The Boston Keratoprosthesis in 2 Patients With Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy.
Cornea. 2010 Jan 21. [Epub ahead of print]
Yildiz EH, Saad CG, Eagle R, Ayres BD, Cohen EJ.
From the Wills Eye Institute, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA.
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.