SUBJECT: Recommendations for 2023 Canadian Association of Radiologists (CAR) Diagnostic Imaging Referral Guidelines (current Guideline development project i.e. update from 2012 publication in-process) and Future of Radiology Care

June 26, 2023

To: CAR info@car.ca email administrator - time sensitive - please forward immediately to the appropriate direct email addressees of the listed Radiology Leaders with thanks.

c/o Candyce Hamel PhD chamel@car.ca and CAR Guideline Development Team Members: Candyce Hamel PhD, Ryan Margau MD, Paul Pageau MD, Marc Venturi MHA, Leila Esmaeilisaraji MD, Barb Avard BA, Sam Campbell MB BCh, Noel Corser MD, Nicolas Dea MD, Edmund Kwok MD, Cathy MacLean MD, Erin Sarrazin NP, Charlotte J. Yong-Hing MD, Kaitlin Zaki-Metias MD

c/o Anna Kielar MD, President CAR and Radiologist at the Joint Division of Medical Imaging, Canadian Association of Radiology at info@car.ca

c/o Gilles Soulez MD, past-president and vascular and interventional Radiologist at gilles.soulez.chum@ssss.gouv.qc.ca

c/o Emil Lee MD and members of Canadian Radiological Foundation (research and education - focusing on the future of radiology) crf@car.ca

c/o Michael Patlas MD, and members of Canadian Emergency Trauma and Acute Care Radiology patlasm@mcmaster.ca

c/o Jaron Chong MD, AI Standing Committee Chair and Working Group members jaron.chong@mcgill.ca / info@car.ca (specifically, Scientific Planning Committee, Continuing Professional Development, Magnetic Resonance Accreditation Program, Referral Guidelines Working Group, structured reporting working group. - and hopefully, the future Head and Neck Working Group)

c/o Charlotte Yong-Hing MD and members of Canadian Radiology Women bcrs@bcradiology.ca and Kiana Lebel MD resident Radiologist and EDI advocate kianalebel@gmail.com

FROM: Ruth McGroarty and members of Canadian CCJ Patient Support Group (see attached signatures, more to follow)Dear Canadian Association of Radiologists (CAR) President and Leaders, 2023 Guide Update Project Team, Canadian Radiology Foundation, Canadian Emergency Trauma and Acute Care Radiology and Canadian Radiology Women:

We are writing this letter to express our support for your efforts to update the 2012 radiology guide for physicians, and make recommendations for your consideration from a patient advocate perspective. But firstly, we would like to let you know that we are thankful of your initiative to provide updated guidelines to better meet the needs of patients in Canada, and that CAR members are working towards the advancement of radiology in Canada. We would like to make recommendations for the updated radiology guide 2023 for your consideration which will be noted on the following page. But first, a little bit about who we are and how we got to where we are.

Who is the Canadian CCJ Patient Support Group?

We are patients diagnosed (Dx) with Fibromyalgia (FM), Myalgic Encephalomyelitis (ME/CFS), Head and Neck Trauma (WAD/TBI), Ehlers-Danlos syndrome (EDS), Lyme disease, and Long Covid, or patients with neurological symptoms awaiting investigation and diagnosis; but primarily, we are parents, spouses, children of aging parents, and formerly highly active professionals whom are now quite disabled, often bed or home bound and requiring assistance. The majority are Canadian women but there are also several Canadian men, that have formed a support group (which is primarily focused in medical science and Canadian healthcare resources) for those experiencing disorders related to the craniocervical junction (CCJ) / symptoms related to the head and neck.

Since 2004, there have been studies, such as the “Clinical evidence for cervical myelopathy due to Chiari malformation and spinal stenosis in a non-randomized group of patients with the diagnosis of fibromyalgia” suggesting patients with a Dx of FM should have Chiari malformation, stenosis and cervical myelopathy ruled out as differential Dx.

Fast-forward to 2017, the documentary film called “Unrest” by Jennifer Brea, PhD candidate at Harvard University tells the story of how she suddenly became ill, never recovered from a viral infection and was Dx ME/CFS. In a worldwide online group (which many Canadians are members), Jennifer explains how a physician contacted her after watching Unrest, and suggested that her normal imaging may in fact, not be normal due to a visualized empty sella. Jennifer since, has had craniocervical fusion as a result of ligament laxity and states she has almost completely (98% recovered) from her neurological symptoms.

Jeffery Wood, M.A. and writer of mechanicalbasis.org has a similar experience, and went online to tell his story to other ME/CFS patients. He is also now fused, recovered and working again. It would seem that there may perhaps be some form of adult Grisel syndrome occurring with the new, stronger virus’ of our time? Since then, hundreds or closer to over 1000 of these online members (including Canadians) have obtained dynamic imaging, confirmed craniocervical abnormalities and sought/are seeking treatment options.

More recently, in 2020, a study was published in Sweden, “Signs of Intracranial Hypertension, Hypermobility, and Craniocervical Obstructions in Patients With Myalgic Encephalomyelitis/Chronic Fatigue Syndrome”, which once again highlighted medical abnormalities related to the craniocervical junction.

There’s also a significant number of people in our Canadian support group that suffered head and neck trauma, such as car accidents, sport accidents (ski, mountain bike, toboggan, equestrian) and their radiology reports indicated no significant findings. They were not knocked unconscious at the time of accident but gradually, post-accident continued to decline with neurological symptoms, becoming bedridden and a few group members suffering from dystonic seizures. Others didn’t have any type of onset significance and later tested positive for Lyme or EDS.

Some of us that have the fortunate means to go out of country, have confirmed radiology findings positive with abnormal craniocervical junction measurements, empty sella, IJV compression, IIH, occult tethered cord and venous congestion; and we all wish we could have received this medical imaging, information and support in Canada. Most others across Canada, are still waiting for care.

An important learning from our patient-led medical learning and advocacy, as well as our CCJ radiology reports, out of country assessments and diagnostics, is the impact of a misalignment of a craniocervical junction. This may cause pressure and tension on the craniocervical junction, as well as the anatomy and systems within and around it (for example, the brain, brainstem, vertebral and carotid arteries, internal jugular veins (Dr. V. Pereira in Toronto currently working on this), cerebrospinal fluid, nerves, lymph vessels etc.). Another significant learning is that static, supine imaging is sometimes or perhaps more often than believed, insufficient to examine the craniocervical junction, especially given craniocervical junction’s mobility and complexity. Conversely, dynamic imaging, both MRI and CBCT/CT Scan, can provide useful information and insights for symptom causes.

Generally speaking, physicians need to know our symptoms improve when laying down, not completely nor much better, but enough to relieve some symptoms; And that our symptoms are exacerbated in dynamic positions. Many of us that have had dynamic imaging have discovered that our craniocervical junction (and its surrounding anatomy and systems) is abnormal when we are in certain positions such as upright, flexion, extension and/or rotation. We believe that our future care is in your hands, which is why we are emphatically writing you today.

Four (4) Recommendations for Your Consideration:

We humbly write to you with four (4) recommendations for your consideration, for the upcoming updated radiology guide 2023, and the future of radiology care. These recommendations include adding the craniocervical junction as it’s own section, leading the recommendation and utilization of dynamic imaging for specific patient populations, implementing standard structured CCJ radiology reporting, and last but not least, placing the craniocervical junction as a significant area of focus within working groups, organized future research, education, AI and protocols for emergency department, trauma and acute care.

1. Adding the Craniocervical Junction as its own Section (i.e. making the guide 14 sections)

We believe that adding the craniocervical junction as its own section to the upcoming, updated CAR Guidelines will provide the significance and focus as the major junction / joints, that the CCJ is. Doing so will help both Physicians who are seeking your expertise, and benefit a significant number of patients. In return, it will improve the effectiveness and cost efficiency of the healthcare systems in Canada, as often reported, these patient groups see up to 30 physicians and specialists over several years before receiving an ME, FM or EDS diagnosis. As an article authored by Department of Neuroradiology at the Royal London Hospital, in London, UK mentions, “The craniocervical (craniovertebral) junction represents the complex transitional zone between the cranium and the spine and comprises a complex balance of different elements: it should be considered anatomically and radiologically a distinct entity from both the cranium and, in particular, the cervical spine”.

2. Leading the Recommendation and Utilization of Dynamic Imaging for Specific Patient Populations

We suggest for patients with a history of neurological symptoms or Dx Myalgic Encephalitis/ Chronic Fatigue Syndrome (ME/CFS), Chiari, Fibromyalgia (FM), Rheumatoid Arthritis, Down syndrome, Ehlers Danlos syndrome (EDS), Chronic Whiplash Associated Disorders, Multiple Sclerosis (MS), Chronic Lyme disease and Long Covid, that dynamic imaging in the form of Dynamic Digital Radiography, MRI and CBCT/CT is recommended in the updated Guidelines.

There are many radiology technologies that can facilitate dynamic (i.e. positions of neutral, flexion, extension, rotation and lateral flexion) radiology diagnostics. Currently, a majority of patients do not get referred to specialists due to “normal” radiology imaging reports. This leaves chronic complex care patients in the hands of front line, primary care physicians, whom are not adequately positioned to diagnose and treat these conditions. Therefore, due to the likelihood of craniocervical junction issues in the above stated patient groups, we suggest that the guidelines recommend dynamic neutral, flexion and extension imaging, such as but not limited to:

• Dynamic Digital Radiography (Konica Minolta)
• Static MRI Brain and Cervical Spine (from skull to T1, not C3 down)
• Dynamic Upright MRI
• Dynamic CBCT - can be used flexion, extension, and lateral flexion (note: it is being utilized by world renowned orthopaedic and neurosurgeons in assessments and head and neck surgeries; which is higher quality than Xray, 3D renderable and less radiation than CT)
• Dynamic CT Scan Head and Neck (flexion, extension, rotation)
• Dynamic MRV/ CTV Brain and Neck (skull to chest), then catheter
• Dynamic Venous and Arterial Ultrasound Head and Neck
• If positive for CCJ concerns, also complete MRI Thoracic and Lumbosacral to rule out occult tethered cord and other spine issues

3. Implementing Standard Structured CCJ Radiology Reporting

We suggest that for patients with a history of neurological symptoms or Dx Myalgic Encephalitis/ Chronic Fatigue Syndrome (ME/CFS), Chiari, Fibromyalgia (FM), Rheumatoid Arthritis, Down syndrome, Ehlers Danlos syndrome (EDS), Chronic Whiplash Associated Disorders, Multiple Sclerosis (MS), Chronic Lyme disease and Long Covid, that standard structured radiology reports for the Brain, Craniocervical Junction and Cervical Spine include a review and findings of the following (below):

• Atlantodental Interval (ADI)
• Clivoaxial Angle (CXA)
• Harris Measurements (BDI, BAI)
• Grabb-Oakes Line
• Chamberlain Line
• McRae Line
• Powers Ratio
• Sum of Spence / Lateral Mass Displacement (LMD)
• Degree of Rotation
• Optic Nerve sheath
• Empty Sella
• Transverse sinus
• CSF Leaks or congestions, or presence of Tarlov cysts
• IIH signs
• CCJ/Cerebral Arterial or Venous congestions in the Head, Neck and Chest
• Cervical spondylotic changes affecting surrounding anatomy/systems
• Elongated, calcified styloid processes
• Distal Spinal Cord
• Conus Medullaris
• Filum Terminale
• Cauda Equina
• etc.

These assessments are to include measurements in each position (i.e. neutral, flexion and extension, lateral flexion and rotation*). Many of the leading radiologists and CCJ specialists worldwide currently complete these assessment and measurements in both static and dynamic imaging, and include such in their radiology reports.

4. Placing craniocervical junction as a significant focus within working groups, organized future research, education, AI and protocols for emergency department, trauma and acute care.

Many of the people in our group have been told by Canadian physicians, “if you had craniocervical instability, you wouldn’t be standing here, you’d be on the operating room table or - (implying dead)”.

We are writing to tell you that because some of us have had dynamic imaging and examinations / full workup (with level/grade 1 & 2 studies to back the findings) by specialists around the world, our conditions were accurately identified. This led to some patients recovering due to CCF (fusion) surgery or a trial of orthobiologics injected through percutaneous implantation of the transverse and alar ligaments, styloidectomy surgery or other recommended treatment modalities. Dynamic MRI, CBCT/CT imaging and Dynamic Angiograms and Venograms are necessary for our patient population; and static imaging and X-rays aren’t adequate for our patient population.

We believe that care for our patient population can be expedited through the updated Radiology Guide and equally important, with present working groups, research, education, AI and emergency departments interweaving the significance of the craniocervical junction into all radiology aspects going forward. We are asking that you consider reviewing and including existing, recent craniocervical junction and dynamic imaging research in your guides and practice, gather and review patient data and consider conducting new research (note: we can assist getting grant funding, if needed) to advance the future of radiology in Canada alongside or ahead of international medical providers.

To conclude, Dr. William Osler is quoted to have said “Listen to your patient; he is telling you the diagnosis”, and here we are, just a short 5 years since Jennifer Brea and Jeff Wood started a grass roots movement to empower patients to advocate for their healthcare and well-being; and sadly, it’s been almost 20 years since the 2004 medical study that provided evidence that fibromyalgia patients suffered with craniocervical junction conditions. It’s time for change. It’s time for radiology guide updates and advancements in a key and essential part of the body - the craniocervical junction. We are patients telling our Canadian Association of Radiologists what imaging is needed for our patient population, which is, by estimation, 5.7% of the Canadian population. Is CAR listening?

We believe these recommendations are highly useful, practical and cost-effective, and would help PCPs and treating specialists by providing all encompassing accurate radiological information to develop effective treatment plans for these patient groups. In turn, improving the conditions and quality of life for patients, and potentially curbing the long-term neurodegenerative effects caused by craniocervical disorders.

We appreciate your willingness to work towards improving patient outcomes, and hope you will consider these suggestions going forward. We genuinely hope you can see and hear us now, and would be more than happy to have further discussions or communications, if you wish.

Thank you for taking the time to read this letter and for your commitment to improving patient care.

Sincerely,

Ruth McGroarty and individuals (see attached excel sheet for electronic signatures)

Brampton, ON

MEDICAL & OTHER REFERENCES:

• The craniocervical junction: embryology, anatomy, biomechanics and imaging in blunt trauma by Curtis Edward Offiah and Emily Day. (Nov. 2016) https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5265194/
• Canadian Cases from reliable sources: Fibromyalgia (900,000+ cases), ME/CFS (580,000+ cases), and EDS (possibly 1:200 / 187,950 cases), Chronic Whiplash (26,313 cases), Chronic TBI (22,554 cases) (RA 374,000 cases) (Down Syndrome 45,000 cases), (MS cases 97,300), and (long covid TBC)
• The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions by Michael Flanagan (2015) https://www.hindawi.com/journals/nri/2015/794829/
• Direction and magnitude of cerebrospinal fluid flow vary substantially across central nervous system diseases by Per Kristian Eide, Lars Magnus Valnes, Erika Kristina Lindstrøm, Kent-Andre Mardal & Geir Ringstad (April 2021) https://fluidsbarrierscns.biomedcentral.com/articles/10.1186/s12987-021-00251-6#Abs1
• Signs of Intracranial Hypertension, Hypermobility, and Craniocervical Obstructions in Patients With Myalgic Encephalomyelitis/Chronic Fatigue Syndrome by Björn Bragée Anastasios Michos, Brandon Drum, Mikael Fahlgren, Robert Szulkin, Bo C. Bertilson (2020 Aug 28)
• Clinical evidence for cervical myelopathy due to Chiari malformation and spinal stenosis in a non-randomized group of patients with the diagnosis of fibromyalgia by Dan S. Heffez, Ruth E. Ross Yvonne Shade-Zeldow, Konstantinos Kostas Sagar Shah Robert Gottschalk Dean A. Elias Alan Shepard Sue E. Leurgans and Charity G. Moore (Oct. 13 2004)
• Craniocervical Instability in Ehlers-Danlos Syndrome-A Systematic Review of Diagnostic and Surgical Treatment Criteria (Canada) by Laura-Nanna Lohkamp, Nandan Marathe, Michael G Fehlings (2022 Feb 23) https://pubmed.ncbi.nlm.nih.gov/35195459/
• Reference values of four measures of craniocervical stability using upright dynamic magnetic resonance imaging by Leslie L. Nicholson, Prashanth J. Rao, Matthew Lee, Tsz Ming Wong, Regen Hoi Yan Cheng & Cliffton Chan (Jan 2023)
• CT of the head and neck, part 1: physical principles by A C Miracle, S K Mukherji (June 2009) and CBCT Evaluation of Atlantoaxial / Atlanto-occipital joints for cervical instability risk assessment (Jan. 2019) and Craniocervical Junction Visualization and Radiation Dose Consideration Utilizing Cone Beam Computed Tomography for Upper Cervical Chiropractic Clinical Application a Literature Review by Greg DeNunzio, Tyler Evans, Mychal E Beebe, Jaime Browning, Juha Koivisto (June 2022)
• Webinar on venous congestion, brain and neck disorders by Dr. Ferdinand Hui and team https://www.youtube.com/@VenousCongestionCommittee and Artery insufficiency in rheumatoid atlantoaxial subluxation M. W. JONES AND J. C. E. KAUFMANN (Canada) https://jnnp.bmj.com/content/jnnp/39/2/122.full.pdf