Creutzfeldt-Jakob disease following corneal transplantation

OBJECTIVE: To report a case of possible iatrogenic Creutzfeldt-Jakob many years following penetrating keratoplasty. DESIGN: Observational case report. METHODS: We reviewed the patient's chart, available literature on Creutzfeldt-Jakob disease, and current recommendations from the Eye Bank of America Association. RESULTS: An 80-year-old woman with a history of granular dystrophy underwent penetrating keratoplasty in 1978 (OS) and 1987 (OD) for decreased visual acuity. In early July 2002 the patient developed rapidly progressive dementia. Attempts were made to procure information regarding the corneal donors. No information was available. The patient expired slightly less than 1 month after her onset of symptoms. Review of the EBAA medical standards before October 2002 reveal that donor records were to be kept for a minimum of 10 years. CONCLUSION: Creutzfeldt-Jakob disease occurred 15 to 24 years after bilateral penetrating keratoplasy, but donor-to-host transmission was uncertain because no information on the corneal donor could be retrieved. Eye-bank records should be kept indefinitely because CJD may occur decades after iatrogenic exposure.