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Reviews

Keratoconus

Gulani Keratoconus patients back in their demanding professions.

Dr. Gulani is a world renowned eye surgeon and teacher sought not only patients but also by fellow eye surgeons for his innovative and time tested outcomes over the last three decades.

We empathize with keratoconus patients that contact us from all over the world in despair since their eye doctors either provided no solution or gave options between contact lenses, cross-linking, and transplants, all of which do not address the “Ask” of the patient, which is “Vision”! Many of these patients lament that they underwent these procedures and are left with no plan for “vision”.

With nearly three decades of experience with cross-linking technologies all over the world, Dr. Gulani does offer it as an important ingredient in the spectrum of keratoconus treatments, but most eye doctors are indiscriminately cross-linking every keratoconus patient with a promise of preventing worsening. This is analogous to putting cement on a bent back (scoliosis) with a promise of preventing further bends in a spine, but why wouldn’t a doctor straighten the spine (keratoconus) first?

As a renowned corneal transplant surgeon himself, Dr. Gulani believes transplants should be the last option for keratoconus patients because not only are they an interventional surgery that involves donor tissue (that can be rejected), but also because it doesn’t lead to vision with freedom from contact lenses or glasses.

Dr. Gulani believes that contact lenses are an important adjunct in the treatment for keratoconus, however putting stable, adult keratoconus patients into contact lenses without trying to correct the keratoconic cornea surgically, would be analogous to putting a brace on a broken bone which though may allow that leg to walk, essentially leaves that leg disabled every time the brace is removed (contact lens removed) while also allowing worsening of the condition to then miss the opportunities for vision corrective surgeries, leading them to the last resort; corneal transplants.

If for some reason you have already undergone cross-linking, a corneal transplant or wear contact lenses, you still don’t need to despair. Though this may decrease your chance of reaching a 20/20 outcome, Dr. Gulani has successfully corrected numerous such keratoconus patients from all over the world.

In this website you shall find what is a hallmark of Dr. Gulani’s practice with surgical skills and drive, patient safety and ethics and a no-nonsense approach in helping Keratoconus patients achieve freedom from their “Vision Imprisonment.” Patient reactions and testimonies with no incentive or scripts, surgical videos for surgeons in selflessly sharing his techniques and medical articles with a dedication to educate.

He approaches Keratoconus  (Keratoglobus and Pellucid Marginal Degeneration) as a refractive disorder and custom-designs surgery uniquely to each patient aiming to make these patients see without glasses or contact lenses keeping corneal transplants as the last resort.

     
     

World renown for his corneal surgical skills and inventor of the super specialty, Corneoplastique, Dr. Gulani is a corneal transplant surgeon but holds that as the last resort. Dr. Gulani teaches this concept with over 20 Keratoconus surgery choices and over 40 combination techniques and technology choices worldwide and has named it as “Think Outside the CONE”

Over the past three decades of seeing patients with keratoconus from all over the world, Dr. Gulani found many instances in which the referring surgeons have done an excellent job in helping their patients, but the patients were still referred to me since they were dissatisfied with the vision endpoint.

In nearly every case, he found that had the surgeon only believed (mindset) that the patients could see 20/20, they could have completed their endeavor rather than reached a mediocre visual endpoint. Most of these surgeons were true heroes but could not collect their trophies due to their presumed notions resulting in limited endeavors. Additionally, in many such cases, Dr. Gulani did not have to undo the surgeons’ work but instead picked up the “baton and took these patients to their vision end zone.”

These referring surgeons also send him pages and pages of topographies and abstract vision tests that had compelled them to believe that each case of keratoconus was basically destined for poor vision and found that Dr. Gulani explaining to them to take the next step was always met with fear of success!

First, let’s recap a few basics. All ocular surgeries should end with the best vision that individual patients can achieve. This concept of using brief, topical, aesthetically pleasing, least interventional, and visually promising techniques singly or in combination is what he has introduced as a super-specialty called Corneoplastique.

Corneoplastique involves the full spectrum of kerato-lenticulo-refractive surgery to include all levels of LASIK, cataract, and corneal surgery to manipulate the optical elements of the eye singly or in a staged fashion and results in a final emmetropic outcome. The backbone of this thought process and surgical planning is based on the 5S system (Dr. Gulani likens this system to a mental sorter).

Success in ophthalmology does not mean merely stopping progression of the patient’s disease or execution of surgical acrobatics: If the eye has the potential for vision, then it is the surgeon’s job to retain that vision or even to achieve better vision regardless of how complex the starting point. As long as there is no ongoing disease or irreversible blindness, every eye deserves unaided emmetropia.

Keratoconus is a word that creates immediate gloom for the patient and a lowered visual set-point for the diagnosing surgeon. It is a diagnosis that when heard by the patient, makes them immediately anxious to get onto Google, find out everything about the diagnosis, and then quickly come to a conclusion of two choices: an interventional transplant surgery or uncomfortable contact lenses, which either way provides less-than-perfect visual endpoints.

The surgeon in the meanwhile starts to think of all the latest and greatest advances in corneal transplant surgery that they could now use for yet another case and completely misses the point that in many cases of keratoconus, the patient could actually see and see even without glasses or contact lenses-with their own keratoconic cornea!

To avoid this knee-jerk reaction, Dr. Gulani teaches fellows and visiting surgeons to “break down the giant into a smaller, surmountable dwarf” and then confidently create a plan of attack, straight to emmetropia. In breaking down this giant, he helps them visualize most Keratoconus cases as nothing more than a thin cornea with high keratometry, decentered apex, irregular astigmatism, and associated ammetropia (myopia or hyperopia). By breaking down the disease into its smaller components, it becomes conquerable. Now take this mindset further and actually imagine that these patients should deserve refractive surgery candidate expectations.

As proposed previously, size down any complications by sorting through the 5S system to break them down into simple components of visual impact and see your plan of action unfurl before your eyes so this so-called complex condition will logically and elegantly sort itself out into simplistic components that adversely affect vision and present themselves to be corrected in a single or staged fashion.

Consider, for example, the case of a patient with keratoconus with a corneal thickness of 450 µm and a documented cone on topography with low myopia (1 diopter or less) and high astigmatism (up to 5 D). In terms of the 5S system, we first want to make sure that he has potential to see, i.e., sight (visual acuity [VA]). Although the patient cannot tolerate hard contacts, the optometrist has noted that he has a VA of 20/25 with them.

Therefore, whatever we do, the patient should still have a VA of 20/25 or better. The patient has sight, there is no scar, and the site (center or periphery) is unaffected. Moreover, we know the patient has a relatively thin cornea (strength) and high astigmatism (shape). In terms of the 5S system, we must therefore correct for sight and shape.

Now using our Gulani-Nordan criteria for laser PRK candidacy, I would review that approach in this case and also categorize with patient using the following classification.

I. Laser as primary treatment (In this subset, the patient is informed that we can strive for vision directly with the laser keeping the surgical interventions noted in level II as a backup plan to be applied in single or combined approaches to address any complication induced or progression of cone if needed).
Class I: Clear cornea
Class II: Scarred cornea

II. Laser as staged secondary treatment
Class I after corneal surgery:
a. Intacs (Addition Technology, Inc.)
b. Lamellar keratoplasty (femtosecond laser, manual)
c. Penetrating keratoplasty
d. Collagen crosslinking
e.  Conductive keratoplasty

Class II after intraocular surgery
a. Phakic implant (anterior, posterior)
b. Cataract surgery with lens implant (monofocal, toric, accommodative, piggyback)

In this case, the corneal strength is adequate and there is no scar, and therefore no need for lamellar keratoplasty. Continuing with this example, I would perform laser advanced surface ablation (ASA) surgery, because astigmatic treatments remove the least tissue and correct the astigmatism to bring the  VA close to 20/20. From a medicolegal standpoint, I would explain to the patient that, with this technique, I should be able to reshape the cornea to provide predictable vision. I would remind them that the outcome would not be like that of a virgin eye with 20/20 VA. I would also inform the patient that I have Intacs as a backup option if their keratoconus progresses naturally or because of the laser.

Dr. Gulani also explains the use of corneal collagen crosslinking (CXL) as a staged part of any of these surgeries, because it can stabilize this new shape (following laser vision surgery). He teaches surgeons that they should first correct the scoliotic spine (corneal shape = vision) before making the shape permanent with CXL unless the cornea is changing enough to justify CXL before any refractive correction.

Now let’s look at the same case and consider that it has a thinner cornea with a best-corrected VA less than 20/40. Now I would choose Intacs surgery.
The most common Intrastromal Corneal Ring Segments (ICRS) are Intacs, which are semicircular inserts made of polymethylmethacrylate.

Dr. Gulani tells patients that having this kind of keratoconus is like being 11 feet tall. People of normal height go to the mall and buy a suit based on their measurements. He says, he is going to put you in braces (Intacs) and make you 6’ 5” or 5’ 8”. Dr. Gulani doesn’t know for sure because Intacs are not mathematically predictable, but he knows that you will be moving in the right direction, and the chances are that you will be able to walk into a mall and find a suit that will fit, i.e., Intacs help get patients back into contact lenses and glasses. Dr. Gulani performs Intacs in various forms, single, paired, steep axis, and varied various thicknesses and sizes to customize each shape effectively.

Dr. Gulani can also perform Laser ASA over the Intacs to treat residual astigmatism. Again, astigmatic laser procedures remove the least tissue, and he has achieved stability and safety with the braces (Intacs) in place. Crosslinking also provides a stability factor which can be timed accordingly.

Now let’s add a scar to this same patient and using our 5S system we now need to clear the scar while correcting the shape Keeping our in-cornea and on-cornea scar approach, we shall proceed with Laser PRK and use the above-mentioned protocol.

Now let’s keep scar and also make the cornea thinner and more unstable. Based on the 5S system, Dr. Gulani now needs to not only clear the central scar but also add (Strength, i.e., thickness) Such a patient therefore can undergo a variety of lamellar corneal transplants to address these affected “S” systems. Six months to 1 year later, we can perform laser ASA for the refractive error (correcting Shape) aiming for an emmetropic outcome.

In many cases, high myopia could be the main culprit along with astigmatism and here, depending on the age of the patient, he or she can undergo a phakic implant surgery such as Implantable Contact Lens (ICLs) or if in the cataract age group, cataract surgery with monofocal or toric intraocular lenses (IOLs).

The recent FDA approval of CXL is a boon to patients with keratoconus. Dr. Gulani believes that crosslinking could become the final step in every keratorefractive surgery to stabilize and make their outcomes permanent.

CXL also can be performed before refractive surgery in cases of fluctuating refractions/topographies, and once the cornea has become stable and measureable, refractive approaches can be planned.

CXL consists of application of riboflavin (vitamin B2) to saturate the corneal stroma followed by activation with ultraviolet (UV) light. The resulting photochemical reaction causes collagen to form additional covalent connections between fibers, strengthening the collagen structure within the corneal stroma.

An exciting new development in the pipelines is a system by Avedro, which can deliver UV light to the cornea in different patterns and shapes. This system can be used to perform photorefractive intrastromal crosslinking (PiXL). PiXL has the potential to change corneal curvature to achieve a refractive effect. This has advantages over excimer laser treatment because it works without removing tissue and could be that modality I have been manually combining for over a decade.

In conclusion, using the 5S system, we can approach practically any ocular situation from virgin refractive errors to keratoconus. Once we develop the mindset of looking at keratoconus with a new level of commitment and expectations, we can start designing vision with the same responsibility and passion as we do in refractive surgery cases.
By first working with the patient’s optometrist, I can get a picture of the patient’s refractive stability and history and his or her visual potential from a hard contact lens trial. It is then Dr. Gulani’s job to maintain that level of vision or improve it.

Of course, there will be cases with extreme levels of keratoconus that require a corneal transplant. However, even when performing a transplant, surgeons should not forget to take the patients to their next level of vision in a staged fashion.

Approaching keratoconus as a refractive surgery will change the way both ophthalmologists and patients will approach this surmountable condition to bring in a new era of not only relieving but also enhancing the lifestyle of this deserving patient population.

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