Use of Fluid-Ventilated, Gas-Permeable Scleral Lens (SL) for Management of Severe Keratoconjunctivitis Sicca Secondary to Chronic Graft-Versus-Host Disease.
Authors: Takahide Kikuchi , Parker Pablo Miguel , Wu Michael , Carpenter Paul A. , Stehr Barbara , Rosenthal Perry , Martin Paul J. , Forman Stephen J. , Flowers Mary E.D. 
Published: October 2019

Dry eyes or keratoconjunctivitis sicca (KCS) occur in 50 % of patients (pts) with chronic graft-versus-host-disease (cGVHD) after allogeneic hematopoietic cell transplantation (HCT) and may represent the only, or most significant, clinical manifestation of cGVHD in some pts. Artificial tears, long-acting ocular lubricants, punctal plugs, ophthalmic cyclosporine, topical corticosteroids, moisture chamber eyewear and oral cholinergic agents are the primary supportive therapies of ocular cGVHD. The liquid corneal bandage provided by a fluid-ventilated, gas-permeable scleral lens (Boston Scleral Lens, BSL) has been effective in mitigating symptoms and resurfacing corneal erosions in pts with a variety of causes of KCS refractory to conventional therapies. Here we report outcomes in 9 pts with cGVHD after related and unrelated HCT who had severe KCS treated by fitting a BSL because of debilitating ocular discomfort, visual impairment, or keratopathy despite systemic and local therapies and other supportive care. Diagnoses at time of HCT were CML (n=3), MDS (n=2), AML (n=1), AA (n=1), CLL (n=1) and MM (n=1). Median pt age at the time of SL fitting was 56 (range, 25–64) yrs. All pts had prior cGVHD involving multiple sites that required immunosuppression therapy (IS) with prednisone, +/− calcineurin inhibitors, +/− mycophenolate mofetil or other therapies. Manifestation of cGVHD other than eyes at the time of SL fitting were skin (n=2), mouth (n=5), liver (n=3), GI (n=1), lungs (n=1) and vagina (n=1). At the time of SL, 7 pts were receiving IS and 2 pts had discontinued all IS because of resolution of other manifestation of cGVHD. The response to the SL was assessed by patient-reported ocular disability and use of ancillary eye care, and by use of the Ocular Surface Disease Index (OSDI) in 5 pts. The OSDI is a valid and reliable tool used to assess severity of dry eye symptoms and vision-related function on a 0–100 scale, with higher scores correlating with increasing symptom severity and vision-related disability. Table below presents patients-reported outcomes. The use of the BSL led to ocular improvement in all cases. No serious adverse events or infections attributable to the BSL occurred. The median OSDI available improved from 87.7 to 20.8 within 2 weeks of BSL fitting and was 12.5 (4.2–52.7) at the time of last contact, 8–23 months (median, 15.2) after BSL fitting. Seven pts were no longer disabled by KCS after the BSL. All 9 pts reported reduced use of artificial tears, and 2 of 3 who were using moisture chamber eye glasses pre BSL were able to discontinue them. One pt had significant difficulty inserting the BSL, even with assistance. We conclude that BSL can be a safe and effective procedure for pts suffering from severe cGVHD-related KCS that is refractory to other therapies.

No. of Patients 9 Artificial Tear Use (Times per day, N) - Before fitting Scleral Lens (SL) > 100 - After fitting SL (median, range) 3 (0–50) Punctal Plugs (times replaced, N) - Before fitting SL (median, range) 3 (1–>6) - After fitting SL 1 (1 pt) Subjective Assessment of Disability - Before fitting SL Yes (7 pts) - After fitting SL Yes (1 pt)