Fig. 13.1
Image of a patient’s well-fit scleral lens
When initial topical therapy and lid hygiene management cannot adequately relieve a patient’s symptoms, gas-permeable scleral lenses are an option to improve the ocular surface, provide symptomatic relief of dry eye symptoms, and improve visual acuity [1]. The large diameter of the device covers the ocular surface and bathes the cornea and conjunctival tissue in a fluid reservoir. This environment promotes healing of the surface epitheliopathy while improving pain and photophobia associated with chronic ocular GVHD [2].
Graft-versus-host disease (GVHD) describes inflammatory manifestations that occur after transplant when there is activation of donor T cells causing an extensive inflammatory reaction often affecting multiple organ systems. GVHD can be described as acute, occurring within the first 100 days of transplant, or chronic. Acute GVHD most often involves the mucous membranes of the mouth and intestinal tract, liver, and skin. Chronic GVHD occurs in 60–70% of patients [3].
Ocular manifestations of GVHD are commonly relapsing and remitting and affect an estimated 25% [4] to 50% [5] of individuals with systemic GVHD. One of the most common ocular sequelae, as seen in our patient, is keratoconjunctivitis sicca . This complication has been reported to occur in 53% of individuals with chronic GVHD [6]. Lacrimal gland dysfunction causes severe aqueous deficiency and is usually complicated by meibomian gland dysfunction . Additional findings can include scleroderma-like eyelid changes, filamentary keratitis, and corneal ulcers [7].The patient was evaluated and fit with 18.2 mm diagnostic scleral lenses in office. His comfort and lens fit were evaluated after a settling period of 30 min. The first set of lenses were ordered.
What Are the Key Components for Evaluating the Fit of a Scleral Lens or PROSE Device?
First, it is essential to determine if the lens completely clears and vaults over the cornea. If there is complete corneal clearance, next evaluate the limbal area to ensure limbal clearance. If central or limbal touch is suspected, fluorescein dye can be applied to the bowl of the lens, and a thin, white light optic section can help identify areas of touch. Lastly, evaluate the haptic portion of the lens that rests on the sclera. The haptic should not compress the blood vessels and should align with the sclera avoiding both edge impingement and excessive edge lift. Once appropriate corneal vault is achieved, a spherical over-refraction should be performed to obtain best corrected visual acuity.
The patient returned 2 weeks later and was trained on safe lens handling and instructed to use preservative-free saline to fill the bowl of the lens. Visual acuity was 20/20 OD/OS, and he reported immediate relief in his dry eye symptoms.
Therapeutic scleral contact lens fits have proved efficacious at improving dry eye symptoms with a high rate of continuation of scleral lens wear in patients with GVHD (90%) over an average of 32 months [8].
What Are Some Special Considerations with Scleral Lenses in Patients with Severe Ocular Surface Disease?
Patients with OSD should be monitored closely. We typically schedule follow-up visits 1 week after the initial lenses are dispensed and then every few weeks as needed. Patients may need to be seen more frequently if they experience issues with lens application and removal or require many lens modifications to improve fit. At each visit, we measure visual acuity, evaluate the fit, and ensure there is adequate corneal and limbal clearance. We also monitor the conjunctiva for areas of compression, stain, or signs of haptic tightness. These issues could cause inadequate tear film exchange and lead to corneal edema or neovascularization from hypoxia. Topical therapies, including immunosuppressive therapies or serum tears, may also be tapered or eliminated in some patients.
It should be emphasized that only non-preserved solutions should be used to fill the bowl of the lens. We recommend prescribing 0.9% inhalation sodium chloride solution available in 3 mL or 5 mL sterile unit vials. Another option is preservative-free buffered ophthalmic saline that is available in a larger 4 ounce bottle. In general, topical medications should be used without the scleral lens in place. If you prescribe medications to be placed directly in the bowl of the device, care should be taken to use preservative-free formulations. If the patient is using autologous serum tears, we generally encourage them to continue their use before and after lens wear or overtop of the lens. In some circumstances, you may recommend they place a drop of serum tears directly into the bowl of the lens before application; however, you should monitor for complications or infection.
Despite having a good scleral lens fit, patients with severe ocular surface disease often experience issues with lens fogging . The fogging can be due to poor surface wetting or buildup of debris in the fluid under the lens. This can contribute to progressive blurring of the vision after a few hours of lens wear. If the fogging is related to the lens surface, the patient can increase the frequency of artificial tears or remove and reapply the lens. A trial of off-label Mucomyst (10% N-acetylcysteine) eye drops may be tried to inhibit mucus buildup on the surface. Clouding of the tear film reservoir can be seen in patients with scleral lenses that have areas of corneal touch, excessive edge lift, or, alternatively, tight edges with poor fluid exchange. If fluorescein dye placed on the surface immediately seeps beneath the edge, the haptic can be tightened in that area. Some of these cases may represent inflammatory cells or epithelial cells. We have anecdotally noticed that loose conjunctiva (chalasis) may contribute to the collection of cells and debris in the reservoir.
Discussion
Bandage soft contact lenses can also be considered in the management of chronic ocular GVHD. Russo et al. found that extended wear silicone hydrogel lenses provided symptomatic relief but did not improve the condition of the ocular surface [9]. Another study of extended wear bandage soft contact lenses found improvement in manifestations of ocular GVHD with less punctate epithelial erosions in 58% of patients after 2 weeks [10]. While neither study reported any complications, consideration must be given to the use of prophylactic antibiotics given the risk of microbial keratitis with extended wear soft lens use on an already compromised epithelium (e.g., ofloxacin or polymyxin-trimethoprim 1–2 times a day). We have used daily wear soft lenses successfully in ocular GVHD patients, particularly if they have a history of contact lens wear in the past. Overall, soft lenses improve patient symptoms however seem less effective for improving signs compared to scleral lenses. In our experience, most patients with severe disease are ultimately transitioned to scleral devices.
Consistent with current literature, this patient reported immediate improvement of ocular surface disease symptoms. He felt a relief from burning, less grittiness, decreased eye pain, and decreased foreign body sensation with the gas-permeable scleral lenses. In this particular case, when conventional therapy had failed to alleviate symptoms, the gas-permeable scleral lenses provided much needed relief to the patient while also protecting the cornea and conjunctiva from desiccation and blink-related trauma [2].
Case 2
A 78-year-old male patient was referred due to complaints of contact lens intolerance and decreased lens wear time. He had a history of keratoconus with previous penetrating keratoplasty surgery in both eyes 35 years ago, pseudophakia, ocular hypertension, and dry eye syndrome. He complained of constant, severe, burning eye pain. His ocular medications included fluorometholone 0.1% qd OU, bimatoprost 0.01% qhs OU, timolol qam OS, cyclosporine 0.05% bid OU, and preserved artificial tears q2h.
Best corrected visual acuity with 9.5 mm corneal gas-permeable lenses was 20/20 in both eyes. Vision was correctable to 20/30 in both eyes with a manifest refraction of − 5.25 + 6.00 × 173 OD and − 6.00 + 5.25 × 120 OS.
On slit lamp examination, there was severe lid telangiectasia and gland inspissation with thick, turbid meibum that could not be expressed. There was 1+ diffuse bulbar conjunctiva injection and corneal grafts with inferior ectasia and 1+ punctate staining. The gas-permeable lenses were flat-fitting with central corneal touch and excessive edge lift.
Intraocular pressures were 16 mmHg OD and 15 mmHg and pachymetry was 584 OD and 534 OS. The dilated fundus exam was normal with healthy optic nerves and cup-to-disc ratios of 0.25 in the right eye and 0.3 in the left eye.
Given the Patient’s History of Corneal Ectasia, What Type of Optical Correction Would You Consider?
Spectacles and soft contact lenses are considered; however, they are not ideal for this patient due to high amounts of refractive astigmatism and irregular corneal astigmatism. Gas- permeable lenses are a better option as they mask corneal irregularity and generally provide superior visual clarity.
The decision was made to refit his corneal gas permeable lenses and reduce corneal bearing. We discussed a range of corrective options available including small-diameter gas-permeable, piggyback lens systems , hybrid and large-diameter gas-permeable lenses including scleral lenses, or PROSE devices.
What Recommendations Would You Make for This Patient Based on His Dry Eye Symptoms and Clinical Findings of Meibomian Gland Dysfunction?
The patient was instructed to discontinue all benzalkonium chloride (BAK) preserved artificial tears and instructed to use preservative-free artificial tears. Education on meibomian gland dysfunction and lid hygiene was provided, and warm compresses with lid cleaning were initiated.
At the follow-up visit, he reported clear vision with his new corneal gas-permeable lenses but continued to experience eye and eyelid pain. He described the soreness as severe and constant, occurring with or without lenses on.
In consultation with his cornea and glaucoma specialists, a plan was made to reduce all preserved ophthalmic medications. Humphrey 24–2 visual field exams were full, but given his history of intraocular pressures in the mid-30s, prophylactic therapy was continued. Timolol was changed to a preservative-free formulation and bimatoprost was discontinued. Fluorometholone 0.1% was replaced with preservative-free loteprednol 0.5% (ointment), and preservative-free erythromycin ointment was added for use at bedtime.
If Further Intraocular Pressure Lowering Is Necessary, What Other Alternative Treatments Would You Consider?
A number of topical glaucoma medications are available in preservative-free formulations including timolol, dorzolamide/timolol combination, and prostaglandin analog tafluprost. Additional options such as Alphagan®P with Purite (Allergan, Dublin, Ireland) and Travatan Z® with SofZia (Alcon, Fort Worth, Texas) have a less irritating, vanishing preservative. If additional intraocular pressure lowering is deemed necessary, oral agents, argon laser trabeculoplasty, selective laser trabeculoplasty, or glaucoma surgeries could also be considered.
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