Keywords
central sterilizing department - Creutzfeldt–Jakob disease - prion disease
Case Report
A 69-year-old woman was admitted with a newly diagnosed large right sphenoidal wing
meningioma. She had a background history of suspected Prion disease and was at low
risk of developing Creutzfeldt–Jakob disease (CJD). She had been treated with human
pituitary hormone for infertility in Australia’s first computerized medical program,
the Australian Human Pituitary Hormone Program, prior to 1986.
The patient underwent elective grade 2 Simpson excision of the meningioma without
any complications. We present here the steps taken to safely proceed with a neurosurgical
intervention in a patient with suspected Prion disease, specifically in the context
of instrument availability and sterilization [Fig. 1].
Fig. 1 MRI brain (T1+C) showing (A) large extra-axial mass arising from the right greater wing of the sphenoid bone.
(B) Post excision of the large right sphenoid wing meningioma. MRI, magnetic resonance
imaging.
Planning for Neurosurgery in a CJD Patient
Preoperative Planning
Consultations held with the neurosurgeons within the department, the director of surgery,
the CJD support group director, the codirector of the Australian CJD registry, and
a member of the CJD incident panel; consultations also between infection-control teams
at the Royal Hobart Hospital and Westmead Hospital in Sydney (where a similar situation
arose several years ago).
Detailed discussion with the theater in charge, nurse unit manager, anesthetic team,
the patient, and family members.
One major preoperative issue was the availability of instruments. Instruments cannot
be re-used and have to be incinerated or kept aside for the same patient.
Also, there were safety concerns with regard to the handling of higher infectivity
tissue for the health professionals involved. Hence, the entire neurosurgical team
involved with this case in theater were prepared well with regard to the infection
control protocol for this case.
Intraoperative
All other cases were cancelled on the day of the surgery.
Instruments used for the procedure are detailed in [Fig. 2].
Fig. 2 Copy of the checklist of instruments used for the procedure.
Postoperative (Sterilization)
Sterilization and storage–central sterile supply department (CSSD):
The nurse unit manager, infection prevention & control unit, created a plan based
on the CJD infection control guidelines in consultation with the heads of the theater
and the central sterilizing department (CSD).
All instruments were stored in theater for future use for this patient after sterilization.
The management plan for reprocessing of the instruments followed the seven-step detailed
protocol of the CJD infection control guidelines 2013, which is briefly summarized
below.[1]
STEP 1: Transfer of reusable instruments to the CSD.[1]
STEP2: Initial management of instruments[1]
Cleaning of instruments with ionic detergent
STEP3: Waste management[1]
After the initial cleaning, the operator disposes the soiled PPE (personal protective
equipment) in the cytotoxic bin marked as HIGH-RISK, places it in the waste collection
area, and notifies environmental services to collect it as soon as possible.
STEP 4: Management after initial cleaning and before sterilization.[1]
STEP 5: Cleaning of macro area[1]
STEP 6: Packing for sterilization[1]
-
A clearly defined area in the approved space is used for packing. Clean dry instruments
are transferred to the packing room where a spare wrap is placed on the surface, a
wrap required for the pack is placed on the top, and packing done as per normal practice.
STEP 7: Sterilization and postmanagement[1]
-
The instruments are sterilized as a separate load. The packs are handled carefully
to prevent crossover and bundled together in a sealed peel pack, with clear labeling
of patient identifying stickers, and subsequently placed in a location appropriate
for storage of sterile items, but separated from general trays and instruments, for
future use for this patient.
Discussion
Creutzfeldt–Jakob disease is a fatal human Prion disease belonging to the transmissible
spongiform encephalopathies (TSEs)[1]. CJD occurs in familial, sporadic, and acquired (iatrogenic and variant CJD) forms,[1] and this condition is caused by a pathological accumulation in the brain of an aberrant
form of a normal cell surface glycoprotein or Prion Protein (PrP).[1]
Given that there is presently no test available to confirm the presence of CJD, the
decision to implement additional procedures for equipment reprocessing is based on
risk assessment tools which classify the spectrum of infectivity of the tissues exposed,
and by identifying patients with definite versus potential risk of CJD transmission.[1]
The mode of transmission is via instruments contaminated with the central nervous
system tissue or contaminated tissue implants or products like dura mater grafts,
corneal grafts or pituitary products.[1] Prion protein is the infective agent in CJD and is resistant to routine reprocessing.
Hence, additional sterilizing procedures are devised to prevent the risk of transmission.[1]
Chemicals like anionic detergents, hypo chlorites, harsh acids, and alkalis have some
activity against Prion disease.[1] However, the major concerning factor in these cases is that multiple reprocessing
steps will only reduce infectivity.[1] There is no established method found yet to fully sterilize the instruments, so
that they can be reused in other patient’s safely.[1] Hence, it is recommended to use single-use instruments wherever possible, without compromising on patient care[1]
.
The case discussed here is an iatrogenic CJD, secondary to receiving human pituitary
hormone product for infertility. This is considered as a low-risk case since the patient
has only a potential risk of CJD transmission. It is not a definite risk; however,
the necessary preparations are cumbersome.
The procedures outlined here are a guide for similar surgical situations. Even though
this a rare scenario, being well-prepared for these situations will help significantly,
especially in cases requiring emergency surgery. Planning for instruments required
for the surgery which can be incinerated, is a major challenge. Also, extensive preparation
of the entire team in the theater with regard to the infection control measures preoperatively
helped the staff involved significantly in dealing with a rare scenario like this.
We were fortunate since this patient was stable and we had adequate time to consult
with multidisciplinary teams and prepare for the situation in a timely manner. All
the instruments used have been labeled and stored separately for future use in this
patient if required.
Conclusion
The large sphenoid wing meningioma found in this patient with suspected Prion disease
was successfully and safely managed by timely and methodical intervention. The steps
taken for proceeding with the surgical management and aftercare may be useful for
similar cases in future.
