Limbal stem cell transplantation for the treatment of subepithelial amyloidosis of the cornea (gelatinous drop-like dystrophy)

Purpose. Subepithelial amyloidosis of the cornea, or gelatinous drop-like corneal dystrophy (GDD), is a severe form of corneal stromal amyloidosis predominantly found in the Japanese population. GDD causes severe visual disturbances with irritating symptoms, and keratoplasty is inevitably complicated by early recurrence. This study was conducted to evaluate the efficacy of limbal stem cell transplantation (LSCT) for the treatment of GDD. Methods. Nine consecutive eyes of seven patients with GDD were treated with LSCT. Limbal grafts were obtained from cadaver eyes. All eyes underwent lamellar or penetrating keratoplasty simultaneously with LSCT. Intensive epithelial management and immunosuppression therapy was performed. Results. With a mean observation period of 4 years, eight (88.9%) of nine eyes did not show any signs of recurrence. Five grafts remained clear, and seven eyes had an improvement in vision. Glaucoma and rejection were observed in five and three eyes, respectively, and two eyes with glaucoma required surgical intervention. Conclusions. Limbal stem cell transplantation is effective for the prevention of recurrence in GDD. Control of intraocular pressure and rejection are the keys to long-term maintenance of clear grafts.