But a patient who just feels bad (even if they feel very bad), and especially if they do not have positional triggers and their imaging does also not demonstrate constant brainstem or otherwise vascular compromise that fits with the symptoms, then diagnosing such a patient with CCI or AAI and claiming its presence as the culprit of their symptoms, is madness. More information about surgical treatment. One is especially predisposed to this problem if the affected vertebral artery is highly dominant (much higher caliber than its contralateral counterpart) or if the contralateral artery is extremely hypoplastic, or, finally, the contralateral artery terminates as the posterior inferior cerebellar artery rather than at the basilar artery (Josy & Daily, 2015). BDI, ie. In cases of hyperlaxity, It is not uncommon to find subaxial cervical alterations (levels below C3 to C7 . Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. Request an appointment or second opinion, refer a patient, find a doctor or view test results with MGfC's secure online services. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. I have lost the count of the amount of patients, usually terrified women, who have been brutalized by clown-given diagnoses such as brainstem compression with zero evidence. For the sake of relevance, this article will mainly address ligamentous and muscular injuries, as these topics, especially when mild, are much more controversial than incidences of CVJ fracture. Education Type three involves anterior subluxation of the entire atlas due to combined full rupture of the TAL and partial rupture of the capsules and other structures. ), induction of symptoms (all or nearly all of your symptoms, not some neck pain) with maximal rotation, nor during flexion or extension. This would apply for patients with obvious hypermobility but who do not have clinical triggers compatible with CCI or AAI (induction of symptoms in flexion, extension or rotation, and complete normalization when in neutral). Although there were no current grounds for surgery? AAI and CCI are diagnoses that mainly cause the risk for either brainstem damage or injury to the arteries that supply the brain with blood, and this can cause paralysis or stroke if left untreated in cases where there is legitimate evidence for pathology. She started researching on certain online forums, in which she was advised to look into AAI and CCI. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. Look for jugular vein compression, dural sinus and neck vein integrity, exclude typical patholgies such as aneurysms etc., exclude vertebral or carotid dissections, evaluate the thoracic outlet for interscalene, costoclavicular or subpectoral stenosis), Doppler of the carotid and vertebral arteries (look for signs of hypertension, cf. Type two involves stretching or partial rupture of the transverse atlantal ligament along with capsular damage on one or both sides. Suboccipital symptoms that occur only with cracking, if the MRI shows arthritis or joint effusion, especially if the neck locks in rotary fixation, then this could be a case of legitimate AAI or CCI. Ultimately, the reader must discern for themselves. Commonly misunderstood and overemphasized measurements. Common findings: Ovalization of the orbitae, dilated optic nerve sheaths, pituitary concavity, Chiari malformation, tight brain appearance, jugular vein compression with or without white-vessel signs, dilation or narrowing of the lateral and possibly third ventricles, periventricular ependymal T2 FLAIR hyperintensities), Neck MRI (general evaluation of the neck integrity), CT angiogram of the head neck and subclavian arteries with the arms raised (contrast infusion via femoral vein. This can also damage the brainstem and produce symptoms similar to what is described above. There is a growing trend, however, within the (or, at least, certain) alternative medical communities, where patients with normal or virtually normal imaging, and with the absence of clinical triggers that would suggest atlantoaxial or craniocervical instability, still end up diagnosed with these relatively sinister diagnoses. Pain medications and anti-inflammatories are typically also prescribed. Mild and often even moderate circumstances of AAI and CCI can be treated with appropriate (specific, not generic) physical therapy to strengthen the muscles that prevent hypermobility. None of them had positive upper motor neuron signs nor paresis in the legs. To compress the brainstem it must be compressed from both sides, both infront and behind. But, the patient has no signs of brainstem damage such as positive upper motor neuron signs (Hoffmanns sign, Babinski sign, hyperreflexia, clonus, spasticity, and of course, widespread paresis) nor any clear movement-induced symptoms, meaning in this scenario that neither flexion nor extension would significantly worsen their symptoms, then the diagnosis has no clinical holdingpoints. In most circumstances, even if there is poor overlap but no evidence of frank facetal luxations (clinical history or with provocation), then conservative therapy can usually prevail in management. Symptoms of VBI develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the triggering position. PMID: 24475346; PMCID: PMC3899735. Thus we control the spinal cord and nerves (cranial and cervical) in order to avoid potential damages to these important structures. Patients with horizontal instability of the craniovertebral junction but without rotary subluxation may not necessarily demonstrate the same level of rigidity, but may show induction or resolution of symptoms as they venture into flexion vs. extension. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. In moderate stages, the MRI will appear abnormal, but the CTV will still appear relatively OK (because the patient tends to be placed on a neck wedge which protracts the head in the CT machine this reduces the compression). This may cause the patient to become afraid and to google their symptoms, which in and by itself is reasonable enough. Foramen magnum decompression or syrinx manipulation was not performed in any patient. In BI, the compression tends to be constant. But this measurement in and by itself, when it is 9 or 10 or even higher, but there is no brainstem compression not even in flexion-extension imaging this cannot be interpreted as a surgical indicator. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? Gweon HM, Chung TS, Suh SH. In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. Unfortunately, she was not compliant to the treatment that I prescribed (TOS, TOS CVH) other than the treatment for AAI, which she was convinced that was her problem. Atlantoaxial instability is a relatively frequent finding in individuals with Down syndrome. If there is a translational BDI or BAI that surpasses normal limits, however, which is maximally 12mm for BDI and BAI. Whats interesting, regardless, is that one year after we had the first consultation she underwent another uMRI (due to lack of improvement of symptoms), which showed completely resolution of the atlantoaxial subluxations, which were now overlapping at about 30%; 300% improvement (remember: >20% is normal). We can still treat it preventatively, but it wont resolve the symptoms. Specialist imaging research to help diagnosis. Let us help you navigate your in-person or virtual visit to Mass General. Neuronavigation assistance guides us all through the surgery, thus it diminishes (though it does not eliminate) the risks while placing the screws for the fusion. If there are no symptoms, then what reuslts are you talking about? In dogs with atlantoaxial subluxation, instability of the atlantoaxial joint results from a loss of ligamentous support of the axis, often with concurrent aplasia, hypoplasia or dysplasia of the dens. However, I also told her that she may end up having fixation surgery in the future to prevent foreseeable compressive damage to the brainstem. The patient should demonstrate some brainstem symptoms, and may develop quadriparesis if the compression is sufficiently hard and constant. Diagnostic imaging: Spine, 3rd edition. This webpage is intended to provide health information so that you can be better informed. Your email address will not be published. However, appropriate inclusive criteria must be used to render the diagnoses; subtle findings and the lack of a strong clinical correlation is not enough, and will easily lead to misdiagnosis and related anxiety and suffering. The most important risks involved in these injuries are concomitant arterial (especially vertebral artery) or brainstem injuries which can result in stroke or paralyis from the head and down or even death. Headaches certainly can develop from instability of C1-2. In vertical dissociation of the CVJ, the main dangers will similarly as above involve potentially dangerous pulling and pushing on the blood supply to the brain (carotid and vertebral arteries) as well as the brainstem itself, potentially causing dissection of the arteries. The patient had headache, dizziness, fatigue, pain in the arms and chest and often felt difficulty breathing. If the latter, could be JOS obstruction, or could be placebo. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. This will be predominantly evident on a flexion/extension scan, where the basion-dens interval (BDI) will be dynamically increased, and greater than 10-12mm (Ross & Moore, 2015; Deliganis et al. This, seriously augmented by poor hinge neck postures (Larsen 2018). Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. 2009), but this is extremely rare. Kjetil Larsen is a Researcher and a injury rehabilitation specialist, and is the owner of MSK Neurology. Surgical reduction and fixation would be the only appropriate treatment. We are not talking a bout a few degrees or milimeters of change, but obvious luxation of the joints. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. Instability in the hip can result in dislocation, ligament tears, muscle damage and wear of the joint. Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. Neurosurg Rev. (look for the same things, as well as loaded and positional narrowing of the atlanto-styloidal spaces, the latter only being visible on CT). For TOS CVH the patient will generally feel better when stress is reduced along with taking beta blockers (confer with your doctor). DMX I dont recommend getting a DMX. Stay put for 30-60 seconds, look for worsening of symptoms while in the test. 2014 Aug;4(3):197-210. doi: 10.1055/s-0034-1376371. Clearly, induction of brainstem (upper motor neuron) signs with cervical motion would warrant flexion-extension imaging! Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. Knowing this it allows to anticipate any possible problems in the postoperative period. 2015. After hospital discharge, doctors usually control patients at least once a week after discharge on an outpatient basis, to make sure everything is correct before flying back home, thus we recommend to stay in Barcelona after discharge for 10-15 days. This would depend on whether or not the compression of the brainstem is constant, which again would depend on several factors. Does thoracic outlet syndrome cause cerebrovascular hyperperfusion? If it is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted. Patients with craniovenous outlet obstruction due to JOS may induce their symptoms with a Queckenstedts test, that is in essence a manual compression test of the internal jugular veins. Surgical management is recommended for those with severe signs and for those who have tried and failed medical management. It mainly consists of the posterior fusion of the affected vertebrae, in this case, the atlas (C1) and the axis (C2). Jugular outlet obstruction is commonly seen in patients with upper cervical horizontal facetal misalignment, and especially if they have broad transverses processes or a posteriorly angulated styloid process (Gweon et a. The deep neck flexors should not engage as this lessens the compression. In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. A review of the diagnosis and treatment of atlantoaxial dislocations. You also have the option to opt-out of these cookies. Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. Atlantoaxial instability is an uncommon condition of dogs in which there is abnormal movement in the neck, between the atlas (first cervical vertebra) and axis (second vertebra). Diagnostic markers for occult craniovascular congestion. and craniovenous outflow obstruction) will frequently cause severe fatigue, migraine, headache, dizziness, tinnitus, pain in the upper neck/back of the head (this is hypertensive migraine, not atlas pain Larsen et al 2020), POTS, memory loss, cognitive decline or fluctuating cognitive ability, syncopal event, seizures, and even, sometimes, hemi or paraparesis and other stroke-like symptoms. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. Now, what if there is no frank compression nor clinically medullary signs and triggers, but there is a very small space both infront and behind the medulla that has been gradually getting worse. Therefore, when there is evidence of equivocal findings such as signal changes in ligamentous structures without expected adherent findings such as gross hypermobility compatible with the injury at hand, this can generally not account as someting sinister. It is advisable to obtain just a lateral view first. Request Appointment. 3. For example, if the patient blacks out every time she turns her head to the left, a followup dynamic catheter angiography could be done, and may demonstrate high-grade stenosis of the vertebral artery when turning to the left. If you are very concerned that you have craniocervical and atlantoaxial instability, then I recommend getting workups for both these but also relevant differential diagnoses. That is why they are much less affected by actual neck position than legitimate CCI AAI patients are, and certainly do not become symptom free in neutral positions. Advanced Surgical Neuro-oncology Fellowship, Complex and Minimally Invasive Spine Deformity Fellowship, Endovascular Surgical Neuroradiology Fellowship, Neurosurgical Spine Innovation Fellowship, Neurosurgical Peripheral Nerve and Spine Fellowship. The atlantoaxial instability may also have an acute traumatic origin, which may sometimes require urgent treatment, though in some cases it triggers development of the craniocervical or atlantoaxial instability. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. The patient may seek out their GP or a local neurosurgeon who will, usually, and usually rightfully so, dismiss these claims, as the patients imaging is normal and also lack neurological signs that would fit with neurovascular compromise. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. I diagnosed her with mild (benign) atlantoaxial instability and TOS CVH. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. Magnetic resonance imaging assessment of the alar ligaments in whiplash injuries: a case-control study. Not sure what you mean here. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. As always, it is important to do a clinical radiological correlation to make an accurate assessment. The patient will hinge back at their neck while simultaneously flexing the cranium. J Craniovertebr Junction Spine. She was never evaluated for clinical correlation for these alleged findings, ie., no one evaluated if these findings had actual compatibility with her clinical symptoms and, especially, triggers. Necessary cookies are absolutely essential for the website to function properly. This website uses cookies to improve your experience. Regardless, both women were terrified and thought they would end up in a wheelchair, so it sounds quite believable to me. He specializes in the treatment of chronic pain and has developed several distinctive protocols both with regards to diagnosis and conservative rehabilitation of difficult conditions. Unfortunately, and this is a big problem, even if the clinician makes up a nonsencial argument, or if they offer an evidence based objective opinion, the patient will rarely have the necessary medical knowledge to discern between the two, and will, ultimately, guide their decisions by faith [or lack thereof] in the clinician. What Is Atlanto-Axial Instability (AAI)? We offer diagnostic and treatment options for common and complex medical conditions. PMID: 18708935. Save my name, email, and website in this browser for the next time I comment. The other side of the AAI/CCI coin is the risk for facetal luxation; a less sinister-, but still a problem that warrants surgical treatment. Henderson FC Sr, Rosenbaum R, Narayanan M, Koby M, Tuchman K, Rowe PC, Francomano C. Atlanto-axial rotary instability (Fielding type 1): characteristic clinical and radiological findings, and treatment outcomes following alignment, fusion, and stabilization. collected, please refer to our Privacy Policy. 2011 Apr;15(1):41-47. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. Copyright Dr Gilete Neurosurgery & Spine Surgery. My experience has been that these approaches do not work, and certainly do not cause long term results. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. The joint between the upper spine and base of the skull is called the atlanto-axial joint. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. Thanks for your help! If combined with Chiari malformation, compression of the cerebellar tonsils can cooccur and will occur with lower measurements than normally needed to cause brainstem compression alone, due to filling of the space behind it (the descended cerebellum). TOS increases perfusion rates to the brain, to which the brain is very sensitive and may dysfunction depending on how high the pressures are (Larsen et al 2020), often resulting in severe fatigue, dizziness, headaches and especially occipital headaches/pain (these are hypertensive headaches, not an atlas problem). She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. How is one supposed to know, if no one knows what you have in the first place? If there is a 1mm listhesis, however and the patient has no neurological symptoms and the medulla is utterly free of compression, then performing fusion is completely unnecessary. Washington University neurosurgeons have extensive experience treating problems in this area and are recognized nationally as experts in providing innovative treatments for this unique and complex area of the neck. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. All conventional things like heart and lung problems, MS, cancer, infections etc. zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. Dynamic angiograms could also be applicable in certain circumstances, cf. Atlantoaxial instability | Cervical Fusion or Prolotherapy PRP Stem Cell treatment options Surgical treatments for Cervical Instability Disc, disc, disc may be wrong, wrong, wrong In These cookies will be stored in your browser only with your consent. Facetal rigidity and dysarticulation is very common in patients with poor cervical postures and functionality of the neck muscles, and especially the muscles that restrict rotation and attach directly onto the spinous or transverses processes in the spine. The General Hospital Corporation. This, usually due to trauma, but can also occur gradually due to certain autoimmune disorders such as rheumatoid arthritis, gross congenital hypermobility (such as Ehler Danlos syndrome or Marfan syndrome), or certain congenital syndromes such as Downs syndrome (Yang et al. Traumatic Atlantoaxial Lateral Subluxation With Chronic Type II Odontoid Fracture: A Case Report. There can be, and are indeed many more potential explanations for these symptoms than just AAI and CCI. The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. Case Rep Neurol 2019;11:295298, Waldock WJ, Higgins NJ, Axon P. A case report of gastroparesis resolved by styloidectomy. Once the diagnosis of atlantoaxial instabilityis made, one should consult the neurologist, neurosurgeon, and a geneticist if the patient is a child. Pearls and Other Issues The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. This Complete rupture of the transverse atlantal ligament, however, will generally promote dorsal and cranial migration of the odontoid process (the atlantodental interval (ADI) will be increased (> 3,5mm) while in flexion) causing it to compress the brainstem dorsally (in the upper neck), or to migrate into the foramen magnum and compress the brainstem there (basilar invagination), where the tip of the odontoid will be seen far above the Chamberlains line, whereas it in normal patients sits about 2mm below the line. Finally, beware that many of these uMRI clinics render horrible images that barely show any anatomy, yet somehow still manage to determine various complicated diagnoses from them. Treatment, depending on the neurological symptoms and related pain, may be surgery. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. TOS is also a common cause of dyspnea (respiratory difficulty), although these patients will have normal blood oxygen levels, which was also the case here. In the Axis, pedicle screws are usually the first choice although, depending on the patients anatomy, placement of isthmic screws may be considered. This is easily seen on imaging, especially on CT, as the alignment of the joint will be unequivocally abnormal to the extent that would not be achievable without tremendous ligamentous injury. 15 Piscataqua DriveNewington (Portsmouth), NH, 03801 603-431-3600, 8 Maple Street, Suite 2 Meredith, NH 03253 603-279-1117, 2023 All rights reserved | Sitemap | Legal | Law Firm Essentials by PaperStreet Web Design, Caudal Cervical Spondylomyelopathy (Wobblers). Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. If not, does the patient actually have any significant symptom induction with rotation? Your email address will not be published. I have not receiving anything that comes close of what they produce. had been excluded by her primary care physicians and local hospital. This can result in AAI where the bones are less stable and can damage the spinal cord. Horizontal misalignment of the facet joints often cause dorsal migration of the C0 and C1 facets which cause approximation of the styloid process and the C1 transverse processes. Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon.
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