title: Boston Type 1 Keratoprosthesis for Gelatinous Drop-Like Corneal Dystrophy.
abstract: To report the outcomes of Boston type 1 keratoprosthesis in the management of advanced gelatinous drop-like corneal dystrophy (GDLD).
  
  
  
  A retrospective, noncomparative, interventional case series was conducted at Ramathibodi Hospital, Bangkok, Thailand. Four eyes of three siblings with molecularly and histologically confirmed GDLD from a Thai family underwent an uneventful Boston type 1 keratoprosthesis implantation for visual rehabilitation. Clinical data were obtained from a review of the medical records. Visual acuity, device retention, and postoperative complications were the main outcome measures. The follow-up ranged from 8 to 96 months.
  
  
  
  One eye received keratoprosthesis surgery as a primary penetrating procedure. The other three eyes had the surgery as a secondary procedure after graft failure. Best-corrected visual acuity was favorably improved from counting fingers to 20/25 in two eyes, from hand movement to 20/20 in one eye, and from hand movement to counting fingers at 2 feet in one eye caused by severe amblyopia. The improved vision was maintained for 8 months to 6.2 years after surgery. Postoperative complications included disease recurrence in the donor graft (N = 3), manageable retroprosthetic membrane (N = 3), intraocular pressure elevation responded to antiglaucoma drugs (N = 2), and Pseudomonas keratitis with severe corneal melting requiring device removal (N = 1). All of our patients failed to have a comfortably well-fitting contact lens after surgery.
  
  
  
  Boston type 1 keratoprosthesis could be considered as a reasonable option in the management of advanced GDLD. However, patients remain at risk for sight-threatening postoperative complications as long as the keratoprosthesis is retained. The use of Boston keratoprosthesis implantation needed to be individualized on a case-by-case basis.
authors.affiliation: *MD †MD, PhD Department of Ophthalmology, Ramathibodi Hospital, Mahodol University, Bangkok, Thailand (KL, PJ, PC); and Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan (PJ, TI, SK).
authors.forename: Kaevalin
authors.initials: K
authors.lastname: Lekhanont
authors.forename: Passara
authors.initials: P
authors.lastname: Jongkhajornpong
authors.forename: Pichaya
authors.initials: P
authors.lastname: Chuephanich
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Inatomi
authors.forename: Shigeru
authors.initials: S
authors.lastname: Kinoshita
mesh_terms: D000328:Adult; D028226:Amyloidosis, Familial / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D001187:Artificial Organs; D003315:Cornea; D003317:Corneal Dystrophies, Hereditary / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D011183:Postoperative Complications; D019736:Prostheses and Implants; D019919:Prosthesis Implantation; D012008:Recurrence; D012189:Retrospective Studies; D013785:Thailand; D014786:Vision Disorders / Q000534:rehabilitation*; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult