Boston type I keratoprosthesis for treatment of gelatinous drop-like corneal dystrophy after repeated graft failure

Semin Ophthalmol. 2015 Mar;30(2):150-3. doi: 10.3109/08820538.2013.833266. Epub 2013 Oct 11.

Abstract

Purpose: 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.

Keywords: Boston type I keratoprosthesis; gelatinous drop-like corneal dystrophy; genetic analysis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amyloidosis, Familial / genetics
  • Amyloidosis, Familial / physiopathology
  • Amyloidosis, Familial / surgery*
  • Antigens, Neoplasm / genetics
  • Bioartificial Organs*
  • Cell Adhesion Molecules / genetics
  • Consanguinity
  • Corneal Dystrophies, Hereditary / genetics
  • Corneal Dystrophies, Hereditary / physiopathology
  • Corneal Dystrophies, Hereditary / surgery*
  • Corneal Transplantation
  • Female
  • Graft Rejection / diagnosis
  • Graft Rejection / surgery*
  • Humans
  • Lens, Crystalline / surgery
  • Mutation
  • Prostheses and Implants
  • Prosthesis Implantation*
  • Visual Acuity / physiology

Substances

  • Antigens, Neoplasm
  • Cell Adhesion Molecules
  • TACSTD2 protein, human

Supplementary concepts

  • Corneal dystrophy, gelatinous drop-like