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Journal Abstracts
Management of keratoconus: A review

Management of keratoconus is often complex and differs

at different stages and state of progression of the disease.
Special attention should be given to optimize visual outcome
depending on the need of the patient. The various modalities
available today can be broadly divided into nonsurgical and
surgical. In early cases, spectacles may provide adequate
visual correction but subsequently, contact lenses provide
better correction due to presence of irregular astigmatism.
Contact lenses are the main stay of nonsurgical management
in moderate and advanced keratoconus. Corneal collagen
cross- linking should be considered as a treatment modality
in patients with progressive disease. Surgery is indicated for
keratoconus when management with contact lenses fails. About
10 to 25% of keratoconus progresses to a condition where
extreme thinning or scarring warrants a keratoplasty. In the
present article, we have reviewed various management options
that have been tried with variable success.

Management
Comparison of current treatment options
Tan et al. (Curr Opin Ophthalmol. 2007;18(4):284-9)
observed that intrastromal corneal ring segments, recent
developments in anterior lamellar keratoplasty enabling
targeted replacement or augmentation of corneal stroma
without replacement of endothelium, microkeratome or laserassisted anterior lamellar surgery, and peripheral tectonic
lamellar keratoplasty procedures are new forms of surgical
alternatives to conventional penetrating keratoplasty (PK) for
restoration of tectonic integrity in corneal ectasia.
Tan et al. (Trans Am Ophthalmol Soc.2006;104:212-20)
described the results of intrastromal corneal ring segments
(Intacs) and a new variation of lamellar keratoplasty,
termed intralamellar keratoplasty (ILK), for the treatment of
keratoconus: Mean uncorrected visual acuity (UCVA) and best
corrected visual acuity (BCVA), and manifest and topographic
astigmatism improved in both the procedures.
Crews et al. (CLAO J. 1994;20(3):194-7) described clinical
management of 118 eyes with keratoconus in a 6 year
retrospective study. Twenty-one eyes received glasses or
required no correction. Rigid gas permeable (RGP) lenses,
Dura-T style PMMA lenses, and specially designed gas
permeable lenses were used to successfully fit 63 eyes. Twentyeight eyes underwent PK, and an additional six eyes were PK
candidates. Factors associated with the need for PK included
BCVA of 20/40 or worse, average keratometry > 55D, and the
presence of apical scarring.
Carney et al. (CLAO J. 1991;17(1):52-8) compared the visual
performance of keratoconus patients whose vision had been
corrected with one of the following: RGP lenses alone, PK, or
epikeratoplasty. Despite similar BCVA, there were statistically
significant differences in the visual responses for the three

correction types, with the epikeratoplasty procedure providing

the poorest results.
Contact lens
Abdalla et al. (Cornea2010;29(1):5-8) discussed the initial
results of fitting SynergEyes hybrid contact lenses (SynergEyes,
Inc, Carlsbad, CA) for keratoconus (KC) and pellucid marginal
degeneration (PMD). The most common indication for
SynergEyes was RGP lens intolerance (50.8%). The success rate
was 86.9%; most failures (8 eyes of 6 patients) discontinued the
lens within the first 1-2 months because of discomfort (5 eyes)
or unsatisfactory vision (3 eyes).
Yanai et al. (Eye Contact Lens. 2010;36(2):86-9) determined
whether Aphex KC, a newly designed RGP contact lens (CL)
with dual aspherical base curves, improves visual acuity and
lens wear time in 24 patients with keratoconus. A total of 19
eyes (65.5%) had a BCVA of 0.2 logarithm of the minimum angle
of resolution (logMAR) (20/29) or better with Aphex KC. The
mean wear time was 12.63.2 hr/day with Aphex KC and 0.4
0.7 hr/day with a previously prescribed spherical RGP CL.
The numbers of eyes wearing Aphex KC or the previously
prescribed RGP CL for >12 hr/day were 26 and 0, respectively.
Schornack et al. (Eye Contact Lens. 2010;36(1):39-44)
described the use of Jupiter scleral lenses (Medlens Innovations,
Front Royal, VA; and Essilor Contact Lenses, Inc., Dallas, TX) in
the management of keratoconus in 52 eyes of 32 patients. Thirty
eyes (19 patients) were fitted successfully. The average number
of lenses ordered per eye was 1.5. The fitting process required
an average of 2.8 visits. Standard lenses were prescribed for
23 eyes, and custom designs were needed for 7 eyes. Median
BCVA improved from 20/40 (mean, 20/76) before scleral lens
fitting to 20/20 (mean, 20/30) after fitting. Follow-up ranged
from 3 to 32 months.
Yamazaki et al. (Arq Bras Oftalmol. 2006;69(4):557-60)
evaluated the fitting and use of a soft CL in 66 keratoconic
patients (80 eyes). The main reasons for this special lens fitting
were due to reduced tolerance and poor fitting pattern achieved
with other lenses. The majority of eyes of patients using CL
(91.25%) achieved visual acuity better than 20/40. The most
frequent base curve was 7.6 mm (61%).
Edrington et al. (Optom Vis Sci. 2004;81(3):182-8) identified
factors associated with rigid contact lens comfort in the CLEK
Study. Measures of disease severity (steep keratometry and
the first definite apical clearance lens) were not associated
with lens comfort. There was no difference in self-reported
contact lens comfort between patients fitted with apical touch
vs. apical clearance. Patients with a peripheral clearance rating
of "minimal unacceptable" were approximately half as likely to
report good contact lens comfort compared with patients with
"average" peripheral clearance.
Lee et al. (Eye Contact Lens2004;30(1):20-4) evaluated the

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IndianJournalofOphthalmology

clinical outcome and fitting characteristics with a multicurve

lens for keratoconus in a Korean population. Three-point touch
was applied in 59 eyes, and apical clearance was adopted in an
eye with a persistent epithelial defect. Flatter lens was related
to better visual acuity. The mean daily wearing time was 11.9
hours. Customization with the multicurve lens enabled patients
with keratoconus to wear the CL comfortably.
Segal et al. (Cornea2003;22(4):308-10) described the visioncorrecting and therapeutic benefits of gas-permeable scleral
contact lenses (GP-ScCL) and reported marked subjective
improvement in quality of life by 86% of patients, mainly
due to improvement in their visual function and reduction in
discomfort.
Betts et al. (Optom Vis Sci. 2002;79(8):493-501) studied
the visual performance and comfort with the Rose K lens for
keratoconus. There were no statistically significant changes
in high- or low-contrast visual acuity with the Rose K lenses.
There was statistically significant improvement in self-reported
assessment of vision and self-reported assessment of comfort in
the eyes with more advanced keratoconus. At the conclusion of
the study, 72% of patients preferred the Rose K lenses over their
habitual lenses, and 87% reported that they would continue
wearing the Rose K lenses.
Lim et al. (Eye (Lond). 2002;16(1):54-9) observed that
16.4% of all patients attending the Contact Lens clinic had
keratoconus. The mean age at referral was 28.6 years and the
mean age of keratoconus during the study period was 34.9
years. The types of contact lenses used included PMMA lenses
(2.7%), rigid gas permeable lenses (96.1%) of the spherical,
elliptical and special cone lens designs, Keratosoft or Softperm
lenses (0.8%) and scleral lenses (0.4%). Sixty four percent of eyes
that had undergone PK required CL. They concluded that RGP
CLs remain the mainstay treatment for advanced keratoconus.

Vol. 58 No. 4

of under 20/40 [despite good contact lens fit] (43%), contact

lens intolerance (32%), frequent lens displacement (13%), and
significant peripheral thinning of the cornea (12%).
Intacs
Coskunseven et al. (J Refract Surg. 2010 Feb 25:1-4)
described the visual and refractive outcomes in three eyes (two
keratoconic patients) with previous intrastromal corneal ring
segments (INTACS, Addition Technology Inc) that underwent
adjuvant single KeraRing (Mediphacos) intrastromal corneal
ring segment implantation. Six months following surgery, the
UCVA improved from 20/100 and 20/200 to 20/32 and 20/40
in the right and left eyes, respectively, of patient 1, and from
20/400 to 20/50 in the treated right eye of patient 2. Keratometric
measurements also improved.
PiAero et al. (Clin Experiment Ophthalmol. 2010;38(2):15467) summarized all the scientific literature regarding the use
of intracorneal ring segments (ICRS) in corneal ectatic disease
and concluded that ICRS technology is a promising therapeutic
option in avoiding or deferring the need of a corneal graft.
Titiyal et al. (Eur J Ophthalmol. 2010 Mar 23. [Epub ahead
of print]) successfully performed deep anterior lamellar
keratoplasty (DALK) with Intacs explantation in two patients
with poor visual outcome with Intacs for KC. At 2 years followup, the grafts were clear in both patients, with a BCVA of 20/25.
Feldman et al. (J Cataract Refract Surg. 2010;36(2):332-5)
reported the enhanced effect of double-stacked intrastromal
corneal ring segments in a patient with poor visual acuity
and contact lens intolerance due to advanced keratoconus.
Postoperatively, both rings migrated inferiorly and overlapped
each other in a double-stacked formation. This resulted in a
dramatic central shift of the cone and flattening of the inferior
paracentral cornea with significant improvement in vision.

Edrington et al. (Optom Vis Sci.1996;73(6):369-75) developed

a standardized rigid CL fitting protocol for keratoconus. The
initial trial lens's base curve was the average keratometric
reading; sequentially steeper lenses were applied until definite
apical clearance was observed.

Sansanayudh et al. (J Cataract Refract Surg.2010;36(1):1103) reported the efficacy and safety of ICRS implantation using
femtosecond laser in 10 eyes with advanced keratoconus.
The mean UCVA and aberrations improved significantly at 6
months (P= 0.007), and there was no complication.

Yeung et al. (Am Optom Assoc.1995;66(9):539-43) published

their clinical experience with piggyback contact lens systems
(PBCLS) on 16 keratoconic eyes. Vision and average lens
wearing time were stable, if not improved, for the 16 patients
when the data was compared to the patients' wear of RGP CL.
Although two patients developed neovascularization and one
patient developed giant papillary conjunctivitis (GPC), all preexisting corneal complications resolved with PBCLS wear. No
gross corneal edema was noted upon slit lamp observation.

Kymionis et al. (Open Ophthalmol J. 2009;3:77-81) reviewed

a case of early stage keratoconus after nine years of bilateral
Intacs implantation and reported a significant improvement
and postoperative stability in patient's visual acuity with
absence of any long-term, sight-threatening complication.

Kok et al. (Cornea1993;12(1):60-4) published their results of

40 eyes in 26 patients who were consecutively fitted with a new
aspheric combination lens. Thirty-five eyes (87.5%) showed
no discomfort, acceptable wearing time, and visual acuity.
Failures were found in five eyes (12.5%), which were caused
by GPC (two eyes), severe keratoconus (one eye), difficulty in
lens handling and lack of motivation (two eyes). The successful
use of an aspheric piggyback combination lens in rigid lensintolerant keratoconus may preclude early surgical therapy.
Dana et al. (Ophthalmology1992;99(8):1187-92) observed
that the primary reasons for PK in keratoconus were, a BCVA

Shetty et al. (Cornea2008;27(9):1022-9) evaluated the

surgical outcomes of microthin INTACS implantation in 14
eyes with advanced KC. At 6 months, the UCVA improved
from 0.05 0.08 to 0.16 0.11 (P < 0.05), and BCVA improved
from 0.50 0.23 to 0.67 0.00 (P= 0.01). Younger age, male sex,
and minimum central pachymetry of more than 400 seemed
to be associated with better outcomes.
Ertan et al. (J Cataract Refract Surg. 2008;34(9):1521-6)
evaluated the efficacy of intrastromal ring segment (Intacs,
Addition Technology, Inc.) implantation and analyzed
outcomes according to different keratoconus stages in 306 cases.
At a mean follow-up of 10.395.04 months, BCVA improved
in 71.6% of eyes and UCVA improved in 75.7% of eyes. The
mean keratometry (K) decreased from 50.7 diopters (D) to
47.9 D (P<0.05). Intacs treatment with femtosecond laser was

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Sinha, et al.: Management of Keratoconus
July - August 2010

effective for management of keratoconus of all stages, however,

there was less improvement in UCVA in severe keratoconus.
Kymionis et al. (Am J Ophthalmol. 2007;143(2):236-244)
evaluated long-term follow-up of Intacs microthin prescription
inserts in 17 eyes of keratoconus. No late postoperative
complication occurred in any patient. At five years, the
spherical equivalent (SE) error was statistically significantly
reduced.
Zare et al. (J Cataract Refract Surg. 2007;33(11):1886-91)
assessed the safety and efficacy of intracorneal ring segment in
30 keratoconic eyes. All patients were contact-lens intolerant.
The mean UCVA improved from 0.60 0.31 preoperatively to
0.29 0.20 at 6 months postoperatively (P<0.001) and the mean
BCVA, from 0.250.16 to 0.13 0.14, respectively (P<0.001). The
mean keratometry decreased from 49.84 3.58D to 47.90 3.58D
postoperatively (P<0.001). Three eyes had ring exposure, and
1 eye had bacterial keratitis and ring exposure.
Ertan et al. (J Cataract Refract Surg. 2006;32(12):2039-42)
assessed the outcomes of Intacs implantation for keratoconus
using the femtosecond laser in 118 eyes of 69 patients. Intacs
were successfully implanted in all eyes. At the end of the first
postoperative year, 81.3% of eyes had improved UCVA and
73.7% had improved BCVA. The mean keratometry decreased
from 51.56D to 47.66D, and the mean refractive spherical
equivalent decreased from -7.57 D to -3.72D.
Hellstedt et al. (J Refract Surg. 2005;21(3):236-46) reported
that asymmetric Intacs placement improves BCVA and UCVA
and reduces astigmatism in patients with mild to moderate
keratoconus; however, the correction of astigmatism was
unpredictable.
Siganos et al. (Am J Ophthalmol. 2003;135(1):64-70)
studied the use of Intacs microthin prescription inserts for the
management of keratoconus and reported improvement in
UCVA and BCVA in majority.

Collagen Cross linking

Standard technique
Mazzotta et al. (Acta Ophthalmol. 2010 Apr 23. [Epub
ahead of print]) investigated the correlations between corneal
structural modifications assessed by in vivo corneal confocal
microscopy with visual function after corneal collagen
cross-linking (CXL) in 44 eyes with progressive keratoconus.
Epithelial thinning associated with stromal oedema and
keratocytes apoptosis explained initial tendency towards
slightly reduced visual acuity (VA) and more glare one
month postoperatively in 70% of eyes. There was a significant
correlation between visual functions and morphological
findings.
Goldich et al. (Cornea2010 Feb 15. [Epub ahead of print])
observed that CXL is a safe procedure that does not cause
damage to the corneal endothelium and central retina.
Comparative preoperative and postoperative results showed
stable endothelial cell density (2730 cells/mm, 2793 cells/mm,
and 2640 cells/mm, preoperatively, at month 6, and at month
12, respectively) and stable foveal thickness (203, 202, and 205
, preoperatively, at month 6, and at month 12, respectively).
Stojanovic et al. (J Refract Surg. 2010;26(2):145-52) evaluated

355

a combination of topography-guided custom ablation and

CXL in a single procedure for the treatment of keratectasia.
Mean UCVA increased from 20/1000 preoperatively to 20/125
at 12 months postoperatively. Mean astigmatism reduced
from 5.402.13D to 2.701.44D, and keratometric asymmetry
decreased from 6.381.02D to 2.760.73D and no progression
of ectasia occurred during the observation period of 12 months.
Caporossi et al. (Am J Ophthalmol. 2010 Feb 5 [Epub
ahead of print]) reported stability in 44 eyes after 48 months
of minimum follow-up after CXL, while fellow eyes showed a
mean progression of 1.5D in more than 65% after 24 months.
There was a reduction of 2D in the mean K value, with
reduction in coma aberration and improvement in VA.
Coskunseven et al. (J Cataract Refract Surg. 2009;35(12):208491) reported their results of combined ICRS implantation and
CXL in progressive keratoconus. In this study, CXL was
followed by ICRS implantation (Group 1) or ICRS implantation
was followed by CXL (Group 2). The UCVA, BCVA and corneal
parameters improved in both the groups, however there was
greater improvement in Group 2.
Constantin et al. (Oftalmologia. 2009;53(2):88-91) observed
an increase in corneal thickness, and reduction in keratometric
values in keratoconus patients after CXL.
Coskunseven et al. (J Refract Surg. 2009;25(4):371-6)
assessed the progression of keratoconus in patients treated with
CXL. The worse eye was treated with collagen cross-linking,
and the fellow eye served as the control. The group treated with
CXL showed a mean decrease in spherical equivalent refraction
and cylinder and an increase in UCVA and BCVA. The maximal
curvature decreased by 1.57 1.14D, and no statistical difference
was noted regarding central corneal thickness (P=0.06) and
endothelial count (P= 0.07). They concluded that CXL appears
to inhibit the progression of keratoconus.
Hafezi et al. (J Cataract Refract Surg. 2009;35(4):621-4)
studied the effect of CXL in advanced keratoconus with thin
cornea by preoperatively swelling thin corneas to a stromal
thickness of at least 400 using hypoosmolar riboflavin solution
in 20 patients with no complication.
Grewal et al. (J Cataract Refract Surg. 2009;35(3):425-32)
used Scheimpflug imaging to illustrate their results on CXL.
There were no significant differences (P > 0.05) in mean
preoperative and postoperative values at 1 year in BCVA, SE,
and cylinder vector.
Wittig-Silva et al. (J Refract Surg. 2008;24(7):S720-5)
reported flattening of the steepest simulated keratometry
(sim-K) value (K-max) by an average of 0.74D (P = 0.004) at 3
months, 0.92D (P = 0.002) at 6 months, and 1.45D (P = 0.002) at 12
months after CXL. In the control eyes, mean K-max steepened
by 0.60D (P= 0.041) after 3 months, by 0.60D (P= 0.013) after 6
months, and by 1.28D (P 0.0001) after 12 months. Preliminary
results of this study suggest a temporary stabilization of all
treated eyes after CXL.
Raiskup-Wolf et al. (J Cataract Refract Surg. 2008;34(5):796801) published their long term results of CXL in 272 patients
with progressive keratoconus. The steepening decreased
significantly by 2.68D in first year, 2.21D in second year, and
4.84D in third year. Two patients had continuous progression
of keratoconus and required repeat CXL.

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IndianJournalofOphthalmology

Transepithelial technique
Leccisotti et al. (J Refract Surg. 2010 Feb 25:1-7) evaluated
the clinical effects of transepithelial CXL on 51 keratoconic
eyes pre-treated with substances enhancing epithelial
permeability. Gentamicin, ethylenediaminetetraacetic acid and
benzalkonium chloride were instilled for 3 hours. Riboflavin
0.1% in 20% dextran-T500 and oxybuprocaine were instilled
for 30 minutes. Finally, UV-A irradiation to the central 7.5
mm of cornea was applied for 30 minutes, while riboflavin
was instilled every 5 minutes. The mean BCVA improved
by 0.036 logMAR after CXL and worsened by 0.039 logMAR
in the control eyes (P<0.05). Mean refraction (SE) decreased
by 0.35D (less myopic) after CXL and increased by 0.83D in
control eyes (P<0.05). Mean sim-K decreased by 0.10D after
CXL and increased by 0.88D in control eyes (P<0.05). A limited
but favorable effect of trans-epithelial CXL was noted without
complications.
Comparison of standard and modified procedure
Bakke et al. (J Cataract Refract Surg. 2009;35(8):1363-6)
compared the severity of postoperative pain and the rate of
penetration of riboflavin between eyes treated by CXL using
excimer laser superficial epithelial removal, programmed to
35 (Group 1), and mechanical full-thickness epithelial removal
with a brush (Group 2). Postoperative pain was severe in 40.0%
of patients in Group 1 and in no patient in Group 2 (P= 0.009)
and moderate in 53.3% and 33.3%, respectively (P= ns).The
mean time to riboflavin saturation was 43.710.8 minutes in
Group 1 and 31.33.0 minutes in Group 2 (P = 0.001). Superficial
epithelial removal using excimer laser resulted in more
postoperative pain and the need for prolonged application of
riboflavin to achieve corneal saturation.
Baiocchi et al. (J Cataract Refract Surg. 2009;35(5):893-9)
evaluated the intrastromal concentrations of riboflavin in CXL
with (standard) and without epithelium (transepithelial). The
HPLC quantitative study showed that a theoretically safe
and effective stromal riboflavin concentration of 15 g/g was
obtained only after epithelial removal and after at least 10
minutes of riboflavin application every 2 minutes.
Wollensak et al. (J Cataract Refract Surg. 2009;35(3):540-6)
observed that CXL without epithelial debridement reduced
the biomechanical effect by approximately one fifth compared
with standard cross-linking, probably because of restricted
and inhomogeneous stromal distribution of riboflavin. The
cytotoxic damage was restricted to 200 stromal depth, which
is an advantage over the standard method.
Laboratory studies
Ahearne et al. (Br J Ophthalmol. 2008;92(2):268-71) studied
the effect of CXL on the mechanical properties of collagen
hydrogels and reported that UVA irradiation without riboflavin
showed decreased mechanical integrity and strength. Cell
viability was reduced with increased UVA exposure time.
Wollensak et al. (Cornea2004;23(5):503-7) studied the
collagen fiber diameter in rabbit cornea after CXL and reported
a significantly increased diameter by 12.2% (3.96 nm) in the
anterior stroma, and by 4.6% (1.63nm) in the posterior stroma,
compared with the control fellow eyes. This suggests that
CXL effect is strongest in the anterior stroma because of rapid

Vol. 58 No. 4

decrease in UVA irradiance across the corneal stroma as a result

of riboflavin-enhanced UVA absorption.
Complications of CXL
Koller et al. (J Cataract Refract Surg. 2009;35(8):1358-62)
evaluated 117 eyes of 99 patients, approximately 90% of
which completed 12-month follow-up. The complication rate
(percentage of eyes losing 2 or more Snellen lines) was 2.9%.
The failure rate of CXL (percentage of eyes with continued
progression) was 7.6%. A high preoperative Kmax reading was
a significant risk factor for failure. Sterile infiltrates were seen in
7.6% of eyes and central stromal scars, in 2.8%. Results indicated
that a preoperative maximum K reading less than 58.0D may
reduce the failure rate to less than 3%, and restricting patient
age to younger than 35 years may reduce the complication
rate to 1%..
Parez-Santonja et al. (J Cataract Refract Surg. 2009;35(6):113840) reported a case of Staphylococcus epidermidis keratitis
following an uneventful CXL for keratoconus.
Sharma et al. (J Cataract Refract Surg. 2010;36(3):517-20)
reported a case of Pseudomonas aeruginosa keratitis occurring
one day after CXL for keratoconus. They attributed presence
of epithelial defect, use of a soft bandage contact lens, and
topical corticosteroids in the immediate postoperative period
as the risk factors.
Kymionis et al. (J Cataract Refract Surg. 2007;33(11):19824) reported geographic epithelial keratitis and iritis following
CXL without prior history of herpetic disease.
Toric intraocular lens
Navas et al. (J Cataract Refract Surg. 2009;35(11):20247) described two cases of forme fruste keratoconus (FFKC)
wherein toric intraocular lens (TIOL) implantation was done
following cataract surgery. One year postoperatively, the UCVA
was 20/25 in both cases, and no progression or IOL rotation was
observed in either case.
Venter (J Refract Surg. 2009;25(9):759-64) observed that
implantation of Artisan iris-fixated phakic intraocular lens
(PIOL) in patients with stable keratoconus for correction of
astigmatism and myopia is safe, predictable, and effective with
minimal complications.
Kamiya et al. (J Refract Surg. 2008;24(8):840-2) implanted
toric implantable Collamer lenses (toric ICL, STAAR Surgical)
in two patients with high myopic astigmatism and stable
keratoconus. Postoperatively, the manifest refraction in the two
eyes was +0.50 -1.00 x 90 in case 1 and -0.25 -1.25 x 100. They
concluded that toric ICL may be an alternative in managing
eyes with stable keratoconus.
Coskunseven et al. (Am J Ophthalmol. 2007;144(3):387389) evaluated the results of combined Intacs and posterior
chamber toric ICL implantation at intervals between six and 10
months following Intacs in keratoconic patients with extreme
myopia and irregular astigmatism. There was improvement
in UCVA and BCVA with no intraoperative or postoperative
complication. All eyes were emmetropic within 1D.
Budo et al. (J Refract Surg. 2005;21(3):218-22) also reported
that Artisan toric PIOL may be an alternative for treating

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Sinha, et al.: Management of Keratoconus
July - August 2010

astigmatism and myopia in keratoconus patients with contact

lens intolerance.

Corneal Transplantation
Penetrating keratoplasty (PK)
Fukuoka et al. (Cornea2010;Mar 17 Epub ahead of print)
investigated long-term results of PK for keratoconus. With
an average follow-up period of 15.3 9.7 years, 9.6% eyes
experienced rejection and 4.0% eyes experienced graft failure.
The average BCVA 10, 20, and 25 years after surgery was 1.54
0.68, 0.06 0.22, 0.03 0.17, and 0.14 0.42, respectively. The
cumulative probability of graft survival at 10, 20, and 25 years
after PK was 98.8%, 97.0%, and 93.2%, respectively.
Kymionis et al. (J Cataract Refract Surg. 2009;35(1):113) performed arcuate keratotomy [anterior arcuate side
cut (400 thick, 6.5mm diameter)] using the keratoplasty
software, with femtosecond laser in a 68-year-old woman
with irregular astigmatism 28 years after PK for keratoconus.
No intraoperative or postoperative adverse events were seen
during the follow-up period. Six months after the procedure,
the UCVA improved from 20/60 to 20/50 and mean manifest
astigmatic correction decreased to 0.50D.
Wagoner et al. (Cornea2009;28(1):14-8) evaluated graft
survival, postoperative complications, and visual outcome after
PK for keratoconus in 464 eyes with (17.2%) or without (82.8%)
a history of vernal keratoconjunctivitis (VKC). There were
no statistically significant differences in Kaplan-Meier graft
survival or complications between the 2 groups at any time
interval. However, late-onset persistent epithelial defects were
significantly more likely to occur in eyes with VKC (P= 0.04).
Wagoner et al. (Cornea2009;28(8):882-90) evaluated graft
survival after PK for various indications and reported that
5-year survival probability was 96.1% for keratoconus, 71.1%
for stromal scarring, 85.9% for stromal dystrophy, and 40.3%
for corneal edema.
Bahar et al. (Am J Ophthalmol. 2008;146(6):905-12)
compared the visual outcomes following DALK, penetrating
keratoplasty (PK), and manual top-hat PK (TH-PK) in subjects
undergoing corneal transplantation for keratoconus. The
median BCVA at 12 month follow-up was 20/40 in DALK eyes
and 20/30 in traditional PK and TH-PK eyes. DALK, PK, and
TH-PK provide comparable visual outcomes in keratoconus
patients. Although DALK and TH-PK induce more HOA,
they speed up the time to suture removal and provide higher
endothelial cell density at one year follow-up.
Jaycock et al. (Ophthalmology2008;115(2):268-75) used
same-size donor and recipient trephines for PK in eyes with
keratoconus. The mean SEs for the same-size and oversize
donor trephine groups, respectively, were -1.45D and -1.41D at
1 year (P =0.6) and -1.74D and -2.19D at 2 years postoperatively
(P = 0.3). They noted that use of same-size donor and recipient
trephines did not reduce myopia and was associated with an
increased risk of postoperative wound leaks.
Shoja et al. (Saudi Med J.2007;28(9):1389-92) in their study
found that PK with 0.25 mm disparity had a better final VA
and lesser myopic shift than those with 0.50 mm oversize in
patients of keratoconus.

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Gordon et al. (Am J Ophthalmol. 2006;142(6):923-30)

identified baseline demographic and clinical factors associated
with high likelihood of PK that included younger age, steeper
keratometric values, worse VA, corneal scarring, poorer contact
lens comfort, and poorer vision-related quality of life.
Lamellar corneal surgery
Chan et al. (Cornea. 2010;29(3):290-5) reviewed patient
outcomes after femtosecond laser-assisted mushroom
configuration DALK and showed good morphological outcome
with stepped corneal wounds. At 3 months, mean BCVA was
20/40.
Feizi et al. (Cornea2010;29(2):177-82) evaluated the
visual and refractive outcomes after DALK using big-bubble
technique in 129 eyes with keratoconus. In 3 eyes, the operation
was converted to PK. Bared descemets membrane (DM)
was achieved in 103 eyes, whereas in 23 eyes pre-Descemet
dissection was performed. DM perforation occurred in 5 eyes.
Shi et al. (Ophthalmology2010;117(2):226-31) performed
a modified deep lamellar keratoplasty for advanced-stage
keratoconus and reported similar efficacy to PK with lesser
risk of immune rejection. Javadi et al. (Cornea2010 Feb 17Epub
ahead of print) compared DALK using big-bubble technique
to PK in patients with moderate to advanced keratoconus and
reported comparable results.
Sarnicola et al. (Cornea2010;29(1):53-9) reported outcomes
in 236 cases with Descemetic (dDALK) and predescemetic
(pdDALK) DALK. There was no difference in VA between the
two groups at an average follow-up of 30.4 months, although
the eyes in dDALK group had faster visual recovery.
Javadi et al. (Cornea. 2009;28(10):1130-4) reported that
relaxing incisions and suturing at the steep meridian followed
by selective suture removal can effectively and predictably
reduce post-DALK astigmatism in patients with keratoconus.
Jones et al. (Invest Ophthalmol Vis Sci. 2009;50(12):5625-9)
compared DALK and PK in keratoconus and noted that the
risk of early graft failure for DALK was almost twice that for
PK (P= 0.02). Excluding these early failures, there was little
difference between the 3-year graft survival. DALK recipients
were less likely to achieve BCVA of 6/6 and were more likely
to have -3D or worse myopia.
Han et al. (Am J Ophthalmol. 2009;148(5):744-751) compared
outcomes after PK and two techniques of DALK in patients with
keratoconus. DALK group had a significantly lower incidence
of complications compared with PK cases, including allograft
rejection and glaucoma.
Farid et al. (J Cataract Refract Surg. 2009 ;35(5):809-13)
described a variation of big-bubble DALK using femtosecond
laser zigzag incision. This technique allowed precise depth
visualization for air-needle placement in the posterior stroma
based on the lamellar and posterior laser cuts, thus minimizing
the risk for perforation of DM. The matching donor and host
tissue zigzag cut allowed more precise tissue apposition and
greater surface area for healing. The angled anterior donor
and host junction allows a smooth transition for improved
visual outcomes.
Spadea et al. (J Cataract Refract Surg.2009;35(1):105-12)

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IndianJournalofOphthalmology

evaluated anatomical and functional results of excimer laserassisted lamellar keratoplasty (ELLK) in 41 keratoconus
patients. The UCVA and BCVA were significantly better at
all follow-ups. The mean refractive astigmatism was 2.20D
and mean manifest refraction (SE) was -1.18D. Complications
included corneal melting that was treated with PK (1 case)
and postoperative high refractive error requiring excimer laser
photorefractive keratectomy.
Farias et al. (Cornea2008;27(9):1030-6) compared the efficacy
of lyophilized corneas versus optisol corneas for DALK in
patients with keratoconus. All variables improved similarly
in both groups, except for UCVA in the sixth postoperative
month, which was better in the lyophilized group.
Das et al. (Cornea2007;26(9):1156-7) reported a case of
successful deep lamellar keratoplasty (DLK) in a patient with
keratonous after healed hydrops.
Coullet et al. (J Refract Surg.2008;24(2):166-72) evaluated
a new automated technique microkeratome-assisted additive
stromal keratoplasty (MASK) for the management of
keratoconus. The first step of the surgical procedure consisted
of performing a nasal-hinged flap on the host cornea with
a microkeratome. The second step consisted of extracting a
stromal plano-powered disk from the donor's cornea using an
artificial chamber. The 80 thick lamellar graft was punched
with a 7.5-mm circular trephine and positioned beneath the
flap. Corneal refractive surgery was scheduled for the end of
the sixth postoperative month. At the end of surgery, mean
corneal thickness was increased by 148.75. MASK appeared
to be a safe surgical technique but cannot be considered as an
alternative to PK or DLK in the management of keratoconus
because of imprecise anatomic and refractive outcomes.
Coombes et al. (Br J Ophthalmol. 2001;85(7):788-91)
reported the results of DLK in 44 eyes using lyophilised donor
corneal tissue in keratoconus. Perforation of DM occurred in
nine eyes (20%). A double anterior chamber was noted formed
in five eyes, which resolved spontaneously in three eyes. The
median corrected postoperative acuity was 6/9.
Epikeratoplasty and phototherapeutic keratectomy (PTK)
Ward et al. (CLAO J. 1995;21(2):130-2) performed PTK in
four cases of keratoconus with contact lens intolerance due
to pain and raised, nodular scars. All patients resumed RGP
contact lens wear 2 to 4 weeks after surgery. Visual acuities

Vol. 58 No. 4

ranged from 20/40 to 20/70 before surgery and improved to

20/25 or better in all patients postoperatively. PTK flattened
the central cornea by an average of 5.43D (range: 1.13-10.19
D); and refractive cylinder was reduced by an average of
4.00D (range: 3.25-5.25 D). Goosey et al. (Am J Ophthalmol.
1991;111(2):145-51) studied 47 eyes with keratoconus that were
surgically corrected with either epikeratoplasty (N= 31) or PK
(N= 16). The PK group had higher percentage of eyes with VA
of 20/20 than the epikeratoplasty group (73% vs 24.1%). The
PK group had 3.0D greater keratometric flattening.
Management of hydrops
Poyales-Galan et al. (Arch Soc Esp Oftalmol.2009;84(10):
533-6) successfully managed a case of hydrops occurring
in the eye secondary to keratoconus by intracameral sulfur
hexafluoride (SF6) gas.
Panda et al. (Cornea. 2007;26(9):1067-9) also reported
successful use of SF6 gas in early resolution (4 weeks) of
corneal edema caused by acute corneal hydrops secondary
to keratoconus in 9 eyes in comparison to 9 eyes managed
conservatively (12 weeks).
Wylegala et al. (Ophthalmology2006;113(4):561-4)
performed amniotic membrane transplantation with
cauterization in keratoconus complicated by persistent
hydrops in 10 eyes of mentally retarded patients. Hydrops
was successfully resolved in all eyes. The mean initial corneal
protuberance (7.64.2 mm) resolved after 21.49.5 days in all
eyes.
Sii et al. (Cornea2005;24(4):503-4) reported a case of a
perforated acute hydrops in a mentally retarded patient that
was successfully managed with intracameral SF6 gas and
cyanoacrylate tissue adhesive, thus avoiding the need for an
emergency tectonic keratoplasty. A follow-up examination at 1
month showed a formed anterior chamber with tissue adhesive
in situ and no aqueous leak.

Rajesh Sinha, Noopur Gupta, Namrata Sharma,

Raghav Gupta, Jeewan S Titiyal
Correspondence to: Dr. Rajesh Sinha,
S7, R. P. Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, Ansari Nagar,
New Delhi, India.
E-mail: sinharaj1@rediffmail.com