Related Experiment Video
Updated: Jun 16, 2026
Corneal Donor Tissue Preparation for Descemet's Membrane Endothelial Keratoplasty
Published on: September 17, 2014
The Boston keratoprosthesis in 2 patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
Elvin H Yildiz1, Christine G Saad, Ralph Eagle
1Wills Eye Institute, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
Purpose:
To report the results of Boston keratoprosthesis in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).
Case Reports:
Case 1 is a 36-year-old woman with APECED and ocular history of corneal ulcers in both eyes who presented with severe dry eyes. Her ocular examination showed diffuse corneal vascularization and stromal scarring in the left eye. She underwent Boston type 1 keratoprosthesis, cataract extraction, and sulcus intraocular lens implant in the left eye to improve visual acuity. Case 2 is a 49-year-old man with possible APECED and ocular history of phlyctenular keratitis, recurrent corneal erosions, and scarring presented with dense corneal vascularization and scarring in both eyes. He underwent a Boston type 1 keratoprosthesis, clear lens extraction, and posterior chamber intraocular lens implant in the right eye.
Results:
The surgeries were uneventful. On postoperative day 1, visual acuity of 20/40 was achieved in both patients and it remained stable during the 2-year follow-up period. There were no postoperative complications seen in either patient.
Conclusions:
APECED is a rare disorder, which can be associated with bilateral keratitis or diffuse corneal vascularization in addition to systemic findings and ectodermal signs. The Boston type 1 keratoprosthesis is a promising treatment option to improve visual acuity in patients with APECED with corneal involvement.
