Management of oculus fragilis in Ehlers-Danlos type VI

Purpose, To describe an alternative, novel surgical approach to the repair of a ruptured globe in the second reported patient with keratoglobus, Ehler s-Danlos type VI, and normal lysyl hydroxylase. Methods, Under general anes thesia, a 360 degrees conjunctival peritomy was performed and the epitheliu m was removed from the keratoglobus cornea. Descemet's membrane and endothe lium were removed from a fresh donor corneoscleral ring. An onlay epikerato plasty was then performed and the entire donor corneoscleral button was sec ured to the sclera with 9-0 nylon suture. The conjunctiva was brought into position and racked down over the edge of the donor graft. After 4 months, a full-thickness penetrating keratoplasty was then performed. Results. The patient ultimately had a ruptured globe: in the fellow eye and thus require d the identical procedure OU. Both eyes were successfully repaired with a f inal visual acuity OU of 20/100. Conclusion. The described surgical approac h allowed successful surgical repair of bilateral ruptured globes in a pati ent with keratoglobus, Ehlers-Danlos type VI, and normal lysyl hydroxylase levels. In the previous report of such a patient by Judisch et al. (1), the attempt at surgical repair was unsuccessful and ended in enucleation. The described surgical technique may be used to treat advanced keratoglobus or oculus fragilis.