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C. G. Saad, B. D. Ayres, E. J. Cohen; The Keratoprosthesis in Patients With Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Invest. Ophthalmol. Vis. Sci. 2007;48(13):1894.
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To review the systemic and ocular findings in APECED and to evaluate the outcome of keratoprostheses in two patients with this disease.
Retrospective chart review of two patients with APECED who underwent Boston keratoprosthesis (Type 1) for ocular involvement of this disease.
Two patients with APECED underwent keratoprosthesis procedures for poor visual acuity secondary to corneal scarring and neovascularization. The first patient had a past ocular history significant for bilateral corneal ulcers. She had undergone bilateral permanent tarsorrhaphies and buccal mucosal grafts. Her BCVA in the left eye was 20/400 due to corneal scarring. The patient underwent an uncomplicated Boston keratoprosthesis. On post-operative day one, her vision improved to 20/50 and has remained stable for eight months. The second patient had a past ocular history significant for phlyctenular keratitis, limbal stem cell deficiency, recurrent corneal erosions and scarring. His BCVA in the right eye was 20/100 with a rigid gas permeable lens. The patient underwent a Boston keratoprosthesis with a post-operative visual acuity of 20/50 that has remained stable for four months. There have been no complications in either patient.
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare disorder with ocular manifestations that include chronic phlyctenular keratoconjunctivitis, photophobia, pain, blepharospasm, lacrimation, dry eye, and corneal ulceration. Patients with this condition are poor candidates for penetrating keratoplasty due to limbal stem cell deficiency and corneal scarring. We present two patients who underwent a Boston keratoprosthesis with significant improvement in visual acuity that has remained stable for several months. The keratoprosthesis is a treatment modality that may be successful for visual rehabilitation in these patients.
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