atlantoaxial instability specialist

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A general neck MRI is usually a good idea and may show some arthritis in the atlantoaxial and atlanto-occipital joints along with minor intra-articular effusions, suggesting irritation of the joints. What I prefer to do is to first draw lines that show the actual rotational alignment of the C2 and C1 when looking left and right. However, if the patient has symptoms regardless of being in rotation or not, and has never had a case of alantoaxial rotary fixation, then there is no evidence that this is the cause of the patients symptoms, even if it, indeed, may be a bit loose. The atlantoaxial subluxation may exist in patients neutral position (without neck movement) or may occur in relation to neck rotation movements (when the patient moves the neck to the right and left). I hope that, by now, the reader has understood the importance that clinical measurements, actual pathology and clinical triggers should go hand in hand. This category only includes cookies that ensures basic functionalities and security features of the website. The ligaments supporting these joints are quite strong, but if they become 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. In my experience, although I usually disagree with their diagnoses, is that Medserena in London has the absolute best upright imaging quality in the world. Spine (Phila Pa 1976). A common but severely ignorant misunderstanding that some clinicians make (the patient cannot be blamed for thinking like this, but the clinician should set it straight), is the notion that mild to moderate ligamentous instabilities makes the neck (or the whole body for that matter) tense up to protect against the ligamentous instability, even though there are minimal or no clear MRI findings to support this notion, and that this somehow causes all of the patients symptoms. A review of the diagnosis and treatment of atlantoaxial dislocations. It should be stressed that C1-C2 fusion, indicated by symptomatology, results in the natural cancellation of C1 over C2 movement so it results in approximately a deficit of 50% of the rotation of the neck. And, of course, to determine whether or not the findings actually correlate with the patients symptoms and clinical exam. About It is, technically, possible to perform traction, reduction and fusion to obtain the same result, but this would be like killing a fly with a canon. It is also important to know and evaluate patients concomitant diseases or comorbidities which are frequent in patients affected by Ehler Danlos, such as POTS, Mast Activation Syndrome, cardiac abnormalities etc. Now, the I was told is clearly second-hand information, and I cannot guarantee its accuracy. Having a strong neck and good posture helps a lot as well (details on what this entails can be read in my article on atlas instability). This website uses cookies to improve your experience while you navigate through the website. This madness must stop. The renowned scholar and neurosurgeon professor Atul Goel was the first person, to the best of my knowledge, to acknowledge and document the notion of horizontal misalignment of the craniocervical facet joints and that this would often be present despite a completely normal-looking mid-sagittal slice (where most craniovertebral junction measurements are done). For TOS CVH the patient will generally feel better when stress is reduced along with taking beta blockers (confer with your doctor). Goel A. Facetal alignment: Basis of an alternative Goels classification of basilar invagination. If there is no medullary compression, not even in a flexion/extension scan, then we cannot say that the patient is of surgical degree, even if it is very low, unless they look so bad that it is reasonable to expect frank compression in the near future! Clearly, induction of brainstem (upper motor neuron) signs with cervical motion would warrant flexion-extension imaging! Foramen magnum decompression or syrinx manipulation was not performed in any patient. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. the basion-dens interval, is the distance between the tip of the clivus and tip of the C2. We have remained at the forefront of medicine by fostering a culture of collaboration, pushing the boundaries of medical research, educating the brightest medical minds and maintaining an unwavering commitment to the diverse communities we serve. Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. This is what I said from the beginning; AAI is not the cause of these symptoms, the exam and triggers do not fit. This website uses cookies to improve your experience while you navigate through the website. 1927;11(1):155157. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. https://doi.org/10.13104/jksmrm.2011.15.1.41. It is better to let your doctor know if your son/daughter is having symptoms. Explore fellowships, residencies, internships and other educational opportunities. Request an appointment or second opinion, refer a patient, find a doctor or view test results with MGfC's secure online services. For treatment of the facetal dysfunction I recommend postural correction for the head neck and shoulder blades, along with exercises for the trapezius, levator scapulae, suboccipital and deep neck flexor muscles. 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. AA instability is typically diagnosed by performing radiographs (x-rays) of the neck. J Korean Soc Magn Reson Med. This site complies with the HONcode standard for trustworthy health information: verify here. Be sure to understand the mechanism of induction of symptoms in AAI and CCI before jumping on this potentially dangerous, and often financially devastating bandwagon! In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. J Neurosurg Spine. 2012). It baffles me when I see patients with 130 degree CXA and some additional signs of mild/moderate laxities being butchered with C0-T1 surgery despite there being NO instability in the cervical spine and only mild findings in the upper neck that are not causing any neurovascular conflicts nor facetal lockups (eg., Cock Robin syndrome). Exam for bow hunters syndrome is done dynamically, but thats aother exam. 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. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. Hopefully, this is the result of ignorance combined with poor clinical workup skills (incompetence) and not mere greed and malevolence. Burry HC, Tweed JM, Robinson RG, Howes R. Lateral subluxation of the atlanto-axial joint in rheumatoid arthritis. This website uses cookies to improve your experience. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. For example, although the medical literature (almost exclusively biased reports written by people considered experts on the topics (I am also biased on the topic; all experts are) may suggest a clivo-axial angle lower than 150 degrees as abnormal, this is still a measurement used to associate concrete craniocervical angles with medullary compression. 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. If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. Both positional (ie., upright. This increased mobility causes headache and cervical pain as well as signs of compression of adjacent neural elements that form cervicomedullary syndrome. To the best of my knowledge, I was the first person to document the notion that this was, in essence, a postural phenomenon that is induced due to poor posture over a long period of time (Larsen 2018). No improvement! A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. A critical view on the overdiagnosis of AAI/CCI. How is one supposed to know, if no one knows what you have in the first place? We did the Edens, Roos and Morleys tests for thoracic outlet syndrome, which were all positive. 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. Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. As touched upon in the beginning of this article, that prompted me to write this article, is a huge massive influx of patients over the last few years who have been illegitimately diagnosed with AAI or CCI. This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. 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. For example, if the brainstem is compressed due to a ruptured transverse atantal ligament or due to basilar invagination, a brutally high Grabb-oakes measurement would be expected, and would be a nice extra detail in the report along with the actual information that there is indeed anterior-posterior compression of the brainstem. 2008 Aug 15;33(18):2012-6. doi: 10.1097/BRS.0b013e31817bb0bd. 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). What does this mean? Dysautonomia when standing up is often related to craniovascular problems, whereas difficulty holding the head up suggests mumscular damage. 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. 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. These are typical signs of craniovasculo-hypertensive disorders. Apr 2, 2022 Any experience of Atlantoaxial instability? Does it matter whether these are done laying or sitting down? Would this mean that upper cervical chiropractors (orthogonal, blair technique, gonstead, etc.) Would need a flexion extension MRI and correlate to the patients symptoms. The BDI indicates vertical-, and the BAI horizontal structural integrity. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. 333 Earle Ovington Blvd, Suite 106. A caveat here may be if the the translational value is very high, as this would be a reasonable indication of foreseeable joint damage, but there is no consensus in the literature with regards to how much that is. 2011 Apr;15(1):41-47. fusion from the head, all the way down to the T1 or T2 vertebrae, even though there may be zero evidence for major neurovascular conflict. Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility. Surgical reduction and fixation would be the only appropriate treatment. Surgery to address problems in this area can be risky. Conveniently, she was sent out to a colleague for very expensive nonsense therapy (again, regardless of lacking serious findings that would require surgery) and sent tens of thousands of euros on stemcell and prolotherapy procedures in a desperate attempt to avoid the inevitable wheelchair. BDI, ie. Both patients had severe symptoms regardless of lying down, wearing a neck brace, etc., and did not get worse nor better when turning or moving their necks. Another problem with regards to rotation, is that the measurements are often done wrong. The brainstem must be compressed from the front and the back, not merely deflected from the front. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. Last Update [site_last_modified date_format=Y-m-d H:i:s]. Hopefully, this piece will prevail in explaining logical arguments for legitimate findings in CCI and AAI, and therein lead to a gradual decline and prevention for related misdiagnosis. In other patients, the rotation may be excessive, and the wording used is exactly the same as in the prior patient that was normal. 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. Mild to moderate cases tend to respond well to appropriate conservative therapy (not general therapy), cf., once again, my atlas joint article from 2017 linked several times earlier. Atlantoaxial and craniocervical instability are both real and potentially sinister diagnoses that require treatment. Dr. Christopher Williams | 07/09/2020. 2019 Feb 22;13(1):79-83. doi: 10.14444/6010. Atlantoaxial instability and craniocervical instability are spinal manifestations directly due to ligament laxity. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. Search for condition information or for a specific treatment program. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. A 3D rendered CT scan should easily demonstrate the luxation in cases where the sagittal slices appear normal or close to normal, whereas cases of dens migration will also appear obviously abnormal in the sagittal planes of imaging. Instability in the hip can result in dislocation, ligament tears, muscle damage and wear of the joint. She worsened with arm-loading, and often worsened when lying down, especially the breathing dysfunction tended to exacerbate and become more pronouned at night-time, resulting in anxiety and insomnia. Copyright Dr Gilete Neurosurgery & Spine Surgery. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. What cervical artificial disc should I choose? Adapted from Problems with the upper spine in children and adults with Down syndrome (DS) by E. Margolis, B. Henry, B. Sandella and M. Stephens. Journal of Neuro-Ophthalmology 2013;33:330337doi: 10.1097/WNO.0b013e318299c292, Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. Treatment, depending on the neurological symptoms and related pain, may be surgery. Therefore before proposing surgery, the evaluation of each case must be done really carefully. What cervical artificial disc should I choose? A 32 year-old female patient contacted me in 2019 as she had been diagnosed (by a radiologist alone) with craniocervical and atlantoaxial instability. We can consider that there is atlantoaxial instability or atlantoaxial subluxation (AA subluxation) in cases where there is principally incompetence of the ligamentous elements of the atlantoaxial (C1-C2) joint, which allow a significant increase in the mobility of this area thus considered pathological mobility. J Bone Joint Surg Am. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. If the patient has an elevated Grabb-oakes interval of 10mm and low CXA of 130 degrees, there is some horizontalization (upwards deflection) of the medulla, but no compression from both sides. As mentioned initially in this article, craniocervical instability is mainly associated with jugular outlet obstruction and basilar invagination, whereas atlantoaxial instability can cause posteriorization of the dens and brainstem compression, or rotational dysfunction resulting in either bow hunters syndrome, Cock Robin syndrome or other variants of segmental luxations. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. First of all, studies have shown that FLAIR hyperintensities (suggestive of ligamentous partial rupture or damage) have been found in a lot of asymptomatic patients (Myran et al. English +34 93 220 28 09 Espaol +34 93 198 34 24 But opting out of some of these cookies may affect your browsing experience. 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. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. Atlanto-axial instability is a potentially dangerous condition where the ligament between the atlas (C1`) and axis (C2) vertebrae at the top of your neck is partially torn. Regardless, be it rooted in benevolent or malevolent intention, this does not change the fact that pursuing the diagnosis and especially its related treatment (conservative or surgical strategies) are extremely expensive and potentially dangerous as well. 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. Posture is done for the rest of your life. Articles Look for signs of retinal hypertension (subtle copper wiring, AV nicking, tortuosity of the arterioles, generalized vasospasm or papilledema. If the patient is indeed positionally symptomatic, however, and there is compatible imaging evidence, either atlantoaxial fusion, transverse foraminotomy or certain physical therapies may be warranted depending on how severe the findings and symptoms are. The exam should be done lying down, without a neck pillow. We offer diagnostic and treatment options for common and complex medical conditions. Must be carefully evaluated and correlated with the patients symptoms). Basil R. Besh, M.D. DOI: https://doi.org/10.35975/apic.v24i1.1230. Maybe they temporary fix some compression? 2019 Oct;130:129-132. doi: 10.1016/j.wneu.2019.06.100. Dynamic angiograms could also be applicable in certain circumstances, cf. Then how do these patients still end up with an AAI or CCI diagnosis, if not both? Radiologic spectrum of craniocervical distraction injuries. Safe Care CommitmentGet the latest news on COVID-19, the vaccine and care at Mass General.Learn more. We'll assume you're ok with this, but you can opt-out if you wish. Faris AA, Poser CM, Wilmore DW, et al.. Radiologic visualization of neck vessels in healthy men. ADI laxity is mainly caused by head and neck trauma, so as long as you avoid future collisions, it will probably not deteriorate. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. Magnetic resonance imaging assessment of the alar ligaments in whiplash injuries: a case-control study. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. Contact, Terms & conditions The triggers would be especially relevant, seeing as various symptoms can heavily overlap between hundreds if not thousands of diagnoses. 1. Stay put for 30-60 seconds, look for worsening of symptoms while in the test. Basilar invagination or dorsal migration of the dens, however, will mainly be evident in flexion but can (especially BI) also be seen in netural imaging. Second, because it is such a controversial topic that lacks medical consensus, poor understanding of the actual mechanism of pathology leads to misunderstandings. This webpage is intended to provide health information so that you can be better informed. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. When Atlantoaxial instability occurs along with craniocervical instability, also known as occipitocervical instability (ie instability present also between skull and first cervical vertebra or Atlas), then fusion should consist of adding a fixation to the cranial bone through occipital or condylar screws which would give us as a whole C0 -C1-C2 posterior fusion. But we must see adequate imaging as well as adequate clinical fulfillment of diagnostic criteria to render these diagnoses; it is not enough to feel neck clunking, upper cervical pain, weakness in the neck or wobbleheaded. Let us help you navigate your in-person or virtual visit to Mass General. Because of its role in movement, it is, unfortunately, commonly injured. Due to the poor practice integrity that is often associated with DMX imaging, despite these modalities indeed having some utility in certain cases, I cannot recommend having them done unless done in a serious hospital without a financial incentive (ie., without financial connections to the clinician ordering them), and without a very obvious scope of investigation that could not already be seen in MR or CT imaging. to get a better impression of its actual thickness. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. It is mandatory to procure user consent prior to running these cookies on your website. Necessary cookies are absolutely essential for the website to function properly. As always, it is important to do a clinical radiological correlation to make an accurate assessment. If this was the case, ie., if the brainstem and medulla was being stretched, then the patient would highly likely get neurological symptoms that improve with extension and worsen with flexion (as patients with legitimate tethered cord syndrome do), and would certainly have a positive Slump test, a test which stretches the spinal cord. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. (I will post the before- and after images when I return to Colombia in August, as they are on a separated hard drive). When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. Flexion-extension and cervical rotation on both sides should be evaluated. I see massive amounts of patients with alleged AAI who have normal atlantoaxial facetal overlap, and of course, also lacking clinical correlation. Atlanto-axial rotatory fixation. Ross & Moore. Information about the identification of CVJ fractures will not be applicable for patients with chronic workups and lacking imaging findings over a long period of time. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). Lateral cervical x-ray and flexion-extension views can give us complementary information in regards to atlantoaxial instability, although it does not seem indicated as the first choice method of diagnosis. I told her that, although I dont think theres any evidence to suggests that the AAI is causing your symptoms, we should still treat it to prevent the risk of future frank luxations of the joints. Headache, cerebrospinal fluid leaks, and pseudomeningoceles after resection of vestibular schwannomas: efficacy of venous sinus stenting suggests cranial venous outflow compromise as a unifying pathophysiological mechanism. I have also seen cases of seventh nerve dystonic mimicks several times in JOS, where platysmal dystonia or even oropharyngeal dystonia (hypoglossal nerve) has been identified, worsened with neck tucking (which increases the compression) and resolved with specific strategies for widening the atlanto-styloidal interval (see my atlas article as linked earlier) or Larsen 2018 in the reference list). Kjetil has also published several peer-reviewed studies on musculoskeletal and neurological topics. In cases of hyperlaxity, It is not uncommon to find subaxial cervical alterations (levels below C3 to C7 . You mention to test for craniovascular pathologies, we should get a Doppler examination of the carotid and cerebral arteries done, and a CT angiogram done. I believe that most of these practitioners mean well. However, I also told her that she may end up having fixation surgery in the future to prevent foreseeable compressive damage to the brainstem. 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). 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). Just like the CXA, this measurement is supposed to aid with objective measurements rather than just eyeballing the images, and writing down your impressions. 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. 2019) have documented numerous symptomatic cases of jugular vein stenosis at the craniovertebral junction. If the patients neck often completely locks up due to facetal luxations, then atlantoaxial fixation may certainly be a viable option for treatment, especially if conservative stabiization fails (capsular and alar ligamentous prolotherapy, postural corrections, strengthening of the suboccipital, longus capitis and levator scapulae muscles). Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. From the beginning, the patient doubted my diagnosis that this was a craniovascular problem because she felt pain in the suboccipital area, had cracking and clunking, and felt compatible with several things she had read online and on facebook forums. 404-256-2633. Atlas and axis screws are joined in each side by lateral bars that are unifying the instrumented fusion system. 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. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. Burry et al (1978) documented a rare case of lateral luxation in a patient with rheumatoid arthritis, in which the supporting facet had eroded away. Patients with rotary subluxation will develop torticollis and will generally appear fixed/rigid upon physical exam and may not be able to rotate their necks at all. More commonly, however, a due to asymmetrical tearing of the covering ligaments, rotational subluxation or frank luxation is seen according to the Fielding & Hawking classifications (1977): Type 1, 2, 3 and 4, wherein types one and two are the most commonly encountered ones. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. 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. Surgery is often challenging because of the shape of the C1 and C2 bones, and because the vertebral arteriespass in and around these two bones on the way to the brain. This can also damage the brainstem and produce symptoms similar to what is described above. This is a component of TOS CVH in most circumstances, in my experience, but can certainly scare the patient into believing that they have sinister CCI or AAI due to the location of the pain along with heavy cracking and other symptoms. With the increasing dependence on smartphones, computers, and other devices in our modern It will rarely cause frank luxation, however where the facets dislocate and lock laterally. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. It is not due to mild overall instability that does not cause neurovascular conflicts. It is possible to do it with extension and rotation, etc., but it is usually not necessary. Albeit still a surgically treated problem. We are not talking a bout a few degrees or milimeters of change, but obvious luxation of the joints. 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