Penetrating Limbokeratoplasty for Gelatinous Corneal Dystrophy

Abstract

Gelatinous corneal dystrophy is a very rare disease in Europe and North America. Patients suffer from pain, photophobia and loss of vision, usually before the age of 20. Transplantation of limbal stem cells was proposed due to the high rate of recurrence after conventional penetrating keratoplasty. We present the results of penetrating limbokeratoplasty, a combined transplantation of cornea and limbal stem cells, in patients with gelatinous corneal dystrophy. We present a series of 7 consecutive eyes with gelatinous corneal dystrophy, which were treated with penetrating limbokeratoplasty-4 eyes of 3 patients had already undergone multiple conventional corneal transplantations. We evaluated the follow-up regarding recurrences of the disease, graft rejections, clear graft survival and incidence of secondary glaucoma with the Kaplan-Meier method. Median age at surgery was 35 years. Postoperative treatment included topical steroids and systemic mycophenolate mofetil in all patients. Median graft survival was 6 years. Median time to a recurrence was 3 years. Median time to the first endothelial graft rejection was 2.5 years. Penetrating limbokeratoplasty offers a possibility to restore visual acuity over a long period of time. However, gelatinous corneal dystrophy remains a therapeutic challenge. Even with simultaneous transplantation of cornea and limbal stem cells, recurrences of the disease cannot be prevented permanently. This is also connected with discontinuing a sufficient local and systemic immunosuppression.