The Boston keratoprosthesis in autoimmune disease

Abstract

Abstract Purpose Patients with corneal blindness due to mucous membrane pemphigoid and Stevens Johnson syndrome who undergo corneal transplantation carry a poor prognosis for visual recovery. The Boston keratoprosthesis has been demonstrated to provide excellent retention rates and postoperative visual acuity in patients with corneal graft failure, however, poor visual outcomes still occur in patients with underlying autoimmune disease. Methods We reviewed the current literature to determine the results of keratoprosthesis in patients with blinding autoimmune diseases. Results Much of the published literature on keratoprosthesis fails to clearly differentiate outcomes on the basis of the underlying disorder. Based on available evidence, inflammation, retinal detachment, and glaucoma appear to be the most significant complications after keratoprosthesis in autoimmune patients, and a diagnosis of mucous membrane pemphigoid or Stevens Johnson Syndrome appears to be associated with a significantly higher complication rate than other preoperative conditions. Conclusion Patients with autoimmune diseases carry the worst prognosis for success with keratoprosthesis. Improvement in clinical outcomes might be achieved with changes in keratoprosthesis design and material, perioperative therapy, and/or surgical technique. Possible approaches to complications after Boston keratoprosthesis in patients with underlying autoimmune diseases will be discussed.