Keratoprosthesis in pediatric keratitis-icthyosiform-deafness syndrome

Abstract

We report the first case of Boston Keratoprosthesis (KPro) implantation in a 7-year-old girl with keratitis-ichthyosiform-deafness syndrome and persistent, highly vascular corneal surface disease. An adult aphakic KPro with 8.5 mm backplate was implanted successfully and without operative or postoperative complications following 3 failed penetrating keratoplasties. Visual acuity improved from hand motions to 20/70 in the left eye 22 months after KPro surgery and from hand motion to hand motion with direction in the right eye. Glaucoma developed in the left eye, but intraocular pressure was successfully controlled with Ahmed valve placement and topical dorzolamide-timolol.