Long-Term Outcomes of Boston Type I Keratoprosthesis After Minor Salivary Gland Transplantation and Labial Mucous Membrane Grafting as an Alternative Treatment for Stevens-Johnson Syndrome.

PURPOSE

Total limbal stem cell deficiency (LSCD), severe dry eye disease (DED), and ocular surface keratinization are severe ocular complications of Stevens-Johnson syndrome (SJS) that can be difficult to manage, resulting in poor visual outcomes. Several ocular surface reconstruction and visual rehabilitation techniques have been attempted with no satisfactory outcomes to date. Our purpose is to assess the functional and anatomical outcomes of Boston keratoprosthesis type I (Kpro-I) after minor salivary glands transplantation (mSG) and labial mucous membrane (MMG) grafting in patients with SJS suffering from total LSCD, DED, and ocular surface keratinization.

METHODS

This is a retrospective multicenter case series from 2 tertiary referral centers (New England Eye Center, Tufts Medical Center, Boston, Massachusetts and Federal University of Sao Paulo) assessing long-term outcomes of patients with SJS with severe ocular complications who received mSG/MMG grafting before Kpro-I implantation, including best-corrected visual acuity, Kpro-I device retention, and postoperative complications.

RESULTS

Three patients with SJS with severe ocular complications (total LSCD, symblepharon, DED, and ocular surface keratinization) were treated with mSG/MMG grafting, followed by Kpro-I. Ocular surface keratinization was ameliorated in all patients after mSG. At the end of the long-term follow-up period, all patients retained the Kpro-I (33-63 months) and achieved improved visual acuity (20/40, 20/80, 20/100). Complications included glaucoma (n = 1), requiring a glaucoma drainage device; peripheral corneal thinning (n = 2), which was treated with a corneal patch graft; postoperative infectious keratitis (n = 1); cystoid macular edema (n = 1); and retroprosthetic membrane (n = 1), which was successfully treated.

CONCLUSIONS

mSG/MMG grafting can optimize the ocular surface to allow for successful Kpro-I in patients with severe SJS, providing an alternative approach to Boston type II Kpro.