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Background: Sporadic Creutzfeldt–Jakob disease (sCJD) is potentially transmissible to humans. Objective: This study aimed to summarise and rate the quality of the evidence of the association between surgery and sCJD. Design and methods: Firstly, we conducted systematic reviews and meta-analyses of case–control studies with major surgical procedures as exposures under study. To assess quality of evidence, we used the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. Secondly, we conducted a systematic review of sCJD case reports after sharing neurosurgical instruments. Results: Thirteen case–control studies met the inclusion criteria for the systematic review of case–control studies. sCJD was positively associated with heart surgery, heart and vascular surgery and eye surgery, negatively associated with tonsillectomy and appendectomy, and not associated with neurosurgery or unspecified major surgery. The overall quality of evidence was rated as very low. A single case–control study with a low risk of bias found a strong association between surgery conducted more than 20 years before disease onset and sCJD. Seven cases were described as potentially transmitted by reused neurosurgical instruments. Conclusion: The association between surgery and sCJD remains uncertain. Measures currently recommended for preventing sCJD transmission should be strongly maintained. Future studies should focus on the potential association between sCJD and surgery undergone a long time previously.


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