title: Boston type I keratoprosthesis for treatment of gelatinous drop-like corneal dystrophy after repeated graft failure.
abstract: OBJECTIVE
  To describe the use of a Boston type I keratoprosthesis as a secondary penetrating procedure to treat gelatinous drop-like corneal dystrophy (GDLD), with presentation of pathologic findings, genetic analysis, and discussion of other surgical options.
  
  
  METHODS
  A 43-year-old woman with GDLD in both eyes, best corrected visual acuity (BCVA) of counting fingers in both eyes, and recurrent corneal opacification following two penetrating keratoplasties presented for visual rehabilitation. A Boston type I keratoprosthesis was implanted in her left eye after extracapsular clear lens extraction.
  
  
  RESULTS
  The surgery was uneventful and one month after surgery, best corrected vision improved to 20/30, which has been maintained for a period of more than nine months. At the 12-month visit, her vision was noted to be diminished to 20/200 due to a retroprosthetic membrane and improved to 20/25 two weeks after a Yag capsulotomy. Histopathologic examination of the corneal specimen disclosed predominantly subepithelial amyloid deposition. Genetic analysis is presented.
  
  
  CONCLUSIONS
  GDLD is a rare disorder of primary corneal amyloidosis. Recurrence of this condition following surgery is very common. Boston type I keratoprosthesis as a secondary procedure can be successful in restoring vision in affected patients.
authors.affiliation: Beirut Eye Specialist Hospital , Beirut , Lebanon .
authors.forename: A
authors.initials: A
authors.lastname: Fadlallah
authors.forename: F A
authors.initials: FA
authors.lastname: Jakobiec
authors.forename: P R
authors.initials: PR
authors.lastname: Mendoza
authors.forename: P A
authors.initials: PA
authors.lastname: Zalloua
authors.forename: S A
authors.initials: SA
authors.lastname: Melki
mesh_terms: D000328:Adult; D028226:Amyloidosis, Familial / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D000951:Antigens, Neoplasm / Q000235:genetics; D021522:Bioartificial Organs; D015815:Cell Adhesion Molecules / Q000235:genetics; D003241:Consanguinity; D003317:Corneal Dystrophies, Hereditary / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D016039:Corneal Transplantation; D005260:Female; D006084:Graft Rejection / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007908:Lens, Crystalline / Q000601:surgery; D009154:Mutation; D019736:Prostheses and Implants; D019919:Prosthesis Implantation; D014792:Visual Acuity / Q000502:physiology
chemical_list: D000951:Antigens, Neoplasm; D015815:Cell Adhesion Molecules; C499380:TACSTD2 protein, human
keywords: Boston type I keratoprosthesis; gelatinous drop-like corneal dystrophy; genetic analysis