The cure that wasnt
Rene E. Reeder, OD, FAAO, FBCLA, FSLS, FIACLE, Diplomate AAO Cornea and Contact Lens
3241 South Michigan Avenue, Chicago, Illinois 60616
BACKGROUND
Over the years many different surgical procedures have
been used in the treatment of keratoconus (KC). The
treatment options include various forms of keratoplasty
cross-linking agent corneal rings and certain types of
refractive surgery.1-7 However most forms of refractive
surgery have fallen out of favor due to the increased risk
of progression, hydrops, blindness, infection and overall
weakness of the cornea.8-12 However in certain regions of
the world radial keratotomy (RK) is making a resurgence
in the management of keratoconus.13 In 2009, circular
keratotomy was recommended to improve vision in
patients with early stages of KC in order to improve vision.
Results were variable with approximately 10 percent of
patients actually having worse vision.4 In 2006, RK was
evaluated in mild to moderate KC as well. In this study 20
percent of patients needed deepening of the incisions
due to progressive astigmatism. An additional ten
percent experienced hydrops, perforation, infection and
infection. While the astigmatism was reduced initially the
cylinder returned after about a year. In January 2016, mini
asymmetric radial keratotomy (MARK) was recommended
with corneal crosslinking (CXL)7 for KC despite previous
cases of RK incisions gaping after CXL.8 We review here a
case of RK performed on a known patient with keratoconus
and the changes that ensued.
CASE
In 2011 an 18-year-old Eastern European male presented
to the cornea center with complaints of blurred vision
in his left eye of about six months duration. He had
previously experienced an episode of hydrops in the left
eye and was only wearing a lens in his right eye. Corneal
topographies (Figures 1a and 1b) and slit lamp confirmed
asymmetric keratoconus worse in the left eye which
also had scarring. (Figure 2) The patients best corrected
vision with spectacles with 20/40 and 20/400, right
and left eye respectively. The patient was initially with
the ComfortKone lens in each eye and while his vision
improved to 20/25 and 20/80 he was unable to tolerate
the left lens. Therefore, we chose to initially piggyback the
left lens but he still reported significant awareness. Next,
a scleral lens was tried. He was fit with a 15.6 Jupiter lens
which provided 20/40 vision but the patient again felt that
it was uncomfortable. Approximately six months later, the
Kerasoft IC was launched and he underwent fitting with
the new soft lens product. He was quite successful with
the lens and achieved 20/50 vision. At his final follow up
with the Kerasoft IC lens he reported that he was returning
to his native Kosovo and we discussed the possibility of CXL
to prevent further progression particularly in his right eye
that was seeing so well. At that time he was lost to follow
up for two and a half years.
Upon his return to the US in 2014, he reported that he had
received surgery to cure his keratoconus while he was
in Europe. Presently he reported glare and monocular
diplopia in the right eye and reduced vision with the left.
(Figures 3a and 3b) He was not wearing contact lenses and
did not know if he was able to after the surgery.
Upon slit lamp examination there were multiple incisions
in each eye extending from the limbus of approximately
3mm in length. (figure 4) Thus, it was apparent that the
surgical procedure that the young man had undergone
was actually RK rather than the CXL we had discussed.
Topographical evaluation showed increased irregularity
of the cornea and progressive thinning. (Figure 5a-5d)
The vision in the right eye while improved, fluctuated
greatly. His best vision was a distorted 20/25. However
on some visits his vision was as low as 20/40. He was
also experiencing significantly more coma in the right
eye which had increased from 2.01 to 3.60 microns.
Unfortunately, the patients distortion and glare complaints
could not be eliminated with any lens design attempted.
The left eye required a scleral lens but now only improved
to 20/60. (Figure 6) Consultation with a corneal OMD
resulted in recommendation of crosslinking in the right eye
and penetrating keratoplasty (PKP)in the left eye.
FIGURE 1A-B: Topographies of the right and left eye,
respectively. Notice the asymmetry between the two
eyes.
Figure 1a Figure 1b
FIGURE 2
Prominent
central
scarring
is seen in
the post-
hydrops left
eye.
DISCUSSION
While RK has been shown to reduce astigmatism and in
early stages of KC improve vision, it is not without risks.
Complications have included continued progression,
hydrops, dehiscensce and perforation.8-12 Steinman et
al reinforced these concerns using a porcine model.
Reporting that MARK yes required 50-70 percent less force
to rupture than in unoperated eyes.11 The risk of rupture in
traditional RK persists with cases occurring as late as 10-13
years after surgery. 9,12
FIGURE 3A-B: Simulated VA charts, post RK for the R and
L eye consistent with patients visual complaints
Figure 3a
Figure 3b
FIGURE 4
OCT image of the left eye showing a 3mm long, deep RK
incision extending from the limbus.
Illinois Eye Institute, Chicago, Illinois
The most recent recommendations for incisional treatment
of KC include MARK with CXL.7,13 The theory is that you
are working at to different levels and thereby enhancing
the outcome of the procedure. MARK is performed with
incisions that are limited to a maximum of 2mm in length
and that occur only within the central 8mm of the cornea
so that they fall within the trefine should the patient
ultimately need a PKP.4,7 The MARK procedure is to reduce
the astigmatism and two years post MARK the patient has
CXL. The goal of CXL to stop progression and possibly
increase flattening at the microscopic level.7 However,
one patient who had undergone RK ten years prior to CXL
experienced incisional gaping which ultimately required
suturing.1 The MARK and CXL procedures are now being
touted on the internet as a cure for KC and are reportedly
available in many countries throughout the world including
Australia, China, France, Germany, Hungary, India, Italy,
Japan, New Zealand, US, Qatar, South Africa, and United
Arab Emirates.13
FIGURE 5A-B: Initial postRK topographies.
Figure 5a
Figure 5b
Our patient underwent traditional RK with long deep
incisions extending to the limbus. As a result, the PKP
recommended for the left eye will require peripheral
suturing prior to trefine and strict monitoring during the
post-operative period. The CXL for the right eye may also
require suturing before during or after surgery.8 Sadly, what
he believed was a cure has put his long term eye health
and vision at significant risk.
FIGURE 5C-D
Consecutive post-surgical difference maps showing
continued progression in each eye after RK.
Figure 5c
in KC that extends throughout the KC cornea.15 Eyecare
MARK. Proper education and referral is crucial. Post-
Figure 5d
7. Abbondanza M, et al. Combined corneal collage cross-linking and mini asymmetric
Figure 6. Best fit scleral OS showing highly asymmetrical
cornea postRK.
CONCLUSION
A variation of RK known as mini asymmetric RK has
emerged in certain European countries where it is being
recommended as an option to correct vision with KC.13
While the incisions in this case do not appear to follow
the mini RK appearance but rather traditional RK scars, in
both cases concerns of weakening of the cornea persist.11
Recent studies show that incisional surgeries result in
persistent weakness and incomplete healing many years
postoperatively.9,12 The latest suggestion is that they
never fully heal thereby creating persistent risk of known
complications including perforations and hydrops. This
may occur as result of fractionation of the lamella as seen
providers need to be aware that this practice is emerging
and patients undergoing these procedures may not be
aware of the risks. Or in the case of our patient may not
understand that there is a difference between CXL and
surgically these patients may require more advanced lens
designs and additional surgical procedures to preserve
their vision. Careful education postoperatively regarding
long term risks and the need for protective eye wear is
essential.
REFERENCES
1. Appioti A. Gualdi M. Treatment of keratoconus with laser in situ keratomileuis,
photorefractive keratectomy and radial keratotomy. J Refract Surg. 1999 Mar/Apr; 15(2
suppl):S240-2042.
2. Colin J, Velou S. Current surgical options for keratoconus. J Cataract Refract Surgery.
2003; 29:379-386
3. Lombardi M. Abbondanza M. Asymmetric Radial Keratotomy for the correction of
keratoconus. J Refract Surg. 1997 May/June; 13:302-308.
4. Krumeich JH, Keziran GM. Circular keratotomy to reduce astigmatism and improve
vision in stage 1 and 2 keratoconus. J Refract Surg. 2009 Apr; 25(4):357-365.
5. Mamalis N, et al. Radial keratotomy in a patient with keratoconus. Refract Cornea Surg.
1991 Sep/Oct; 7:374-376.
6. Utine CA. et al. Radial keratotomy for the optical rehabilitation of mild to moderate
keratoconus more than five years experience. Eur J Ophthalmol. 2006 May/Jun;
16(3):376-364.
radial keratotomy for the treatment of keratoconus. Acta Medica Int 2016 Jan/
June;3(1):63-68.
8. Abad JC. Vargas A. Gaping of radial and transverse incisiuons occuring early after CXL. J
Cataract Refract Surg. 2011;37:2214-2217.
9. Panda A, et al. Ruptured globe 10 years after radial keratotomy. J Refract Surg. 1999 Jan/
Feb;15(1):64-65.
10. Sharma N. et al. Acute hydrops in keratectasia after radial keratotomy. Eye Cont Lens.
2010;3:185-187.
11. Steinemann TL, Baltz TC, Lam BL, Soulsby M, Walls RC, Brown HH. Mini Radial
Keratotomy Reduces Ocular Integrity. Ophthalmology. 1998;105(9):1739-1743
12. Vinger PF, et al. Rupture globes following radial and hexagonal keratotomy surgery.
Arch Ophthalmol. 1996 Feb; 114(2):129-134.
13. http://www.abbondanza.org/eng/mini-asymmetric-radial-keratotomy-keratoconus-
mark/
14. Selver OB, et al. Traumatic wound dehiscence after penetrating keratoplasty. Ulus
Travma Acil Cerahi Derag. 2016 Sep; 22(5):437-440.
15. Matthew JH, et al. Lamellar changes in the keratoconic cornea. Acta Ophthalmol.
2015;93:767-773.
CONTACT INFORMATION
Renee E. Reeder, OD, FAAO, FBCLA, FSLS,
Diplomate AAOCCLRT
RReeder@ico.edu www.ico.edu