Advances in the
Surgical Treatment of
Keratoconus
Aylin Kılıç, MD
Joseph Colin, MD
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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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
Intacs Placement
Cornea Thins
Redistribution of
Stress
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.)
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
Crosslinking and propane (14% C3F8) gases have been used with similar
results, with fewer injections needed. SF6 was injected
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.
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
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
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
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