A Contemporary Risk Analysis of Iatrogenic Transmission of Creutzfeldt-Jakob Disease (CJD) via Corneal Transplantation in the United States

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a rare, fatal, neurodegenerative prion disease potentially transmissible through corneal transplantation. While statistical analyses performed two decades ago estimated the overall prevalence of CJD in the corneal donor pool to be low, the recent significant increase in corneal Digital Features To view digital features for this article go to

National Consensus on Contraindications for Corneal Donation for Transplantation in Switzerland

“…Three of the six recipients died, one survived with the liver graft (due to already positive rabies antibodies and corresponding positive vaccination history), and both recipients of the corneal grafts had to be emergently explanted 11 . Indeed, keratoplasty-associated transmissions also occur in other infectious diseases such as Creutzfeldt-Jakob, rabies, hepatitis B, or herpes viruses 12 , 13 , 14 , 15 . Further, very few cases of acquired malignancies associated with keratoplasty have also been described 16 , 17 , 18 , 19 .…”

The Great Masquerade: Donor-derived Infections with Uncommon Central Nervous System Pathogens

“…Transmissible spongiform encephalopathy (TSE) is a general term for prion-related diseases caused by the misfolded proteins, which became well known in the 1980s due to a sudden increase in the incidence of bovine spongiform encephalopathy (BSE) or mad cow disease [66]. Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) (i.e., BSE in humans) are rare, uniformly fatal, transmissible, neurodegenerative prion diseases with no known treatment [66,67]. Clinically, these diseases are characterized by progressive dementia and neuron loss, with an average latency period of 10 years [67].…”

“…Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) (i.e., BSE in humans) are rare, uniformly fatal, transmissible, neurodegenerative prion diseases with no known treatment [66,67]. Clinically, these diseases are characterized by progressive dementia and neuron loss, with an average latency period of 10 years [67]. While the majority of CJD has been acquired through inheritance or sporadically, numerous reports have documented its transmission through contaminated tissues, such as dura mater grafts [68] and corneas [67], among others.…”

“…Clinically, these diseases are characterized by progressive dementia and neuron loss, with an average latency period of 10 years [67]. While the majority of CJD has been acquired through inheritance or sporadically, numerous reports have documented its transmission through contaminated tissues, such as dura mater grafts [68] and corneas [67], among others. Between 1974 and 2006, ten cases of corneal transplant-transmitted CJD have been reported [67].…”

“…While the majority of CJD has been acquired through inheritance or sporadically, numerous reports have documented its transmission through contaminated tissues, such as dura mater grafts [68] and corneas [67], among others. Between 1974 and 2006, ten cases of corneal transplant-transmitted CJD have been reported [67]. Rare reports of possible SOT-transmitted CJD have been published, including a liver recipient who died of CJD two years after transplantation [69] and a kidney recipient who died with CJD [70].…”