JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. Dysautonomia when standing up is often related to craniovascular problems, whereas difficulty holding the head up suggests mumscular damage. If the measurements are within normal limits, the likelihood of dangerous sequelae are low, if not absent. Burry HC, Tweed JM, Robinson RG, Howes R. Lateral subluxation of the atlanto-axial joint in rheumatoid arthritis. 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. Accessory nerve compression can cause weakness of the trapezius and sternocleidomastoid muscles, but can also cause cervical dystonia. When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. Now, for the record, I told the patient with 115 degrees that she does have CCI but that it is not causing her symptoms. Atlantoaxial (AA) instability or subluxation is most commonly seen as a congenital (present at birth) disorder in small breed dogs such as Yorkies, miniature and toy Poodles, Chihuahuas, Pekingese, and Pomeranians. Gweon HM, Chung TS, Suh SH. Due to the instability in the craniocervical junction deformation can occur to the brainstem, upper spinal cord, and cerebellum. Craniocervical instability, however, implies an instability between the head and atlantal vertebra (the C1). In the congenital form of AA instability, the animal is born with abnormal bony or ligamentous connections between the first two vertebrae in the neck. 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). The procedure also comes with various inevitable side effects such as risk of screw failure, severe loss of neck mobility, risk of dural vein puncture as I have seen in several cases of c0-2 fusion, and more. I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. The exam should be done lying down, without a neck pillow. Moreover, tractioning the neck of these vulnerable patients can often cause undesirable effects. Most cases of mild to moderate unilateral compression, sometimes even intermittent occlusion, is asymptomatic due to contribution from the contralateral VA (Faris et al. (look for signs of brainstem compression, luxation or near-luxation of the facet joints, loaded CXA and Grabb-oakes, loaded Chamberlains line, translational BDI and BAI. Knowing this it allows to anticipate any possible problems in the postoperative period. Surgical reduction and fixation would be the only appropriate treatment. 2014 Feb;11(1):75-82. ncbi.nlm.nih.gov/pubmed/24321024, Higgins JN et al. Therefore before proposing surgery, the evaluation of each case must be done really carefully. I am not saying that this applies to every DMX center nor that DMX in and by itself is never useful, but due to the overwhelming lack of competence that tends to come with these studies, I dont recommend them unless unless you have obviously abnormal imaging otherwise and want to look for occult fractures or similar sinister and stubbornly identified problem. In late stages, even the CTV will show severe compression, and at this stage, surgery may be the best option for resolution if there is clinical correlation. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. Specialist imaging research to help diagnosis. 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. It is advisable to obtain just a lateral view first. Privacy policy, Do you really have atlantoaxial and craniocervical instability? 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. 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. A CTV is preferable, but a general neck CT will also do if you have sensitive kidneys and would like to avoid contrast infusion. This category only includes cookies that ensures basic functionalities and security features of the website. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. If your son/daughter does not need surgery, it is important for him/her to be very careful playing sports or doing other physical activities. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. Would this mean that upper cervical chiropractors (orthogonal, blair technique, gonstead, etc.) Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. And, she still had the same symptoms! This may cause the patient to become afraid and to google their symptoms, which in and by itself is reasonable enough. 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. 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. Surgical management is recommended for those with severe signs and for those who have tried and failed medical management. Commonly misunderstood and overemphasized measurements. The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. English. 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. Necessary cookies are absolutely essential for the website to function properly. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. Her symptoms, however, did not at all change when changing her neck position and she had never had torticollis. 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. Spinnato P, Zarantonello P, Guerri S, Barakat M, Carpenzano M, Vara G, Bartoloni A, Gasbarrini A, Molinari M, Tedesco G. Atlantoaxial rotatory subluxation/fixation and Grisels syndrome in children: clinical and radiological prognostic factors. Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. The General Hospital Corporation. Ann Rheum Dis. J Bone Joint Surg Am. 2015. Strong evidence of clinical correlation must be present from a clinician that is familiar with the signs and triggers in upper cervical instability-cases. Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. English +34 93 220 28 09 Espaol +34 93 198 34 24 Rev. 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. This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. 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. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. Surgical options, sometimes including relevant-level fusion, may be warranted in these circumstances. It is imperative to understand that patients with dagerous craniovertebral junction injuries, although one may sometimes require a dynamic CT or x-ray to identify them, will have clear imaging findings combined with clear clinical triggers in the utmost majority of incidences. 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. Would need a flexion extension MRI and correlate to the patients symptoms. 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. Does it matter whether these are done laying or sitting down? Basil R. Besh, M.D. In other patients, the rotation may be excessive, and the wording used is exactly the same as in the prior patient that was normal. The joint between the upper spine and base of the skull is called the atlanto-axial joint. Something I often see reported as alleged evidence of sinister CCI, is a translational BDI or BAI (the basion-axial interval is the horizontal distance between the tip of the clivus and the posterior wall of the odontoid process. Just like the CXA, this measurement is supposed to aid with objective measurements rather than just eyeballing the images, and writing down your impressions. 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). This is a major component in the workup for TOS CVH). Sometimes, the symptoms may trigger within a few minutes after the test as well, depending on various factors which exceed the scope of this article. 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). 2009), but this is extremely rare. Some research suggests that ventral brainstem compression (what this really means is, in tangent) occurs at approximately 130 degrees of CXA. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. It is not due to mild overall instability that does not cause neurovascular conflicts. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. 2000). Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. 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). Donald Corenman, MD, DC. Not sure what you mean here. A review of the diagnosis and treatment of atlantoaxial dislocations. Why rely on Washington University experts for treatment of your atlantoaxial instability? Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. 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. 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 Common arguments for treatment may be claims that, although the MRI and even upright MRIs are normal, their own DMX scan is positive, or that the MRI, which was deemed normal by the local hospital, in reality shows signs of ruptured ligaments and that this fits with the patients symptoms. This is no longer true. 2012). Bow hunters syndrome revisited: 2 new cases and literature review of 124 cases. 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