Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. 2. Uniondale, NY 11553. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. Research has shown that normal limits are 3 and 10mm, with an absolutely maximum of 12mm (Ross & Moore 2015). Clunking, clicking and pain in the upper neck. Atlantoaxial instability (AAI) is a potential complication of all forms of EDS. The natural anatomic C1-C2 movement is basically rotation and approximately implies 50% of necks total rotation movement. When the bones or ligaments of the atlantoaxial complex are injured, the spinal cord is at particular risk for injury, and surgical treatment is often indicated. Does thoracic outlet syndrome cause cerebrovascular hyperperfusion? -Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. Some top offenders may suggest full craniocervical fusion, ie. Moreover, craniovascular disorders often fluctuate depending on whether or not the patient is upright or lying down (sometimes lying down is worse, sometimes standing up makes it worse), and do certainly not return to normal, symptom-free status when the neck is placed in neutral position. It is crucial to understand that the general minor instabilities involved in AAI and CCI are not the cause of symptoms. 2014 Feb;11(1):75-82. ncbi.nlm.nih.gov/pubmed/24321024, Higgins JN et al. Atlas screws are generally placed in the lateral masses. I have not receiving anything that comes close of what they produce. 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. zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. The atlantoaxial joint is normally stabilized by a projection off the axis called the dens, which fits into the atlas, as well as several ligaments between the two bones. There are two causes for the instability, trauma and birth abnormalities. Traumatic instability occurs after forceful flexion of the head, The deep neck flexors should not engage as this lessens the compression. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. Necessary cookies are absolutely essential for the website to function properly. Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. Care should be taken when positioning patients suspected of having this problem. Rather, just like with the CXA, it is an indication of the present spinal health status and perhaps also an indicator as to non-surgical prognosis as well as an indicator of likely outcome if nothing is done. The ligaments holding the bones together can also be injured in trauma, or weakened in certain inflammatory conditions such as rheumatoid arthritis or Downsyndrome. 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. 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. 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. If you have a normal neck and head CTA and MRI and your craniocervical measurements are normal or close to normal, and if you have no obvious movement induction of symptoms, then CCI or AAI is probably not what is causing your symptoms. collected, please refer to our Privacy Policy. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. 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. If the X-ray results are abnormal (different than usual), the doctor will order another imaging test, like a computed tomography (CT) scan or magnetic resonance imaging (MRI) test. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. 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. The problem begins when certain nonsensical articles about CCI and AAI, that do not properly explain relevant clinical correlation nor imaging requirements, but rather, just lists a set of associated symptoms, finds favor in the patient. Powers ratio will be abnormal in cases of both BI and craniocervical dissociation (Ross & Moore, 2015). Atlantoaxial fixation: overview of all techniques. DRAMMEN, NORWAY, Home Postoperatively, the patient stays at the ICU unit for 1 day and then he/she stays in the Neurosurgical Ward. Compression of the glossopharyngeal nerve will frequently cause pharyngeal pain (back of the throat pain) whereas vagal compression may lead to dry coughing, lump in the throat feeling, ear itching and various strange things when unilateral, but has been associated with more problematic issues when bilateral such as gastroparesis (Waldock et al. We use cookies and other tools to enhance your experience on our website and If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? No improvement! 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. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. Beware that suboccipital pain, espeically if your imaging is normal, is a very common sympton in thoracic outlet syndrome, and is actually a migraine variant. 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). However, if there is obvious compromise of a ligament but there is no evidence of sinister hypermobility or structural displacement (eg., very high ADI), the ligamentous should be further examined with high-resolution T2 FLAIR imaging with low slice thickness (supine imaging!) The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a posterior fusion of the first cervical vertebra (C1 or Atlas) and the second cervical vertebra (C2 or Axis). Call 314-362-3577 for Patient Appointments. 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). It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. This pain tends to get worse with stress and with high heart rates, and are often also worse in the morning after lying down. Both neurophysiological monitoring and neuronavigation guidance are safety measures for the patient. KL TRENING & REHAB 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. BDI, ie. 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. 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. Another patient was told by a well-known pain physician in the US that she had brainstem compression and required several expensive prolotherapy procedures. 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). Traditional cases of atlantoaxial instability and craniocervical instability require obvious imaging findings with strong clinical correlation, and, when its criteria are met, are certainly treated (operated) in any skilled and compatible neurosurgical ward. 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. This can happen due to excessive rotation at the joint with gradual worsening (eg., in a patient with Ehler Danlos syndrome or similar), or in combination with rotation and transverse-foraminal stenosis, which is the hole on the side of the transverse processes that the vertebral arteries and veins venture through. 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. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. 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. 1977;59 (1): 37-44. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. As always, it is important to do a clinical radiological correlation to make an accurate assessment. Many of these patients who have been misdiagnosed with AAI or CCI may feel neck wobbliness, heaviheaded, neck weakness, and clicking or clunking in the neck upon movement, often along with upper neck pain. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. If this X-ray is repeated, the AAI might go away. Ross & Moore. And, although there was zero evidence of brainsstem compression, she did indeed have subluxation of atlantoaxial joints with around 10% of overlap when turning to the side. Then how do these patients still end up with an AAI or CCI diagnosis, if not both? If the patient has a Grabb-Oakes of 18mm, however, and the transverse ligament is ruptured with the dens compressing the brainstem from the front and pushing it into the lamina behind it, then this is an emergency that requires timely surgical decompression. The alignment of the atlas itself isnt really the problem; the problem is whether or not a rotation or a horizontal glide is causing encroachment of the jugular outlet. 2008 Aug 15;33(18):2012-6. doi: 10.1097/BRS.0b013e31817bb0bd. Goel A. Facetal alignment: Basis of an alternative Goels classification of basilar invagination. The problem has received various names such as mere jugular vein compression, venous eagles syndrome, but I have called it jugular outlet syndrome (JOS), as it is a problem that not only affects the craniovenous outflow, but also several cranial nerves, and can be culpable in various strange neurological disorders (Read my atlas article (link) I also have an upcoming paper on this topic that I hope to release this or next year). J Korean Soc Magn Reson Med. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. Rather, she would feel awful in general and felt worsening with stress and arm- & shoulder loading, and being upright vs. lying down. I have seen several patients misdiagnosed and become almost paralyzed by anxiety due to an increased Grabb-Oakes measurement where the dens is just barely in tangent with the brainstem, despite zero evidence of actual compression nor signal changes in the brainstem and with normal neurological examinations without any upper motor lesion signs! Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. Sometimes, an X-ray shows AAI when there are no symptoms. More information about surgical treatment. DOI: 10.3171/2015.1.FOCUS14791. But, if a specialist points something out that is not conventionally considered, he should either 1. make sure to emphasize the notion that it is a subtle finding with unsure actual clinical applicability or 2. make sure to prove his points through objective findings. I recommend first measuring the degree of rotation between the C1 and C2 by drawing a line from the bifid process to the middle of the anterior aspect of the vertebra, and then another line from the posterior to the anterior tubercles of the C1. ARTICLE IN PROGRESS The piece is virtually finished, but I am missing some imaging that I dont have access to here while I am on vacation in Norway. 2014). I recommend doing this with a neuro-ophthalmologist, not a general ophthalmologist or opticician, as the findings are often missed. Moderator. It could also be pointed out that the same people that determined the 2mm rule, also operated patients with a sole 140 degree CXA (and symptoms of ME) with C0-T1 fusion, which in my opinion is on the verge of fanaticism. You also have the option to opt-out of these cookies. We did the Edens, Roos and Morleys tests for thoracic outlet syndrome, which were all positive. Patients with normal structural alignment and more or less normal or completely normal radiological imaging, without clinical correlation, end up diagnosed with CCI or AAI due to a slightly low (non-sinister) CXA, say 135 degrees, and some signal changes in the alar ligaments on T2 FLAIR imaging or slight increase in the atlantodental interval (ADI) despite normal thickness of the transverse atlantal ligament (TAL). Apr 2, 2022 Any experience of Atlantoaxial instability? 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. Congenital, inflammatory, traumatic, These cookies will be stored in your browser only with your consent.
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