Case of the Month 

March 2021

Case presentation:

Over the period of eight years, four patients with Creutzfeldt-Jakob Disease (CJD) presented at one private hospital group. CJD is a uniformly fatal, rare, degenerative neurological disease affecting the brain, caused by pathological accumulation of a transmissible form of protein called a prion.

In two cases, the Infection Prevention and Control Practitioner (IPCP) was consulted regarding iatrogenic transmission via surgical instruments only once the patient was on the operating table. In one case, The IPCP was only informed about the patient 11 weeks after the last surgical procedure had been performed on the patient.  

It is not the aim of this case study to discuss prion diseases, but to assist IPCPs to safely manage exposed surgical instruments.

Procedures on high-risk tissues include intradural surgery on the brain (including the pituitary gland) and spinal cord, neuroendoscopy, and surgery on the retina or optic nerve.


Case presentation:

A 61-year-old male was admitted for a total hip replacement after a fall resulting in a fractured hip. His previous recent history included depression and social withdrawal, and later, lower limb pain and ataxia. He then developed cognitive difficulties and began consuming alcohol excessively. An MRI scan showed cerebral atrophy and he was admitted with a diagnosis of Korsakoff’s syndrome.

The patient underwent a total hip replacement procedure. Post-operatively, his mental status deteriorated rapidly. Further tests were consistent with an encephalopathy process and cerebro-spinal fluid was sent to the CJD Unit in Edinburgh.

The patient deteriorated further and returned to theatre for a gastroscopy, colonoscopy and insertion of a PEG tube.

He was discharged to a special care unit.

Four weeks later, a report was received that suggested the patient was most likely suffering from CJD. Five months later, the diagnosis was confirmed.


What is Creutzfeld Jakob Disease?


CJD is a uniformly fatal, rare, degenerative neurological disease affecting the brain, caused by pathological accumulation of a transmissible form of protein called a prion. 


What is the risk of transmission by surgical instruments?


Procedures on high-risk tissues include intradural surgery on the brain (including the pituitary gland) and spinal cord, neuroendoscopy, and surgery on the retina or optic nerve.


A team was formed to review this case, and various expert bodies were consulted, including the NICD Outbreak Response Unit, an ethicist, the UK National CJD Research & Surveillance Unit and decontamination experts in South Africa and the United Kingdom.  

A risk assessment was done for instrumentation management:

Table 1: Risk assessment

Since it was concluded that there was little to no risk from the hip replacement loan set, it was returned to use; however, the highest risk instrument – the Stryker saw – was condemned as it was the most complicated to safely clean, being an electrical instrument. The gastroscope and colonoscope were old and near the end of their lifespan and it was decided to condemn and replace both. The surgical instruments used for the PEG tube insertion were also condemned as lymphoid tissue was implicated, which is classified as “lower-infectivity tissue.’’ There were 2 x 25 litre containers of instruments, including the Stryker saw.

The process included:

  • Full documentation of every step.
  • Counselling of theatre and Sterile Services Department (SSD) staff on occupational risk of transmission while cleaning the affected instruments, the processes and personal protective equipment to be worn.
  • Incineration of the instruments was considered, being the most effective method of decontamination according to the World Health Organization (WHO). (WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies, 2003). However, the incinerator management advised that although CJD instruments can be incinerated at 1000 degrees Celsius, the instruments that were not destroyed in the incineration process due to their high melting point would most likely cause the ash discharge chute to block.
  • It was decided to use option 4 in the SHEA Guideline 2010:
  • Option 1. Autoclave at 134°C for 18 minutes in a prevacuum sterilizer.
  • Option 2. Autoclave at 132°C for 1 hour in a gravity displacement sterilizer.
  • Option 3. Immerse in 1 N NaOH (1 N NaOH is a solution of 40 g NaOH in 1 L water) for 1 hour; remove and rinse in water, then transfer to an open pan and autoclave (121°C gravity displacement sterilizer or 134°C porous or prevacuum sterilizer) for 1 hour.
  • Option 4. Immerse in 1 N NaOH for 1 hour and heat in a gravity displacement sterilizer at 121°C for 30 minutes,then clean and subject to routine sterilization. (W. Rutala et al 2010).
    • All processes were carried out in a separate area of SSD, then packed and labeled clearly and quarantined pending final decision regarding disposal.

  • It was decided, after consultation with a number of healthcare waste contractors, to use the contractor with the most stringent processes. Before collection and transport for eventual encapsulation and burial at a high-hazardous landfill site, the instruments had to be certified as safe. Details were required on the content of the containers, composition of the instruments and disinfection of the products.  Registration details of the collector, transporter and landfill site were supplied.
  • All records were held by the Infection Prevention and Control Practitioner.


The risk of acquiring CJD is said to be about one person in every one million per year worldwide, although, in Europe where surveillance is enhanced, this figure rises to 2.5 per million per year.

Since the discovery of prion diseases, there have been 491 documented incidents of iatrogenic transmission, mostly resulting from prion-contaminated growth hormone (238 cases) and dura mater grafts (238 cases), one of which was described in the South African Medical Journal in 2006. Four cases were reported after gonadotropin treatment, four were transmitted by surgical instruments in the 1950s (UK and France), two by corneal transplant and two by electroencephalogram (EEG) depth electrode. There have been three reports of transmission of variant CJD by blood transfusion, although transmission risk remains theoretical to date.

Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern.

(D. Bonda et al, 2016) 

Abnormal prions are unusually resistant to conventional chemical and physical decontamination methods, including high doses of ionizing and UV irradiation. Prions remain stable at a wide range of pH, are hydrophobic and resistant to most disinfectants. Of all micro-organisms, prions are the most resistant to disinfectants and sterilants, even more so than bacterial spores.

The probability of a surgical device remaining capable of transmitting disease depends on the initial load of contamination and effectiveness of cleaning, disinfection and sterilization.

Standard, effective cleaning of surgical instruments in the sterile services department results in only a 4 log10 reduction of microbes and about a 2 log10 reduction in protein contamination. Infectivity is stabilized by drying of tissues and by fixing with alcohol, formalin or gluteraldehyde.

Effects of cleaning methodologies on surgical instruments, according to S. Brown et al, 2004, include darkening of instruments, damaging of carbon steel and gold-plated instruments and welded and soldered joints. Damage becomes apparent after the first exposure.


 How would you approach management of instruments in this case?


Complete a risk assessment:


  • Treating physicians to have a high index of suspicion where patients might fit the criteria of diagnosis of CJD.
  • Advise the Infection Prevention and Control Practitioner and the Sterile Services Manager as soon as possible – preferably before any surgery is planned or theatre booking is made.
  • Perform intervention in an operating theatre
  • Perform procedure at end of list – allows cleaning before next list
  • Minimum no. personnel to be involved
  • PPE – liquid repellent gown & mask; goggles & gloves (symptomatic patient – incinerate PPE; “at risk patient” – re-usable PPE may be re-processed)
  • Shield complex instruments – drills and saws
  • Use single use, disposable instruments & equipment where possible and destroy after use, or use the minimum number of instruments possible.
  • Track instruments so they can be traced back to the patient they were used on. Surgical instruments that come into contact with high-risk tissues must not be moved from one set to another and must remain within their individual sets. All endoscopes should have a unique identifier and process register.
  • Re-usable instruments – risk assess. Separate out instruments that came into contact with low infectivity tissue for re-processing and re-use
  • Transport instruments to a previously designated area of Sterile Services Department in a closed container.
  • Process instruments as soon as possible after use – do not allow them to dry out, keep them moist.
  • High risk instruments should be cleaned of gross soil, taking care to avoid aerosols & splashing, under water but with water running and draining out continuously. Use a detergent cleaner with known efficacy against prions.
  • Avoid contact with fixative agents such as alcohol and glutaraldehyde.
  • Air dry then place in impervious rigid container and seal with heavy duty tape and label, including responsible person’s name and list of contents.
  • If the diagnosis of the patient is positive, perform a risk assessment.
  • If the risk is unacceptable, preferably incinerate instruments.
  • Or quarantine high risk instruments for use on same patient if further surgery is likely.
  • If instruments are to be disposed of, contact a reputable healthcare waste contractor. Instruments will have to be further treated before acceptance by the contractor. See different methodologies in the WHO guide: WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies, 2003.
  • Clean contaminated working surfaces with hypochlorite at 10 000 ppm with contact time of 1 hour.
  • Keep records of all decisions. 


What are the lessons learned from this case?


  • Treating physicians to have a high index of suspicion where patients might fit the criteria of diagnosis of CJD.
  • Advise the Infection Prevention and Control Practitioner and the Sterile Services Manager as soon as possible – preferably before any surgery is planned or theatre booking is made.

Recommended reading

  1. WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies: Report of a WHO Consultation, Geneva, Switzerland, 23-26 March 1999 - https://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000_3/en/
    1. Although these guidelines were published about 20 years ago, they remain a valuable source of information for IPC concerns.
  2. Rutala, W., Weber, D. (2010). Guideline for Disinfection and Sterilization of Prion-Contaminated Medical Instruments. Infection Control & Hospital Epidemiology, 31(2), 107-117.
    1. This guideline discusses in detail the decontamination and sterilization of prion-contaminated instruments.
  3. CSSD Forums of South Africa (CFSA) Decontamination and Sterilisation Department Standard Operating Procedures updated 2019 contained in the: APPSA/SafMed. 2020. A Guide to Decontamination, Disinfection and Sterilization Practices. Enquiries re purchasing the guide: contact carma@gonet.co.za
    1. This is a South African document with guidelines on numerous decontamination processes. SOP 52 deals with prion-contaminated instruments.
  4. Centers for Disease Control and Prevention CDC (2020). Prion Diseases. Available from https://www.cdc.gov/prions/index.html [accessed 5.2.2021]
  5. National Creutzfeldt-Jakob Disease Research & Surveillance Unit (NCJDRSU), University of Edinburgh (2017). PROTOCOL Surveillance of CJD in the UK. Version 4, April 2017. https://www.cjd.ed.ac.uk/sites/default/files/NCJDRSU%20surveillance%20protocol-april%202017%20rev2.pdf
  6. Bonda DJ, Manjila S, Mehndiratta P, Khan F, Miller BR, Onwuzulike K, et al. Human prion diseases: surgical lessons learned from iatrogenic prion transmission. Neurosurg Focus 2016;41:E10. - https://www.researchgate.net/publication/304708747_Human_prion_diseases_Surgical_lessons_learned_from_iatrogenic_prion_transmission
  7. Crowder LA, Schonberger LB, Dodd RY, Steele WR. Creutzfeldt-Jakob disease lookback study: 21 years of surveillance for transfusion transmission risk. Transfusion 2017;57:1875e8. - https://onlinelibrary.wiley.com/doi/abs/10.1111/trf.14145
  8. Toovey, S and Hewlett, R. A case of dura mater graft-associated Creutzfeldt-Jakob disease in South Africa July 2006, Vol. 96, No. 7 SAMJ - https://www.ajol.info/index.php/samj/article/view/13794
  9. WHO tissue infectivity matrix - https://www.who.int/bloodproducts/tablestissueinfectivity.pdf
  10. Brown, S.A., Merritt,T., Woods, T.Q., Busick, D.N., Effects on Instruments of the World Health Organization–Recommended Protocols for Decontamination after Possible Exposure to Transmissible Spongiform Encephalopathy– Contaminated Tissue. United States Food & Drug Administration. Published online 24 September 2004 in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/jbm.b.30125 - Available at: https://www.cdc.gov/prions/cjd/pdfs/effects-instruments-who-protocol-508.pd

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