THIS CASE HIGHLIGHTS the therapeutic benefit of soft contact lenses as adjunct therapy for neurotrophic keratitis (NK) management when other on-label therapies are unavailable, unsuccessful, or unsustainable.

A 63-year-old Caucasian female presented with complaints of blurred vision OS > OD and burning that was more pronounced OD > OS. Her symptoms had worsened over a few years after diagnosis of chronic graft-versus-host disease (GVHD) secondary to a bone marrow stem cell transplant for myelodysplastic syndrome and refractory anemia with excess blasts type 2.

She was seen by multiple specialists but failed several therapies, including cryogenically preserved and dehydrated amniotic membranes, punctal plugs, topical cyclosporine, multiple rounds of topical steroids, compounded albumin 5% drops, and many over-the-counter gels, drops, and ointments.

Her chemotherapy consisted of azacitidine, busulfan, and cyclophosphamide, and other drugs for GVHD prophylaxis including tacrolimus and methotrexate. At her initial exam, she was on ruxolitinib.

Her initial best-corrected visual acuity (BCVA) was 20/25 OD and 20/50 OS with a moderate myopic spectacle correction. Pertinent ocular health findings included 2+ episcleral injection and highly asymmetric 1+ superficial punctate keratitis (SPK) OD and 4+ diffuse SPK OS (Figure 1A). Corneal sensitivity testing revealed significant corneal hypoesthesia, which was much greater in the left eye.

She was diagnosed with stage 1+ NK OS > OD based on the Mackie classification system (Mackie, 1995) and careful consideration was given to the treatment due to her health status. Therapeutic scleral lenses were a consideration, but the patient rejected the idea, and autologous serum was contraindicated due to her hematologic status.

Her new therapy consisted of lifitegrast 5% ophthalmic solution b.i.d., preservative-free compounded loteprednol etabonate 0.5% b.i.d., preservative-free artificial tears four to six times per day, and therapeutic six-night extended wear soft contact lenses in both eyes. She was monitored weekly to replace her lenses and improved by 50% within 2.5 months (Figure 1B).

She was refit into a daily disposable contact lens and vitamin A ointment was added q.h.s. in both eyes. Her ocular surface health, corneal sensitivity, and visual acuity improved, and BCVA reached 20/20 OD and 20/25 OS (Figure 1C).

She now reports all-day comfort with her lenses, but, most important, she has renewed hope as she heads toward remission.

NK is considered a rare orphan disease with a frequency of fewer than 5 per 10,000 individuals, although a 2022 retrospective cohort study found a frequency as high as 14% in patients who had chronic GVHD (Mertsch et al, 2019; Singh et al, 2022). This was a highly asymmetric and rare presentation of NK with many complicating factors.

We must carefully sift through treatment options that best fit each patient’s systemic health, but also those that are sustainable when managing a chronic disease, even if the approach seems unorthodox. CLS

REFERENCES

1. Mackie I. Neuroparalytic keratitis. In Fraunfelder F, Roy F, eds. Current Ocular Therapy. 4th. WB Saunders, Philadelphia. 1995:506-508.
2. Mertsch S, Alder J, Dua HS, Geerling G. [Pathogenesis and epidemiology of neurotrophic keratopathy]. Ophthalmologe. 2019 Feb;116:109-119.
3. Singh RB, Yuksel E, Sinha S, et al. Prevalence of neurotrophic keratopathy in patients with chronic ocular graft-versus-host disease. Ocul Surf. 2022 Oct;26:13-18.