoconus. They also can be applied to two closely The goal of ring segment surgery is to reduce the
related diseases, pellucid marginal degeneration and degree of myopia and astigmatism, improving UCVA and
post-­L ASIK ectasia. Intracorneal ring segments can BCVA. Shifting the position of the cone more centrally
improve uncorrected (UCVA) and best-­corrected visual within the cornea may also improve higher-­order aberra-
acuity (BCVA), or allow for successful contact lens fit- tions, such as coma. Unlike purely refractive procedures,
ting in a previously unfittable patient, avoiding the need however, the goal is not emmetropia; rather, a success-
for keratoplasty. A new technique called collagen crosslink- ful procedure is one that improves vision enough that
ing (CXL) can increase the biomechanical rigidity of the the patient can be comfortably corrected to an adequate
cornea, f latten the cornea, and prevent progression of level of vision with spectacles or contact lenses, avoid-
the disease. While laser remodeling of the cornea would ing riskier procedures such as keratoplasty. This goal is
threaten to worsen the disease in an untreated cornea, achieved approximately 70% of the time.
it might be successfully and safely performed in a cross- There are a variety of intracorneal ring segments
linked cornea. used worldwide, including the Bisantis Intrastromal
Corneal hydrops, traditionally treated medically, can Segmented Perioptic Implant (Opticon 2000 SpA and
cause a prolonged period of disability while waiting for Soleko SpA, Rome, Italy), the Keraring Intrastromal
the edema to resolve. This period can be shortened by Corneal Ring Segment (Mediphakos, Inc, Belo Hori-
the intracameral injection of a long-­acting gas bubble. zonte, Brazil), the Ferrara Ring Segment (Mediphakos),
PKP has generally been a very successful technique for and the ­MyoRing intracorneal continuous ring (Dioptex
restoring vision in patients with advanced disease. Graft GmbH, Linz, Austria). The Intacs Ring Segment (Addition

Focal Points : Module 2, 2012 3

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Technology Inc, Sunnyvale, California) is the most widely
used corneal ring segment and the only one used in the
Uniform thickness =
Uniform stress United States. In this module, discussion is limited to
the Intacs segments, which are approved by the Food &
Drug Administration (FDA) for use in the United States as
a Humanitarian Device Exemption. The Intacs segments
IOP
consist of a pair of polymethylmethacrylate (PMMA)
semicircular pieces, each having an arc length of 150°
Thinnest point = and a hexagonal cross-­sectional shape. When implanted
Maximum stress surgically, the Intacs rings have an external diameter of
8.10 mm and an internal diameter of 6.77 mm. The thick-
ness of the ring determines the refractive effect. In the
U.S., the FDA-­approved rings are available in thicknesses
IOP
ranging from 0.21 mm to 0.45 mm. Current designs have
a predicted myopic range of correction from –1.00 D to
Figure 7 ​A uniform corneal thickness (top) produces a –4.10 D. A newer Intacs design, Intacs SK, has an inner
uniform stress distribution. A nonuniform corneal thickness
diameter of 6 mm and an oval cross-­sectional shape.
(bottom) produces a stress concentration in the thinnest
region. (Reprinted, with permission from Colin J, Ertan Intacs can be used in patients with keratoconus, pel-
(Kılıç) A, Intracorneal Ring Segments and Alternative Treat- lucid marginal degeneration, and post-­LASIK ectasia who
ments for Corneal Ectatic Diseases. Ankara: Kudret Eye are not adequately and comfortably corrected with rigid
Hospital, 2007:160.) gas-­permeable contact lenses. They should not be used
in patients with significant central corneal scarring or

Intacs Placement

Flattening Central to Segments


Peripheral Steepening Cornea Thickens
Over Segments

Cornea Thins

Redistribution of
Stress

Increased Strain Increased Stress


(Increased Curvature) Concentration

Keratoconus:
Modulation of Biomechanical Properties

Figure 8 ​The red biomechanical cycle reflects disease progression in keratoconus. The blue biomechanical cycle reflects the impact
of Intacs placement. Once the segments are inserted, the curvature is decreased centrally, including the region of the cone. As cur-
vature is decreased in this region, the stress is redistributed, and the decompensatory biomechanical cycle of keratoconus is broken.
(Reprinted, with permission from Colin J, Ertan (Kılıç) A, Intracorneal Ring Segments and Alternative Treatments for Corneal Ectatic
Diseases. Ankara: Kudret Eye Hospital, 2007:164.)

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in patients with untreated atopic disease or evidence ONLINE VIDEO:

of local or systemic infection. While there are no abso- Intacs Insertion: Mechanical Technique, 2 min 00 sec
lute guidelines for the maximum corneal steepness to
be treated with Intacs, some surgeons in the U.S. have The incision and stromal tracks can also be created
suggested 58 D as an upper limit, determined by corneal with the femtosecond laser. Femtosecond laser channel
topography analysis. creation provides a precise tunnel and keratotomy depth,
width, and location; a uniform 360° channel; rare need
Surgical Technique for suture placement; minimal channel haze and edema;
minimal risk of epithelial defect or stromal edema; lower
Intacs procedures are generally performed using topi- risk of infectious keratitis; easier surgical access in deep-
cal anesthesia, as ballooning of the conjunctiva from a set eyes; and completion of the track within seconds
peribulbar block would interfere with the attachment without manipulating the cornea.
of the suction ring used in the creation of the stromal
tracks. Surgery is performed around the geometric cen-
ter of the cornea, which is marked with ink. The incision
axis is determined, and the incision site and location of
the rings is delineated using a specially designed marker.
In the U.S., the “steep axis technique” (placing the inci-
sion at the topographic steep axis of the cornea) seems
to be most popular, but a standard temporal incision can
also be used. After measurement of the corneal thickness
at the incision site, a guarded diamond knife is used to
make the incision depth approximately 70% of the cor-
neal thickness. While there is no requirement pertain-
ing to central corneal thickness, the cornea must have
a thickness of at least 480 µm at the incision site. After
initiation of a stromal channel at the base of the incision,
a complete circular stromal channel is created with a
stromal spreader. The Intacs segments are then inserted a
(Figure 9). The choice of Intacs segments, and whether
done symmetrically or with different-­sized segments, is
based on mean refractive error, amount of astigmatism,
and location of the cone. After correct placement of the
Intacs segments, the incision is closed with tight 10‑0
nylon sutures with the knots buried. Patients are treated
with antibiotic and steroid eyedrops and oral analgesics.
Figure  10 shows examples of pre- and postoperative
topography.

Figure 10 ​Corneal topography examples. a. Preopera-


tive topography prior to Intacs insertion. b. Postoperative
topography, demonstrating flattening of the central corneal
curvature and reduction of topographic astigmatism.

Figure 9 ​Slit-lamp photograph showing perfect placement


of Intacs inserts in a keratonic cornea.

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ONLINE VIDEO: where the cone crossed the 180° meridian. Both groups

Intacs Insertion: Femtosecond Laser Technique, 1 min 35 sec showed similar improvements in UCVA and BCVA.

Complications
Results
Implantation of intracorneal ring segments is generally a
Table 1 summarizes visual and refractive outcomes after very safe surgical procedure. Most significant intraopera-
the use of intracorneal ring segments. While reported tive complications occur while dissecting the channel for
series have varied according to incision placement, size the rings. These include creating too shallow a channel
of rings, and whether rings were placed symmetrically or uneven channel depth, anterior or posterior perfora-
or asymmetrically, results have been extremely consis- tions, and decentration of the channels. These complica-
tent. There is generally a 2 to 4 Snellen line improvement tions should be avoided with the use of the femtosecond
in UCVA, a 1 to 2 Snellen line improvement in BSCVA, laser. Postoperatively, infections are rare but do occur.
a 3 to 4 D improvement in spherical equivalent refrac- In addition, sutures can loosen and lead to secondary
tion, a 4 to 5 D reduction in mean and maximal kera- infection. Segments can migrate, especially if patients
tometry readings, and a mild reduction in astigmatism. rub their eyes, leading to unwanted optical effects (Fig-
Comparisons between the manual and femtosecond laser ure 11). Overlapping segments can cause pressure necro-
methods of Intacs surgery have not shown a consistent sis of the cornea and corneal ulceration. If the ring
difference in results between the two methods. segments cause significant unwanted effects, they are
There have also been comparisons between the use of easily removed, with the cornea reverting to very near
a single ring segment vs paired segments. It stands to the preoperative topography. One advantage of ring seg-
reason that in patients with markedly decentered cones ment surgery is that the central cornea is not violated.
or extreme astigmatism, the best surgical results would Later keratoplasty can be performed, either after ring
be obtained by maximizing the asymmetry of ring place- segment removal, or around the ring segments.
ment (ie, using a single ring). In one report, the surgeon
chose to use a single 0.45‑mm inferior ring in patients
whose cones were entirely within the inferior half of the
cornea, whereas a 0.25‑mm segment was placed superi-
Collagen Crosslinking
orly and a 0.45‑mm segment placed inferiorly in patients While early keratoconus is readily managed with glasses
or rigid gas-­permeable contact lenses, the disease is often

Table 1. Visual and Refractive Outcomes After Placement of Intracorneal Ring Segments in
Keratoconic Eyes
AUT H O R F OLLO W - U P EYES I M P R O V E M E NT

Colin, et al (2001) 12 months 10 Mean K: 4.10 D


Mean SE: 2.12 D
Siganos, et al (2003) 11 months 33 Mean K: 3.23 D
Mean SE: 1.39 D
Ertan (Kılıç), et al (2006) 12 months 118 Mean K: 3.9 D
Mean SE: 3.85 D
Alio, et al (2006) 36 months 13 Mean K: 2.56 D
Mean SE: 1.45 D
Kymionis, et al (2007) 60 months 17 Mean K: 1.57 D
Mean SE: 2.52 D
Kanellopoulos, et al 6 months 20 Mean K: 3.10 D
(2006) Mean SE: 3.69 D
Colin, et al (2006) 6 months 57 Mean K: 4.3 D
Mean SE: 3.1 D
Ertan (Kılıç), et al (2008) 10.39 months 306 Mean K: 2.79 D
5.04 (SD)
Kubaloglu, et al (2011) 18 months 96 Mean K: 3.63 D
Mean SE: 5.88 D
SE: spherical equivalent; K: keratometry

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light depends on its wavelength, its irradiance, and the
irradiation time. Photokeratitis has been shown to occur
in the cornea at wavelengths of 270 to 315 nm (UVB),
with dose densities ranging from 0.12 to 0.56 J/cm2. Cat-
aract has been caused by wavelengths between 290 and
365 nm. Thermal or blue light–induced photochemical
retinal damage is caused by a wavelength range of 400
to 1400 nm. To minimize potential UV toxicity, a precise
370‑nm UVA wavelength is used in CXL. The UVA irradi-
ance must be checked with a meter. In addition, suffi-
cient concentrations of ribof lavin will limit penetration
of the UV light to a depth of 400 µm. To avoid poten-
tial risk for the endothelium, lens, and retina, preopera-
tive pachymetry must be performed in each patient to
exclude patients with extended areas less than 400‑µm
Figure 11 ​Rotation of inferior Intacs segment and migration
across incision into upper channel. (Reprinted, with permis- stromal thickness.
sion from Colin J, Ertan (Kılıç) A, Intracorneal Ring Segments In the traditional technique, the central 9 mm of the
and Alternative Treatments for Corneal Ecstatic Diseases. corneal epithelium is removed by one of various meth-
Ankara: Kudret Eye Hospital, 2007:139.) ods. This facilitates the penetration of 0.1% ribof lavin in
20% dextran, which is administered as an eyedrop every
3 minutes for 30 minutes until the stroma is completely
progressive, and advanced disease leads to the need for saturated and ribof lavin reaches the aqueous. Then the
keratoplasty in as many as 21% of affected patients. A cornea is irradiated using a 370‑nm wavelength ultravio-
procedure that could prevent progression would limit let (UV) lamp at a radiance of 3 mW/cm2 for 30 minutes
the morbidity associated with the disease. That is the (5.4 J/cm2 surface dosage after 30 minutes), continuing
primary goal of corneal collagen crosslinking (CXL). Cur- to instill the ribof lavin drops every 5 minutes during the
rently, CXL is an investigational procedure in the United UV irradiation. The ribof lavin absorbs the UV light to a
States, with several multicenter studies in progress depth of 400  µm, protecting deeper structures within
aimed at gaining FDA approval of the procedure. the eye and activating the photopolymerization process.
Optimal corneal optics require a regular arrange- Therefore, a minimum of 400 µm stromal thickness is
ment of stromal cells and macromolecules. The lattice required for CXL to prevent damaging effects of UV light
arrangement of collagen fibrils embedded in the extra- on deeper structures. After the treatment is completed,
cellular matrix acts to reduce light scattering by means a bandage soft contact lens is placed on the eye, and the
of destructive interference. In keratoconus, a decrease patient is treated with antibiotic and steroid eyedrops
in the number of collagen lamellae, increased separa- for 10 days. The contact lens is removed when the epi-
tion of the collagen bundles, and a loss of the orderly thelium heals.
arrangement of stromal fibrils compromises the optics
of the cornea. CXL alters the biomechanical properties ONLINE VIDEO:

of corneal collagen to arrest these pathologic changes. Collagen Crosslinking for Keratoconus, 8 min 55 sec
This treatment creates additional chemical bonds within
There is active research looking at variations of the
the corneal stroma, increasing its rigidity by 4.5×, and
technique, with the aims of shortening the duration of
increases the diameter of collagen fibrils by means of
the procedure and avoiding the need to remove the epi-
a photopolymerization limited to the anterior corneal
thelium. Modifications of the vehicle of the ribof lavin
stroma.
solution, including altering the tonicity, components
of the vehicle, and inclusion of substances intended
Surgical Technique
to increase epithelial permeability, are being tested to
Ribof lavin is a key component of the photochemical determine whether adequate transepithelial penetration
crosslinking treatment as it increases corneal absorption of ribof lavin can be achieved. Also, higher-­intensity UV
of ultraviolet A (UVA) to approximately 95% and thereby irradiation for shorter periods of time has been tested.
protects the deeper ocular structures, especially the An alternative approach has been to directly inject ribo-
endothelium, from UVA damage. The damage from UV f lavin solution into an intrastromal pocket created with
a femtosecond laser.

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Results treatment with topical steroids. Herpetic keratitis with
iritis, sterile keratitis, persistent epithelial defects,
The results of major trials of collagen are described in microbial keratitis, and permanent corneal haze have
Table 2. After an initial stage in the first postoperative been reported as complications after CXL treatment in
month where the cornea may steepen and develop some keratoconic eyes. All of these complications are related
haze, 6 months after CXL most corneas show some f lat- to the need to remove the epithelium; there is hope
tening compared to the preoperative curvature, with that if transepithelial penetration of ribof lavin can be
an average reduction of approximately 2  diopters. As achieved, eliminating the need for epithelial removal,
a result, there is usually improvement of maximal and these complications can be markedly decreased. Kera-
average keratometry readings, UCVA, and BCVA. After tocyte damage is not considered a major issue, as many
the first 6 months, there is stabilization of the corneal other corneal procedures, including excimer laser refrac-
topography, without progression of the disease. Stud- tive surgery, cause keratocyte damage at similar depths
ies of patients with progressive keratoconus comparing with repopulation by migrating keratocytes occuring by
treated eyes to controls demonstrate statistically signifi- 6 months, with no long-term consequences observed.
cant improvements between the treated and untreated
groups in UCVA, BCVA, maximal and average K readings,
corneal topographic indices, and higher-­order aberra-
tions after 1 year of follow-­up. In patients examined with
confocal microscopy, observations 3 to 6 months after
Combined Collagen
surgery include decreased edema, increased stromal den-
sity, and repopulation of the stroma with activated kera-
Crosslinking and
tocytes in the anterior stroma, with the deep stroma and
endothelium appearing unaffected.
Intrastromal Rings
CXL and intracorneal rings have complementary effects
on the corneal biomechanics and refractive parameters.
Complications
CXL has its effect mainly on the anterior cornea, and
CXL is a simple, easy procedure, but it can cause some intracorneal ring segments provide f lattening effects
adverse outcomes. Inappropriate UV delivery, either and redistribution of stress on deeper layers. Sequential
because of incorrect wavelength or excessive dosage, or or simultaneous treatment with both intracorneal rings
insufficient stromal ribof lavin concentration, can lead and CXL appears to have an additive effect over either
to endothelial damage. The lens and retina are also at procedure alone. Combined treatment results in greater
risk from UV irradiation. improvement of UCVA and BCVA, more reduction of
Stromal haze is common in any procedure where myopia and astigmatism, and a greater than two-fold
the epithelium is removed and generally responds to reduction of mean and steepest keratometry readings

Table 2. Outcomes of Clinical Studies of Collagen Crosslinking


AUT H O R NU M B E R O F PATI E NTS F OLLO W - U P ( M OS ) I M P R O V E M E NT
Wollensak, et al (2003) 22 3–47 Kmax 2.01 D,
BCVA 1.26 lines, SE 1.14 D
Raiskup-Wolf, et al (2008) 241 6–72 Kmax 2.57 D
BCVA improved in 58%
Witting Silva, et al (2008) 24/23 3–12 Kmax 1.45 D, BSCVA 0.12 logMAR
Grewal, et al (2009) 102 12 Stable BSCVA, sph equivalent, curvature
Vinciguerra, et al (2009) 28/28 12–24 Kmax 6.16 D
BSCVA 0.14 lines
Agrawal, et al (2009) 37 12 Kmax 2.73 D in 66%,
BCVA improved ≥1 line in 54%
Capparossi, et al (2010) 44 48 Kmax 2.0 D
BCVA 1.9 lines
Kmax = keratometry value in the steepest meridian, BCVA = best-­corrected visual acuity, BSCVA = best spectacle-­corrected visual acuity,
SE = spherical equivalent refraction

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compared to either procedure alone. Postulated expla- traditionally been treated conservatively with topical
nations for the greater effects include a simple addi- medications including cycloplegics, hypertonic saline
tion of the effect of the two procedures, coupling from drops and ointments, and aqueous suppressants. Cor-
crosslinking of collagen around the ring segments, and neal hydrops occurs in approximately 3% of patients
greater effect of crosslinking due to pooling and concen- with keratoconus.
tration of ribof lavin in the channel around the ring seg- With conservative management, resolution of the
ments. Simultaneous implantation of corneal rings and edema generally takes 2 to 6  months. In 2002, it was
CXL provides the advantage of delivering ribof lavin to found that a series of 1 to 4 air injections into the ante-
the cornea by direct injection into the stromal channel, rior chamber, combined with supine positioning, led
avoiding the need for epithelial removal. to a shortening of the duration of edema from an aver-
age of 64 to 20 days. Because of the need for repeated
injections, the use of longer-­acting gases was proposed.

Combined Collagen Intracameral injections of isoexpansile concentrations


of both sulfur hexaf luoride (20% SF6) and perf luoro-

Crosslinking and propane (14% C3F8) gases have been used with similar
results, with fewer injections needed. SF6 was injected

Photorefractive in a volume of 0.1 ml, whereas C3F8 was injected to fill


two-thirds of the anterior chamber. Injection of a long-­

Keratectomy acting gas into the anterior chamber carries a risk of


pupillary block glaucoma, and one group did routine sur-
Laser refractive surgery has generally been contraindi- gical iridotomies to eliminate this complication. Malig-
cated for keratoconus, based on the premise that abla- nant glaucoma has also been reported. In addition, a rare
tive procedures would further weaken an already weak complication has been migration of gas into the corneal
cornea, worsening the underlying condition. With the stroma, actually prolonging the period of edema. While
advent of collagen crosslinking, however, it is possible gas injection shortens the duration of edema by more
that the increased corneal rigidity created by that pro- than 50%, it does not change the ultimate visual acuity,
cedure would enable simultaneous or subsequent laser once the edema subsides, when compared to eyes that
ablation to be successfully performed. receive conservative management.
Since an initial report of his first case in 2007, Kanel-
lopoulos has described the “Athens Protocol” in which
a limited, topographically guided photorefractive kera-
tectomy (PRK), removing no more than 50 µm of corneal
Lamellar Keratoplasty
stroma to regularize the abnormal keratoconic topog- Until the advent of intracorneal rings and CXL, patients
raphy, is simultaneously combined with CXL. He found who could not achieve adequate vision with optical
significant improvements in UCVA, BSCVA, mean and devices, such as rigid gas-­ permeable contact lenses,
maximal keratometry, and spherical equivalent refrac- had no option other than keratoplasty to improve their
tion, with stable results for up to 4 years. He also found vision. Because of historically superior visual results
that more improvement is achieved with simultaneous when compared to lamellar procedures, PKP has been
treatment compared to sequential treatment of CXL fol- the preferred procedure. PKP, however, is an intraocu-
lowed 6 months later by PRK. lar procedure with significant risks, the most impor-
tant being endothelial graft rejection that, in the young
keratoconus population, occurs in as many as 40% of

Treatment of patients. Lamellar corneal transplantation, which spares


the healthy recipient endothelium and therefore avoids

Corneal Hydrops this complication, might be a better choice of proce-


dure if visual results similar to PKP could be achieved.
With progression of corneal ectasia, Descemet’s mem- Advances in lamellar surgical techniques, using mecha-
brane is stretched to a point where it can tear sponta- nized microkeratome technology to achieve smoother
neously, leading to a rapid inf lux of aqueous f luid into dissection planes or manual techniques that remove all
the corneal stroma, creating significant corneal edema. recipient tissue down to Descemet’s membrane, collec-
This condition, called corneal hydrops (Figure  5), has tively described as deep anterior lamellar keratoplasty
(DALK), have the potential to achieve these results.

Focal Points : Module 2, 2012 9

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Residual posterior stromal thickness is an important corneal surgeons to consider converting to DALK as their
determinant of visual acuity after DALK. Therefore, for preferred keratoplasty technique for keratoconus.
manual techniques, baring of Descemet’s membrane is
important. The most popular techniques for achieving
a dissection to Descemet’s membrane are variations of
Anwar’s “big bubble” technique. In this technique, a Conclusion
half-­thickness trephination is performed, followed by a Keratoconus is a progressive disorder, usually worsening
lamellar dissection and removal of the anterior half of through the fourth decade of life. The traditional treat-
the corneal stroma. A large air bubble is then injected ment of keratoconus has primarily focused on methods
into the residual posterior stroma using a cannula or of visual rehabilitation. In the future, controlling the pro-
30‑gauge needle. If this is successfully performed, the gression of the disease will be another important treat-
air creates a dissection plane at the level of Descemet’s ment goal. That would allow more patients to go through
membrane and separates Descemet’s membrane from life without significant visual impairment. Although long-
the overlying stroma. Once this separation is achieved, term results with controlled studies are not yet available,
the remaining posterior stroma is removed, leaving only early results indicate that intracorneal ring segments
Descemet’s membrane and endothelium. The donor cor- not only provide better visual quality but also may help
nea, from which Descemet’s membrane and endothe- control the progression of keratoconus. With the advent
lium have been removed, is then sutured into place. of collagen crosslinking, and perhaps with the combina-
tion of these modalities, the need for keratoplasty should
ONLINE VIDEO:
decrease. Combined crosslinking and topographically
DALK With Modified Big Bubble, 4 min 20 sec
guided PRK holds promise for even greater recovery of
Several recent studies comparing standard PKP to vision. Moreover, it will be possible to help patients with
Descemet’s-­baring DALK for the treatment of kerato- advanced disease with state of the art lamellar corneal
conus have shown equivalent visual results, comparing surgery. Ultimately, keratoconus will be treated by under-
UCVA and BCVA, refractive spherical equivalent, kerato- standing and controlling the genetic basis of the disease.
metric astigmatism, contrast sensitivity, and wavefront These newer procedures are complementary in man-
aberrometry. The risk of endothelial rejection in these aging patients through the progression of keratoconus.
studies was absent following DALK, compared to 15% When approved, CXL will be used early in the course of
following PKP, although epithelial and anterior stro- the disease, once progression has been identified. Intra-
mal rejections did occur, none leading to failure of the corneal ring segments are most effective in the mid-
graft. In addition, there is better long-term retention of course of the disease, when contact lenses may no longer
endothelial density following lamellar procedures. DALK be effective, but before keratoplasty is the only option.
patients require less postoperative steroids, leading to DALK will be reserved for patients with advanced dis-
faster wound healing, and earlier suture removal means ease, with a decreased risk of endothelial rejection when
faster visual rehabilitation. compared to PKP.
DALK techniques have a steep learning curve, and suc-
cessful baring of Descemet’s membrane is not achieved
in all cases, even in the hands of highly experienced sur-
geons. Surgical time is increased. Poor stromal dissec- Aylin Kılıç, MD, is a practicing ophthalmologist at Dunya
tion or significant perforation of Descemet’s membrane Eye Hospital, Ankara, Turkey.
requires conversion to PKP. In addition, DALK is not
advisable for patients who have suffered from hydrops, Joseph Colin, MD, is a practicing ophthalmologist at
in which a break in Descemet’s membrane has previously Hôpital Pellegrin and Chairman of the Department of
occurred. Given potentially equivalent visual results Ophthalmology at Bordeaux University Medical School,
with lower complications rates, it seems worthwhile for Bordeaux, France.

10 Focal Points : Module 2, 2012

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Clinicians’Corner

Clinicians’ Corner provides additional viewpoints on 1. What clinical exam signs and testing do you use to
identify early keratoconus?
the subject covered in this issue of Focal Points. Con-
Dr. Reidy: The first sign of early keratoconus is decreased
vision from progressive astigmatism. In the early stages
sultants have been invited by the Editorial Review
of keratoconus the slit-lamp biomicroscopic findings are
Board to respond to questions posed by the Acade- usually minimal. Alterations of the red ref lex as viewed
with either a retinoscope or a direct ophthalmoscope are
my’s Practicing Ophthalmologists Advisory Committee often present before frank ectasia can be detected with
the slit lamp. The mainstay of the early diagnosis is com-
for Education. While the advisory committee reviews puterized topographic imaging of the cornea. Traditional
Placido-­based imaging demonstrating focal steepening of
the modules, consultants respond without reading the the inferior cornea with an inferior-­superior difference
of 3 diopters or more is suggestive of keratoconus. More
module or one another’s responses. –Ed. sophisticated Scheimpf lug imaging demonstrates focal
elevation of the anterior surface of the cornea with a
corresponding posterior surface elevation and localized
thinning over the apex of the elevation.

Dr. Schanzlin: The popularity of refractive surgery has


made the diagnosis of early keratoconus very impor-
tant, and over recent years several clinical parameters
have been developed to assist in this task. Classically,
the diagnosis of keratoconus is based on the clinical find-
ings of increasing myopia and astigmatism accompanied
by asymmetric corneal steepening and corneal thinning.
With the advent of Placido disc corneal topography in
the 1980s, Rabinowitz and Klyce taught the importance
of the inferior-­superior difference and radial-­axis skew in
the diagnosis of keratoconus. For the diagnosis of early
keratoconus, however, I rely heavily on the Pentacam
topographic analysis and higher-­order aberration analy-
sis from a Hartmann-­Shack aberrometer.
The Pentacam (Oculus, Lynwood, Washington) is a
rotating Scheimflug camera corneal imaging system that
allows for accurate measurement of the anterior and
posterior corneal surface topography. The Pentacam soft-
ware analyzes the elevation topography of these surfaces
in reference to a best-fit sphere, calculates an axial cur-
vature map based on the elevation data, and determines

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Clinicians’Corner

the thickness of the cornea over the analyzed area. Using patient is then moved to the Intralase unit and docking is
the reports from the Pentacam, we can pick up the earli- achieved in the usual manner. Prior to running the laser
est signs of keratoconus, which include elevation of the pattern, the laser’s software is used to center the treat-
posterior f loat above the best-fit sphere, and the inferior ment pattern over the geometric center of the cornea.
displacement of the thinnest point of the cornea. The Most femtosecond lasers have software for the intracor-
recent addition of the Belin/Ambrosio Enhanced Ectasia neal ring segment surgery. I generally set the software to
analysis software (Oculus) takes the early identification produce a channel at 70% of the corneal thickness in the
of keratoconus to a new level. area of the incision. I set the inner diameter of the chan-
I also find that patients with early or subtle kerato- nel at 6.8 mm, and the outer diameter of the channel at
conus have abnormal coma that is easily observed on 7.8 mm. I set the axis of the incision to match the steep
Hartmann-­Shack aberrometry devices such as the Wave­ meridian of the manifest refraction, specifically the axis
Scan WaveFront System (Abbott Medical Optics, Santa of the “plus” cylinder.
Ana, California) or with similar Zernicke analysis avail- After the channels have been made, and I position
able on some corneal topography devices. the patient again under the VISX microscope with the
ring illumination, I apply a Tegaderm drape to isolate
2. Briefly describe your surgical techniques for intra- the eyelashes. I make a mental note of the corneal mire
corneal ring segments. Do you prefer same-size from the ring illuminator. I then use a blunt “corkscrew”
or asymmetric-­sized segments, including single device of my own design to open the channel 360°. Fol-
segments? lowing this maneuver, the intracorneal ring segments
can easily be placed within the channels. I then place a
Dr. Reidy: I make my incision temporally with a microm-
single 10‑0 nylon suture to reapproximate the wound,
eter diamond blade and perform the lamellar dissection
and I bury the knot. At the end of the case, it is gratify-
manually with the special lamellar dissector from the
ing to see the round mire from the ring illuminator of
Intacs system. The segments are usually centered along
the VISX microscope ref lecting from the corneal surface.
the vertical meridian. If the cone is inferiorly displaced,
I almost always use the same size of intracorneal ring
I insert a thinner segment above and a thicker segment
segments, and similarly I almost always implant two seg-
below in order to displace the cone more centrally. If the
ments. I select the size of the intracorneal ring segments
cone is more centrally located, I use segments of equal
based on the spherical equivalent of the refractive error
thickness that will result in greater overall f lattening.
as recommended by the manufacturer. While I do find
Dr. Schanzlin: For years, I used the manual instruments that approximately 15% of the time I need to remove
developed by KeraVision (Fremont, California) to implant the superior intracorneal ring segment, I rationalize my
intracorneal ring segments. Although occasionally I have approach by saying is easier to take the superior intra-
a case where I prefer the manual technique, most of my corneal ring segment out than it is to put the segment
cases now are done using the Intralase iFS Femtosecond in if I decide I need it later. Unfortunately, in my hands,
Laser (Abbott Medical Optics) to create the channels and I have not been able to predict which cases will do well
the entry incision. I perform the surgeries under the with only one segment.
VISX laser using the ring illuminator.
I begin each case with a povidone-­iodine (Betadine) 3. How often do significant complications with intra-
prep to the lids and lashes, and instill Betadine into corneal ring segments, such as worsening of
the conjunctival cul-de-sac. The patient is then situated vision, occur and how often do the segments need
under the microscope and the geometric center of the to be removed due to complications?
cornea is marked using the inked tip of a Sinskey hook.
Dr. Reidy: Complications associated with intracorneal
I find it important to mark the geometric center of the
rings segments are unusual; however, erosion of seg-
cornea because the apex of the cornea will shift due to
ments into the anterior chamber and extrusion or melt-
the applanation device of the femtosecond laser. The
ing of segments through the overlying cornea stroma

12 Focal Points : Module 2, 2012

FPv30n02_0312.indd 12 1/25/12 4:22 PM


have been reported. Infectious keratitis along the lamel- patients have avoided penetrating keratoplasty. Nonethe-
lar tracts has also been reported. These complications less, in cases where intracorneal ring segments have not
can be sight-­threatening. These types of complications produced the desired effect, I have been able to success-
may be less likely when lamellar dissections are created fully perform penetrating keratoplasty using a standard
with solid-­state lasers that are able to produce incisions technique. Specifically, some surgeons have questioned
more precisely than with the manual systems. Combined whether the Intacs channels make penetrating kerato-
analysis of 3  different studies reported that approxi- plasty more difficult. In my experience, the intracorneal
mately 65% to 75% of patients gained 2 or more lines of ring segments channels do not interfere with either pen-
uncorrected visual acuity (UCVA), while 20% to 30% of etrating or lamellar keratoplasty techniques.
patients had no change in UCVA (defined as + or –1 line
of vision). One of the studies reported 9% of patients lost 4. What is your understanding of the longest-­term
2 or more lines of UCVA. results with collagen crosslinking (CXL)?

Dr. Schanzlin: Intracorneal ring segments have helped Dr. Reidy: The Siena Study has reported results of
many patients achieve improvement in best-­spectacle 44  eyes with a minimum of 4  years of follow-up. The
acuity as well as improvement in correctable vision with mean K value was reduced by a mean of 2 diopters, and
contact lenses. In our practice, patients are not consid- UCVA improved by 2.7 Snellen lines. The Dresden group
ered for intracorneal ring segments until they are proven has reported on 241 eyes with 6 to 60 months of follow-
failures with modern keratoconus contact lenses. In a ­up. The mean K value decreased 2.68 D in the first year
recent review of my cases, I found that over 95% of (142 eyes), 2.21 D in the second year (66 eyes), and 4.84 D
patients achieved 20/40 visual acuity with contact lens in the third year (33 eyes). The BCVA improved by greater
fitting; 60% of these patients were successfully fit with than or equal to 1 line in approximately 55% of patients
a toric soft contact lens, while the rest needed a rigid gas-­ over 3 years. They reported on 23 eyes with follow-­up of
permeable contact lens or hybrid contact lens. between 4 to 6 years, during which time the cornea f lat-
Nonetheless, there are patients in my experience who tening remained stable at approximately 2.5 D.
do not achieve improvement with intracorneal ring seg-
Dr. Schanzlin: Crosslinking of the cornea has been per-
ments. I believe the lack of effect in these cases is most
formed in Europe for more than 10 years, and reports
likely secondary to the extreme elasticity of the cornea.
of long-term follow-up of keratoconus patients treated
In cases where there is no effect, I generally leave the
with this new technique are increasing. These reports
intracorneal ring segments in place for up to 3 months
show significant topographic f lattening, often accompa-
to allow for adequate time for a favorable topographic
nied by improvement in best-­spectacle corrected visual
response, but if the corneal topography is not altered
acuity as well as contact lens corrected visual acuity. Per-
to the point of allowing for a successful contact lens fit,
haps what is most heartening is that stabilization of the
then I will remove the intracorneal ring segments.
attack process seems to be achieved in most cases, and
Complications of intracorneal ring segments have
this effect seems to last over the reported follow-­up peri-
been documented in the literature and include corneal
ods of 4 or more years. Although a few patients have
melt, cornea infection, anterior corneal protrusion, pos-
needed re-­treatment, this does not seem to add a signifi-
terior corneal protrusion into the anterior chamber, crys-
cant risk given the potential benefit of stabilization of
talline deposits in the channel, and epithelial ingrowth
the disease.
into the channel. My experience with complications has
been limited to one case of intrachannel infection, which
5. What complications should clinicians watch for in
was treated with intracorneal ring segment removal and
CXL?
topical fortified antibiotics, and ultimately resolved with
no loss in best-corrected visual acuity (BCVA). Dr. Reidy: Clinicians who perform CXL need to be vigi-
The safety profile for intracorneal ring segments lant for the occurrence of sterile infiltrates occurring in
for the treatment of keratoconus has been good. Many the first several days after treatment. These respond to

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FPv30n02_0312.indd 13 1/25/12 4:22 PM


Clinicians’Corner

topical steroid treatment and usually result in a nebular corrected visual acuity. Other complications seen with
stromal scar. Some patients will progress despite treat- DALK include ruptures of Descemet’s membrane, dou-
ment. Not all patients will respond to treatment. The ble anterior chamber, endothelial cell loss secondary to
Dresden Group reported on complication and failure the air injection, interface debris, interface hemorrhage,
rates seen in 117 eyes of 99 patients that were followed interface vascularization, interface infection, interface
for 1  year. They concluded that if treatment was con- epithelial ingrowth, folds in Descemet’s membrane,
fined to patients 35 years of age or younger, who had sequestered viscoelastic in the interface, and occasional
BCVA less than or equal to 20/25, and maximal K read- corneal epithelial resurfacing problems.
ings of 58 diopters or less, complication and failure rates
could be significantly reduced. 7. Which keratoplasty procedure do you perform (or
favor) for keratoconus? Is that likely to change in
Dr. Schanzlin: Corneal crosslinking is not without com-
the future?
plications, although the incidence of these appears to
be small. In the first several days after the procedure, Dr. Reidy: Until recently, the majority of my surgeries for
ophthalmologists should watch for slow epithelial heal- keratoconus have been PKP, however I have been transi-
ing, bacterial infection, and iritis. Additionally there tioning to DALK over the past year. In the future, I believe
have been reports of herpes simplex keratitis and Acan- DALK could overtake PKP, not only for keratoconus, but
thamoeba keratitis following the crosslinking procedure. for corneal scars and stromal dystrophies as well.
Late onset of stromal haze may rarely limit visual acu-
Dr. Schanzlin: I find that I am doing fewer keratoplasty
ity. Additionally, there have been case reports of diffuse
procedures for keratoconus now that intracorneal rings
lamellar keratitis following crosslinking of post-­L ASIK
are available. When keratoplasty is necessary, however, I
ectasia cases.
still find the visual results with penetrating keratoplasty
gratifying. With PKP for keratoconus, I perform an 8‑mm
6. What are the main advantages and disadvantages
host trephination and I use an 8.25‑mm donor button.
of deep anterior lamellar keratoplasty (DALK) vs
In most cases I use a double-­running suture technique.
penetrating keratoplasty (PKP)?
Since most patients with keratoconus are familiar with
Dr. Reidy: The main advantage of DALK vs PKP is reten- rigid gas-­permeable contact lens wear, I usually fit the
tion of the patient’s own endothelium, and therefore patient with this type of lens at 4 weeks after surgery.
the patient cannot experience endothelial rejection. The Patients tolerate the contact lens fitting very well after
long-term rate of endothelial loss after DALK may be less the surgery, and they achieve excellent visual acuity
than PKP. In addition, the structural integrity of an eye quickly with this approach.
that has undergone DALK is greater than an eye that has
undergone PKP. The main disadvantages of DALK are an 8. Once intracorneal ring segments, CXL, and DALK
increased level of technical difficulty and, for most sur- are fully FDA-­approved and available, what do you
geons, longer surgical times. foresee as the surgical management of keratoco-
nus in the future?
Dr. Schanzlin: The advantages of the procedure include
the following: immune rejection of the corneal endothe- Dr. Reidy: In the future, early detection of keratoconus
lium does not occur; the procedure is extraocular; long- combined with CXL has the potential for eliminating
term use of topical corticosteroids is obviated; sutures most invasive procedures (lamellar and penetrating ker-
may be removed earlier; and the eye is more resistant to atoplasty) for keratoconus. CXL alone, or in combination
traumatic rupture. The disadvantages of DALK include with either ICRS or PRK, may be able to return these
the following: the surgery is difficult to perform and patients to emmetropia.
the learning curve is prolonged; if stromal tissue is
Dr. Schanzlin: Of course, intracorneal ring segments
left behind, BCVA may be limited due to stromal haze;
are approved for use for the treatment of the myopia
and posterior corneal ectasia may result in loss of best

14 Focal Points : Module 2, 2012

FPv30n02_0312.indd 14 1/25/12 4:22 PM


and astigmatism associated with keratoconus under disease early so as to eliminate or drastically reduce the
a Humanitarian Device Exemption for cases that are number of patients who ultimately will need corneal
proven contact lens failures and where the only remain- transplantation.
ing option is keratoplasty. So the real question here is
what will our approach be once CXL is approved. In my
opinion, this is going to drastically alter our approach to James J. Reidy, MD, FACS, is an Associate Professor of
keratoconus. I believe that in the future, patients who Ophthalmology and the Director of the Cornea Service
show the earliest signs of progression of keratoconus at the State University of New York, School of Medicine
will undergo the CXL procedure. For many patients, this & Biomedical Science, Buffalo, New York.
means that they may receive a crosslinking treatment
in their early 20s. For cases with more advanced disease, David J. Schanzlin, MD, is a Professor of Ophthalmol-
I believe our first line of treatment will be CXL alone or ogy, University of California, San Diego and Director,
combined with intracorneal ring segments. The desire Cornea & Refractive Surgery, Shiley Eye Center, San
of this approach will be to halt the progression of the Diego, California.

Focal Points : ModulE 2, 2012 15

FPv30n02_0312.indd 15 1/25/12 4:22 PM


Suggested Reading ribof lavin in progressive keratoconus. Cornea. 2010;29:
409–411.
Agrawal VB. Corneal collagen crosslinking with ribof lavin
and ultraviolet––a light for keratoconus: results in Indian Grewal DS, Brar GS, Jain R, Sood V, Singla M, Grewal SP.
eyes. Indian J Ophthalmol. 2009;57:111–114. Corneal collagen crosslinking using ribof lavin and
­ultraviolet‑A light for keratoconus: one-year analysis using
Alió JL, Artola A, Hassanein A, Haround H, Galal A. One or Scheimpf lug imaging. J Cataract Refract Surg. 2009;35:425–432.
2 Intacs segment for the correction of keratoconus. J Cataract
Refract Surg. 2005;31;943–953. Han DC, Mehta JS, Por YM, Htoon HM, Tan DT. Comparison of
outcomes of lamellar keratoplasty and penetrating kerato-
Alió JL, Shabayek MH, Artola A. Intracorneal ring segments plasty in keratoconus. Am J Ophthalmol. 2009;148:744–751.
(INTACS) for keratoconus correction: long term follow-­up.
J Cataract Refract Surg. 2006;32;978–985. Javadi MA, Feizi S, Yazdani S, Mirbabaee F. Deep anterior
lamellar keratoplasty versus penetrating keratoplasty for
Basu S, Vaddavalli PK, Ramappa M, Shah S, Murthy SI, keratoconus: a clinical trial. Cornea. 2010:365–371.
Sangwan VS. Intracameral perf luoropropane gas in the
treatment of acute corneal hydrops. Ophthalmology. 2011;118: Kamburoglu G, Ertan (Kılıç) A. Intacs implantation using a
934–939. femtosecond laser for management of keratoconus: compari-
son of 306 cases in different stages. J Cataract Refract Surg.
Borderie VM, Sandali O, Bullit J, Gaujoux T, Touzeau O, 2008;34:1521–1526.
Laroche L. Long-term results of deep anterior lamellar vs
penetrating keratoplasty. Ophthalmology. 2011 Nov 4; Epub Kanellopoulos AJ. Collagen cross-­linking in early keratoconus
ahead of print. with ribof lavin in a femtosecond laser-­created pocket: initial
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Related Academy Materials
J, Ertan A, eds. Intracorneal Ring Segments and Alternative Gorovoy MS, Rosenwasser GOD. Advances in Lamellar Corneal
Treatments for Corneal Ectatic Diseases. Ankara: Kudret Göz Surgery. Focal Points: Clinical Modules for Ophthalmologists.
Yayınları; 2007:159–166. Module 4, 2008.

Siganos CS, Kymionis GD, Kartakis N, Theodorakis MA,


Astyrakakis N, Pallikaris IG. Management of keratoconus with
Intacs. Am J Ophthalmol. 2003;135:64–70.

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