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Porter et al¹ assess the potential for iatrogenic transmission of the rare but devastating Creutzfeldt-Jakob disease (CJD) through surgical procedures prior to CJD diagnosis. The authors performed a retrospective analysis of 121 patients with probable or definite CJD at 4 US academic medical centers, 26 of whom underwent a total of 55 procedures in the year before their diagnosis of CJD.¹ Only 1 procedure was performed with sterilization techniques adequate to prevent CJD.¹ There was, however, no evidence of iatrogenic transmission in any cases, although the follow-up period was admittedly too short given CJD’s potentially long incubation period.^1,2

CJD is a cruel entity—symptoms are nonspecific and may be mistaken for age-related cognitive decline, the disease is epidemiologically rare with a long incubation period, it is incurable, and it is uniformly fatal.^2,3 Although most infections occur sporadically, iatrogenic transmission is also possible. To date, approximately 500 such cases have been reported worldwide.² In the 1970s and 1980s, an outbreak of more than 200 CJD cases was found to be caused by transplanted infected cadaveric dura mater with a mean incubation period of 12 years.^2-4 In the 1980s, contaminated human growth hormone was linked to more than 200 cases of CJD with a mean incubation of 17 years.^2-4 Infection from corneal transplant and blood transfusions have also been described.^2,3 Yet, reported transmission from surgical procedures remains rare, with fewer than 10 confirmed CJD cases described in the literature, although the true prevalence is difficult to quantify as confirmed diagnosis requires autopsy.^2,3

Suspected transmission may only be slightly more common than confirmed transmission. Over a 15-year period, 19 suspected iatrogenic CJD exposures were reported to the Centers for Disease Control and Prevention, 2 from ophthalmology procedures and 17 from neurosurgical procedures.⁵ In all 19 cases, the diagnosis of CJD was not known before the intervention and all surgical instruments underwent normal decontamination protocols, which are inadequate to eradicate prion disease.⁵ Prion diseases are well known to be resistant to typical decontamination protocols, as traditional chemical disinfectants and thermal treatments do not adequately denature prion particles; prion stability may even be enhanced by drying or fixation in alcohol products.^2,4,5

Because of the long incubation period, cumbersome retrospective tracking of exposed equipment and patients, and uniformly fatal prognosis once infected, prevention of iatrogenic exposure is paramount. To address these challenges, protocols and preprocedural testing algorithms have been developed to identify patients at risk of having CJD, such as those with undifferentiated neurological conditions. For high-risk neurosurgical or ophthalmologic procedures,² patient tissues and fluids should be treated as suspected CJD contaminants until proven otherwise. Appropriate precautions, including communication among staff and quarantining involved instruments with specialized decontamination protocols, should be performed.⁵ For patients with suspected or confirmed CJD, the World Health Organization has published infection control guidelines to prevent transmission of disease.⁶ These safety guidelines include proper communication with all staff involved in the surgical procedure and sterile processing of supplies to be aware of potential exposure, minimizing number of staff in the operating room, using single-use equipment whenever possible and disposing of it by incineration, using protective coverings for all nondisposable equipment, and scheduling such procedures at the end of the day to allow adequate time for decontamination.⁶

These recommended precautions bring up the dilemma identified by Porter et al¹â€”how good is good enough? How do we, as a society, decide what prevention of a uniformly fatal disease should cost? No individual patient should ever have to be exposed to CJD as a result of a surgical procedure. However, from a population perspective, how much are we all willing to pay to prevent such a rare entity? Given how infrequent transmission is, are the protocols we have in place currently good enough? Or should we be investing more in preintervention prevention, such as enhanced screening techniques or more extensive decontamination between all surgical procedures, irrespective of patients’ risk of CJD? Unfortunately, these critical questions remain unanswered but are applicable for both CJD and future diseases. Humanity will inevitably be faced with new, rare, untreatable diseases in the future, and understanding what we are willing to pay morally and financially for prevention of iatrogenic transmission of these rare diseases will be intellectual time well spent.

Published: March 9, 2022. doi:10.1001/jamanetworkopen.2022.1561

Open Access: This is an open access article distributed under the terms of the CC-BY License. © 2022 Sun BJ et al. ÌÇÐÄvlog Open.

Corresponding Author: Joseph D. Forrester, MD, MSc, Department of Surgery, Stanford University, 300 Pasteur Dr, Ste H3591, Stanford, CA 94305 (jdf1@stanford.edu).

Conflict of Interest Disclosures: Dr Forrester reported receiving unrestricted research funding from Varian and has received grant funding from the Surgical Infections Society. No other disclosures were reported.