1963;13(5):386396. 1978 Dec;37(6):525-8. doi: 10.1136/ard.37.6.525. In severe (very bad) cases, your son/daughter might need neck surgery. Bow hunters syndrome revisited: 2 new cases and literature review of 124 cases. At Dr Gilete we are experts in Ehlers Danlos surgery, craniocervical instability EDS,neuro and spine disorders related to EDS and whiplash. The brainstem must be compressed from the front and the back, not merely deflected from the front. Does it matter whether these are done laying or sitting down? 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. If the measurements are within normal limits, the likelihood of dangerous sequelae are low, if not absent. Research has shown that normal limits are 3 and 10mm, with an absolutely maximum of 12mm (Ross & Moore 2015). These cookies do not store any personal information. Patients with genuine and symptomatic rotational vertebral artery compression will develop symptoms of vertebrobasilar insufficiency when they fully rotate their heads to one or both directions, and may be further worsened if done simultaneous with neck extension (DeKleyn 1927). This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. Headaches certainly can develop from instability of C1-2. Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. 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. Would need a flexion extension MRI and correlate to the patients symptoms. Epub 2020 Oct 16. PMID: 32623537; PMCID: PMC8121728. My poor baby has become completely lame and incontinent in the last 48 hours. These cookies will be stored in your browser only with your consent. 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. Basil R. Besh, M.D. Our surgeons can discuss with you the various treatment options for your specific condition. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. Aggressive craniovertebral junction ligamentous injuries can also result in vertical displacements. We'll assume you're ok with this, but you can opt-out if you wish. In some circumstances, gradual degenerative basilar invagination can also occur due to gradual and progressive degenerative horizontal misalignment of the atlantoaxial joints (Goel 2014), due to certain diseases such as rheumatoid arthritis, but it is usually caused by head and neck trauma. We moved on to perform the Valsalva maneuver (a pressure test), the Queckenstedts test (manual venous compression test), and the cervical retraction test (TOS CVH), in which the first and third tests were positive, reproducing severe head pressure, dizziness, presyncope and profound fatigue. No improvement! 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. Tambin conocer las causas, los signos y los sntomas de la IAA. Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. The diagnosis can be made by means of an Upright MRI (magnetic Resonance Imaging) or with a cervical CT scan with 3D reconstruction. Although the complete differentiation between this and CCI or even occipital neuralgia is something that is complicated and must be done on individual basis after examination, we can, in essence, say that suboccipital pain that worsen with shoulder loading tends to be TOS or occipital neuralgia, whereas suboccipital symptoms that induce when lying down or being upright regardless of neck position tends to be TOS CVH. The deep neck flexors should not engage as this lessens the compression. In my experience, we would expect to see at least 20mmHg maximum venous pressures. 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). Clearly, the expenses involved, including the health risks, may be well worth it if the diagnosis is correct and the patient has legitimate CCI or AAI with strong clinical and radiological evidence. 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. And, of course, to determine whether or not the findings actually correlate with the patients symptoms and clinical exam. The brainstems were completely void of evidence for compression in both cases, and there was no evidence of signal changes (consistent with brainstem damage) on MRI. Request an appointment or second opinion, refer a patient, find a doctor or view test results with MGfC's secure online services. Atlantoaxial malalignment is best visualized on a lateral view. It is not due to mild overall instability that does not cause neurovascular conflicts. I consulted with her and reviewed her imaging: The quality of the images, first and foremost, was very low. Flexion and extension imaging fails to demonstrate any sort of brainstem compression. And, fair enough, I do not expect blind trust nor compliance. Kjetil Larsen is a Researcher and a injury rehabilitation specialist, and is the owner of MSK Neurology. 333 Earle Ovington Blvd, Suite 106. 9/2017. It mainly consists of the posterior fusion of the affected vertebrae, in this case, the atlas (C1) and the axis (C2). Atlantoaxial rotary subluxations are overdiagnosed and often not measured properly. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. For more information about these cookies and the data
Posture is done for the rest of your life. In the Axis, pedicle screws are usually the first choice although, depending on the patients anatomy, placement of isthmic screws may be considered. Copyright statement Learn about the many ways you can get involved and support Mass General. This madness must stop. In many circumstances, conservative treatment (Larsen 2018, atlas joint article as linked earlier) is appropriate. These are typical signs of craniovasculo-hypertensive disorders. 2020). Often, by radiologist alone, based on sparsome imaging findings (eg., alar ligament T2 FLAIR hyperintensity or mild to moderate lateral facetal overhangs) and a lacking compatible clinical workup. In addition to reproducible clinical triggers (positions), the patient should preferably undergo a dynamic catheter angiography of the neck. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. 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. 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). Moreover, tractioning the neck of these vulnerable patients can often cause undesirable effects. Now, the I was told is clearly second-hand information, and I cannot guarantee its accuracy. Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. J NS 2015, V8 issue 4. 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. Musa et al. 2008). Pain medications and anti-inflammatories are typically also prescribed. 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. Postural orthostatic tachycardia syndrome (POTS) and its relation to craniovascular dysfunction, Pectineo-femoral pinch syndrome: A common cause of groin & anterior thigh pain and weakness, Chronic spinal pain and radiculopathy: Diagnostic approach and common imaging pitfalls, Neurogenic genital pain: Pudendal neuralgia and inferior hypogastric plexalgia. Atlantoaxial instability (AAI) is the term for increased motion at the joint between the 1st and 2nd cervical vertebrae (the atlas and the axis). 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. Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. The joint between the upper spine and base of the skull is called the atlanto-axial joint. Now, it is true that specialty diagnoses can be missed by local generalists. It is different from other joints in the vertebral 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. Post count: 8446. Get the latest news, explore events and connect with Mass General. An X-ray is low-cost and low-risk, but it does not always tell whether a person has AAI or not. 3. A lot of things that cause temporary results are just placebo. 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. Dissection of the vertebral and carotid arteries is fairly rare and can be excluded through a doppler ultrasound or CT angiogram. Atlantoaxial instability and craniocervical instability are spinal manifestations directly due to ligament laxity. Seemingly unrelated, Higgins et al (2013) and others (Dashti et al 2012, Li et al. BHS implies rotational compression of the vertebral arteries, which are two out of four arteries that supply the brain (two internal carotid and two vertebral arteries). 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. E7. But opting out of some of these cookies may affect your browsing experience. This, of course, must be evaluated on a case-to-case basis. 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. We use cookies and other tools to enhance your experience on our website and
Ann Rheum Dis. Atlantoaxial instability treatment Contact Dr. Gilete C1 C2 fusion surgery Contact Dr. Gilete Our commitment to reliable health and medical information on the internet This site complies with 2014 Apr;5(2):59-64. doi: 10.4103/0974-8237.139199. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. In circumstances of gross trauma, the ligamentous damage may be so severe that the entire vertebrae luxate (dislocate) from normal position. Dr. Nic Gay and Dr. Masi Reynolds specialize in getting to the root cause of the problem PMID: 24475346; PMCID: PMC3899735. The ligaments holding the bones together can also be injured in trauma, or weakened in certain inflammatory conditions such as rheumatoid arthritis or Downsyndrome. Education These cookies will be stored in your browser only with your consent. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. November 19, 2014 at 8:19 pm. 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). Josy GF, Daily AT. It is, as we say, in tangent with the dens and tectoral ventrally alone. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. Signs of ligamentous damage. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. If unavailable, a CT angiogram can be used, but is less sensitive. 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. The CXA was 138 degrees and the Grabb-Oakes measurement was 8,3mm. There can be, and are indeed many more potential explanations for these symptoms than just AAI and CCI. Then the patient can make an informed decision about whether or not they want to invest in experimental therapy. 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. Goel A. Facetal alignment: Basis of an alternative Goels classification of basilar invagination. 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. Flexion-extension and cervical rotation on both sides should be evaluated. 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). Atlantoaxial (AAI) and craniocervical instability (CCI) are two potentially sinister diagnoses that cause damage to the segmental neurovascular structures due to overmobility of the upper cervical spine. However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. Atlas and axis screws are joined in each side by lateral bars that are unifying the instrumented fusion system. Postoperative hospital stay is usually around 7 days. We examined 404 patients with this chromosome disorder and observed their atlanto-dens intervals and spinal canal widths to be significantly different from children without Down syndrome. (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. Int J Spine Surg. 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. J Bone Joint Surg Am. 1977;59 (1): 37-44. 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. 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. Claims of three, four or even five-level spondylolisthesis due to a 50 micrometer (0.5mm) difference in alignment, only seen in extension, is simply scaremongering and ridiculous medical practice. 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. If its caused by rotation (rare), manipulation may temporarily improve jugular outlet passage, but it will not last. In these cases, the direct signs and indirect signs of atlantoaxial subluxation must be objectified. Sometimes flexion-extension and rotational imaging is necessary. With the increasing dependence on smartphones, computers, and other devices in our modern Global Spine J. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). The triggers would be especially relevant, seeing as various symptoms can heavily overlap between hundreds if not thousands of diagnoses. Another scenario could be that the patient has been diagnosed with atlantoaxial rotary subluxations, as little facetal overlap, lets say, 15%, is seen upon bidirectional rotation. 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). What is atlanto-axial instability? KL TRENING & REHAB It is important to understand that the size of the facets is what determines what degree of rotation would be excessive. The atlantoaxial complex refers to the first two bones of the neck (C1,the atlas, and C2,the axis) as well as the associated collection of ligaments that connect the bones together and the blood vessels that travel through them to the brain. This is really more of a poor posture/misalignment problem than a case of instability (Larsen 2018), but because it is a legitimate upper cervical problem then I will still mention it in this article. In patients with Ehler Danlos syndrome, instability is present frequently in several segments, generally C0-C1-C2 (from occipital to axis). 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. to get a better impression of its actual thickness. 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. Atlas screws are generally placed in the lateral masses. 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). In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. Case Rep Neurol 2019;11:295298, Waldock WJ, Higgins NJ, Axon P. A case report of gastroparesis resolved by styloidectomy. Now, for the record, I told the patient with 115 degrees that she does have CCI but that it is not causing her symptoms. Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. In the cases where it is not possible to obtain autologous bone graft, heterologous graft (artificial bone) may also be used. Supine cervical MRI including T2-w sagittal-oblique sequences at 2mm slice thickness (disc and foraminal health is best evaluated on a supine MRI). doi: 10.1227/NEU.0b013e3182333859. Atlantoaxial fixation: overview of all techniques. 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. This, as significant irritation of the brachial plexus can also cause autonomic coaffection (Larsen et al 2021) and thus derange the function of the phrenic nerves, which in turn control the diaphragm. 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. World Neurosurg. The reason why AAI and CCI are potentially associated with so many symptoms such as headache, dizziness, etc., is due to the potential for neurovascular conflict. The same principles would apply for AAI and CCI: There must be clear imaging findings, and I am not talking about a simple measurement being off, but real pathology proven to be associated with the given diagnosis. If it is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted. The dorsal lamina of the atlas shifts caudally and ventrally against the spinous process of the axis. 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. Two important questions arise: Does the patient actually develop (even if just from time to time) develop frank facetal luxations causing the neck to lock up? 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. This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. Journal of Neuro-Ophthalmology 2013;33:330337doi: 10.1097/WNO.0b013e318299c292, Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. (Fixed rotatory subluxation of the atlanto-axial joint). Our surgeons provide a full range of treatments including non-surgical options as well as surgical repair. 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. Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. 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. Merely feeling worse when standing up, even if indeed feeling awful, is not a strong indicator of AAI CCI As mentioned above, it is the influence of cervical positioning. our TOS CVH paper (Larsen et al 2020). PMID: 18708935. Deliganis AV, Baxter AB, Hanson JA, et al. This is no longer true. 2011 Apr;15(1):41-47. Once in the Operating Room, surgery is performed under general anesthesia, with Neurophysiological monitoring (SSEP somatosensory evoked potentials), neuronavigation guidance and intraoperative fluoroscopy guidance. Instability in the hip can result in dislocation, ligament tears, muscle damage and wear of the joint. 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). What cervical artificial disc should I choose? I prefer to compare mid-jugular to the highest pressure found, usually in the torcula or SSS. 2015. This iatrogenic practice must come to an end. Surgical management is recommended for those with severe signs and for those who have tried and failed medical management. In addition to that we would start treatment for thoracic outlet syndrome. 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). (2019) documented another case where a patient with RA developed odontoid fracture and subsequent anterolateral subluxation of the atlantoaxial joint. #11760. In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. You also have the option to opt-out of these cookies. These problems are much more constant than AAI CCI, which are, for the most part, positional problems. 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). Most dogs with AA instability will develop clinical signs within the first 2 years of life, often after a seemingly mild traumatic event.
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