Making a difference in recurrent erosion syndrome

One of my favorite diagnoses is recurrent erosion syndrome (RES) since patients with recurrent erosions are some of the most satisfying (for the patient and for me) to treat. The majority of patients I see with RES have already been accurately diagnosed by their referring doctor. Patients typically have the classic symptoms of foreign body sensation or frank eye pain occurring suddenly in the middle of the night or upon awakening in the morning. The pain can last from seconds to minutes to hours, depending on the amount of corneal epithelial disruption. Patients often have obvious anterior basement membrane dystrophy (ABMD) in the affected eye or a history of corneal trauma, most commonly from a fingernail, tree branch or paper edge. Much less commonly, patients may also have a readily apparent corneal problem, such as Reis-Bücklers or lattice corneal dystrophy, as a reason for their erosions.

Some patients have a less revealing history and few prominent clinical findings. Occasionally, patients may not recall the corneal trauma initially and may need to be asked specifically about any ocular injury. Additionally, the traumatic incident may not be all that recent. On slit lamp examination, the findings can be quite subtle. Mild ABMD changes are often best appreciated by using a wide slit beam angled from the side. Fluorescein dye may reveal areas of mild negative staining, highlighting regions of ABMD or areas of recent epithelial loosening. Using retroillumination through a dilated pupil, faint epithelial irregularities may become more apparent. Careful examination of the fellow eye may also demonstrate ABMD changes.

Once the diagnosis is made (or highly suspected), I discuss the condition and treatment options with the patient [1,2]. I use the analogy of the wallpaper not sticking appropriately to the wall; I tell patients that at night-time the eyelid acts like a hand grasping the wallpaper and either loosening it, and causing pain for a few seconds to minutes, or actually tearing it off the wall, causing pain for hours to days, depending on the extent of the damage.

Often, at this point in the conversation, the patient tells me that they are not terribly affected by the discomfort or pain, but more so by the unpredictability of the symptoms. They say that if they were told that every Monday morning they would have eye pain for 4 hours, then they could adjust their lives accordingly. However, the symptoms may incapacitate them on the morning of an important meeting or the weekend of a friend’s wedding or in the middle of a family vacation. It is the disruption of their daily lives that is most distressing and demoralizing. I empathize with them and tell them there are several very good options to treat their condition and I am confident we can significantly improve their symptoms, if not eliminate them completely.

I then discuss the stepwise approach to treatment and run through the management options. I begin with the first option; lubrication with artificial tears, or hypertonic saline drops, gels or ointments, explaining that they act as a gel barrier preventing the eyelids from ‘grabbing’ the surface layer of the cornea. Some patients have lubricating drops at their bedside, which they instill in the eye with the erosions and let the drops seep in before they open the eye when they wake up in the morning. The lubrication breaks the ‘adhesion’ of the eyelid and cornea and prevents painful episodes. I am amazed that patients can actually do this, but I have heard the same story multiple times. By the time patients are referred to me, however, they have often tried aggressive lubrication without great success.

The second option is a bandage soft contact lens (BSCL), which serves as an adhesive bandage protecting the surface epithelium. While acceptable as a short-term solution, for example to function at work for a day or two, I do not think extended-wear contact lenses are practical in the long term and certainly not for the 3–6 months I believe they need to be used to treat recurrent erosions successfully.

The third option is anterior stromal micropuncture, which I consider quite effective for localized epithelial abnormalities outside the visual axis. While some surgeons feel comfortable performing anterior stromal micro-puncture over large areas of the cornea, including the visual axis, I do not (unless I am trying to scar the cornea in a patient with painful bullous keratopathy who is not a candidate for a corneal transplant). I find anterior stromal micropuncture most applicable in patients with post-traumatic recurrent erosions outside the visual axis. At the slit lamp, I use a 23 or 25 needle to create multiple corneal punctures 20–25% depth in the area of loose epithelium and the surrounding 1 mm of normal cornea. The procedure can also be performed in areas with an epithelial defect by simply puncturing the bare stroma. Care must be taken to ensure that the patient’s forehead is against the band of the slit lamp during the entire procedure to avoid punctures that are too deep or full thickness. When performed correctly, anterior stromal micropuncture is successful in approximately 90% of cases.

Although I discuss the fourth option, epithelial debridement, I do not perform it anymore as I was disappointed with the high recurrence rate of the erosions after this procedure. It involves simply wiping away the loose epithelium with a cellulose sponge or blunt or sharp blade and allowing the epithelium to heal in the hopes that it will adhere better this time. Unfortunately, it often does not.

My favorite treatment (the fifth option) is epithelial debridement with diamond burr polishing of Bowman’s membrane. Returning to the analogy, I tell patients that this procedure is like removing the damaged wallpaper and sanding away the old glue that is not allowing the new wallpaper to adhere properly. In this procedure, after applying a topical antibiotic, anesthetic and an eyelid speculum, I debride all the loose epithelium with a cellulose sponge and number 15 blade. In eyes with ABMD, the area of loose epithelium is typically much larger than the size of the previous erosions. I then firmly apply a diamond-dusted burr (5 mm diameter burr on a handheld battery-operated motorized handpiece), indenting the cornea. I apply vertical motions over the entire epithelial defect for approximately 5–10 seconds. I am careful to apply the burr uniformly over the entire area to avoid inducing any corneal irregularity.

Postoperative treatment includes a drop of scopolamine 0.25% and antibiotic and either a BSCL or antibiotic ointment and pressure patching. The primary downside of this procedure is postoperative pain, which I manage aggressively with oral narcotics and nonsteroidal agents and frequent ice packs over the eye. Patients are followed-up every few days until the epithelial defect heals. Some patients develop mild anterior stromal haze a few weeks postoperatively, which typically resolves on its own but may be treated with a topical steroid taper over 3–6 weeks. Patients are instructed to use a lubricating gel or ointment every night for 3–6 months to aid healing and permanent attachment of the epithelium. The cornea may take 4–6 weeks to stabilize and the refraction may shift slightly postoperatively. In my experience, the success rate for this procedure is approximately 90%. Some patients do get occasional ‘episodes’ over the first few weeks after the treatment while the epithelium is ‘sticking’, which are managed with antibiotics and lubrication, but they can still attain long-term success. I tell patients that there is an approximately 1% rate of serious complications, including infection, poor healing and scarring, leading to significantly decreased vision.

The sixth option, phototherapeutic keratectomy (PTK), is very similar to the diamond burr procedure but an excimer laser is used to ‘polish’ the cornea instead of a diamond burr [3,4]. Here, the excimer laser is used to ablate 5–6 µm of Bowman’s layer. I take care to ensure that the laser ablation is uniform over the entire treatment area to reduce the chances of inducing astigmatism. Postoperative management, pain, the number of follow-up visits and the risk of complications are the same as after a diamond burr procedure. The success rate of PTK for recurrent erosions is also in the range of 90%.

I feel that although the diamond burr and PTK procedures have similar success and complication rates, the diamond burr treatment is logistically easier and faster to perform [5]. I can perform a diamond burr treatment in my office almost any day, while I have to schedule PTK procedures at the Laser Center. If a patient develops recurrences of the erosions after a diamond burr treatment, it can be repeated, but I will usually perform PTK, if only to try something different. The diamond burr procedure is also used to treat patients with ABMD, causing decreased vision even without painful erosions [6].

I really enjoy managing patients with recurrent erosions since they can tell I appreciate how devastating this condition can be to their lives. I understand that it is not so much about the pain, redness, swollen eyelids or decreased vision. It is about the sword of Damocles hanging over their heads every night when they go to bed. Will the sword fall during the night and make getting through a normal day difficult or impossible? Equally importantly, I give them hope of significant improvement or even ‘curing’ their RES with one of a number of treatment options. The diamond burr polishing procedure, the one I perform most often, does not come without side effects (mainly pain) but it does have a very good success rate. Patients unfortunate enough to have RES in both eyes do not usually ask me to treat the other eye during the first week or two after the first eye is treated. However, once the pain has resolved and the first eye has healed, as a rule, they are eager to have the second eye treated. We are fortunate that, as ophthalmologists, most of our treatments are successful and most of our patients are satisfied, but, for me it is hard to find patients where we make as big a difference in their daily lives as those with recurrent erosions.