Risk of Creutzfeldt-Jakob disease transmission by ocular surgery and tissue transplantation

Eye (2009) 23, 19261930

& 2009 Macmillan Publishers Limited All rights reserved 0950-222X/09 $32.00 http://www.nature.com/eye

Web End =www.nature.com/eye

WJ Armitage1, AB Tullo2 and JW Ironside3

Risk of Creutzfeldt Jakob disease transmission by ocular surgery and tissue transplantation

CAMBRIDGEOPHTHALMOLOGYSYMPOSIUM

1Academic Unit of Ophthalmology, University of Bristol, Bristol, UK

2Manchester Royal Eye Hospital, Manchester, UK

3National CJD Surveillance Unit, University of Edinburgh, Edinburgh, UK

Correspondence:WJ Armitage, Academic Unit of Ophthalmology, University of Bristol, Bristol Eye Hospital, Lower Maudlin Street, Bristol BS1 2LX, UK Tel: [phone];

Fax: [phone].

E-mail: mailto:[email protected]

Web End =w.j.armitage@ mailto:[email protected]

Web End =bristol.ac.uk

Abstract

CreutzfeldtJakob disease (CJD) is a rare, fatal neurodegenerative disease that occursin sporadic, genetic, variant, and iatrogenic forms. The transformation of normal prion protein (PrPC) to the abnormal form (PrPSc)is a key step in the pathogenesis of CJD and leads to the accumulation of amyloid and spongiform changes in the brain. The presence of PrPSc in tissue is a surrogate marker for CJD infectivity. Sporadic CJD, whose cause is unknown, is by far the most frequent form with 12 cases per million population occurring every yearFthe genetic formsof CJD are rather rarer. The majority of variant CJD cases have occurred in the United Kingdom, where there have been four reports of transmission of vCJD by blood transfusion. The great majority of iatrogenic transmissions of CJD have resulted from the use of pituitary-derived hormones or dura mater with only a very few cases attributable to neurosurgical instruments or corneal transplants. In the absence of a validated test for CJD infectivity in eye donors, the application of appropriate donor selection criteria and the use of single-use instruments in eye banks are currently the most effective means of reducing the risk of CJD transmission. Onward transmission by reusable ophthalmic surgical instruments has not been reported, but the risk cannot be excluded. Use of appropriate cleaning and disinfection protocols and the ability to identify and quarantine instrumentsthat may have been used on aninfected patient are importantsafeguards.

Eye (2009) 23, 19261930; doi:http://dx.doi.org/10.1038/eye.2008.381

Web End =10.1038/eye.2008.381 ; published online 9 January 2009

Keywords: CreutzfeldtJakob disease; prion disease; cornea; retina; transplantation; surgical instruments

CreutzfeldtJakob disease (CJD) is a member of the family of prion diseases, all of which are associated with the presence of...