title: 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.
authors.affiliation: Department of Ophthalmology and Visual Sciences, Yale School of Medicine, New Haven, Connecticut. Electronic address: Cortlyn.brown@yale.edu.
authors.forename: Cortlyn
authors.initials: C
authors.lastname: Brown
authors.affiliation: Department of Ophthalmology and Visual Sciences, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Megan
authors.initials: M
authors.lastname: Rowlands
authors.affiliation: Department of Ophthalmology and Visual Sciences, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Daniel
authors.initials: D
authors.lastname: Lee
authors.affiliation: The Eye Care Group, New Haven, Connecticut.
authors.forename: Joel A
authors.initials: JA
authors.lastname: Geffin
authors.affiliation: Department of Ophthalmology and Visual Sciences, Yale School of Medicine, New Haven, Connecticut.
authors.forename: John
authors.initials: J
authors.lastname: Huang
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D001187:Artificial Organs; D001705:Bioprosthesis; D002648:Child; D003131:Combined Modality Therapy; D003315:Cornea; D003638:Deafness / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005901:Glaucoma / Q000209:etiology / Q000628:therapy; D020327:Glaucoma Drainage Implants; D006801:Humans; D007057:Ichthyosis / Q000503:physiopathology / Q000601:surgery*; D007429:Intraocular Pressure; D007634:Keratitis / Q000503:physiopathology / Q000601:surgery*; D019919:Prosthesis Implantation; D014786:Vision Disorders / Q000503:physiopathology / Q000601:surgery*; D014792:Visual Acuity / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents