Boston Type I Keratoprosthesis for Treatment of Gelatinous Drop-Like Corneal Dystrophy After Repeated Graft Failure.

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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 .