Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. 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. 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. As stated, although rooted in postural dysfunction, this is not really a problem of pathological instability, and therefore I dont recommend neck fusion to treat this problem. This iatrogenic practice must come to an end. 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. Sometimes, an X-ray shows AAI when there are no symptoms. 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. I see massive amounts of patients with alleged AAI who have normal atlantoaxial facetal overlap, and of course, also lacking clinical correlation. A lof patients have clicking and clunking in the neck along with severe suboccipital pain. Would need a flexion extension MRI and correlate to the patients symptoms. 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). 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. 2008). Compare the two to obtain the degree of rotation. PMID: 25083363; PMCID: PMC4111952. We also use third-party cookies that help us analyze and understand how you use this website. One patient was told by a famous alternative european neurosurgeon that she has CCI and AAI, and although there is no evidence for current surgery, she would probably be in a wheelchair within a few years and might even die. Global Spine J. Request Appointment. PMID: 749697; PMCID: PMC1000289. 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. Case Rep Neurol 2019;11:295298, Waldock WJ, Higgins NJ, Axon P. A case report of gastroparesis resolved by styloidectomy. It is different from other joints in the vertebral These problems are much more constant than AAI CCI, which are, for the most part, positional problems. In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. 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. 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. Surgery to address problems in this area can be risky. Atlas screws are generally placed in the lateral masses. Spine (Phila Pa 1976). In these cases, the direct signs and indirect signs of atlantoaxial subluxation must be objectified. The deep neck flexors should not engage as this lessens the compression. It is not a substitute for medical advice and should not be used to treatment of any medical conditions. Your email address will not be published. We offer diagnostic and treatment options for common and complex medical conditions. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. 2005 Dec;53(4):408-15. Review. Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. Acute or chronic spinal cord compression causing clinical signs consistent with an upper cervical myelopathy can result from this instability [2]. The term AAI can also be used in cases of transverse ligament rupture, in which the odontoid process (the axis of the C2) may, especially if there is also damage to the tectorial membrane, dislocate dorsally and compress the brainstem. 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. Anaesth pain intensive care 2020;24(1)69-86. Dynamic angiograms could also be applicable in certain circumstances, cf. ADI laxity is mainly caused by head and neck trauma, so as long as you avoid future collisions, it will probably not deteriorate. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. 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. 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! More information about surgical treatment. In patients with Ehler Danlos syndrome, instability is present frequently in several segments, generally C0-C1-C2 (from occipital to axis). In the Axis, pedicle screws are usually the first choice although, depending on the patients anatomy, placement of isthmic screws may be considered. 2014 Aug;4(3):197-210. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. That said, yes, it is my opinion that the treatment is nonsense. Post count: 8446. At Dr Gilete we are experts in Ehlers Danlos surgery, craniocervical instability EDS,neuro and spine disorders related to EDS and whiplash. Ross & Moore. Burry HC, Tweed JM, Robinson RG, Howes R. Lateral subluxation of the atlanto-axial joint in rheumatoid arthritis. If you or your veterinarian is concerned that your pet may have AA instability, please schedule a consultation with our Neurologist by calling us at our Manchester or Newington location today. 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. Now, the I was told is clearly second-hand information, and I cannot guarantee its accuracy. 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). En este folleto, aprender sobre la IAA y cmo afecta a las personas con sndrome de Down. 2014). 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. 2. PMID: 25210334; PMCID: PMC4158632. These cookies do not store any personal information. Neurologic signs of a cranial cervical myelopathy typically present at a young age and can range from cervical pain (hyperesthesia) to paralysis. 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. This This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. Musa et al. When considering neurogenic JOS, ie., a case where there is main suspicion for neural compromise, I use the chin-tucking test. The doctor will tell you which sports and activities are safe for your son/daughter. 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! The atlas can sublux anteriorly, posteriorly, laterally, or vertically. Traumatic Atlantoaxial Lateral Subluxation With Chronic Type II Odontoid Fracture: A Case Report. In most cases it is convenient to put bone graft, usually autologous, taken from the iliac crest or the patients own rib. <9mm), which overestimate the pathologies and are much misunderstood due to unrealistic consensus of what is normal) will clearly be abnormal, such as the Harris measurement (BAI), basion dens interval (BDI), or Powers ratio. Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. The instability present between these vertebrae can cause the vertebrae to shift and injure the spinal cord. Thus, it is important to measure both the percentile overlap as well as the degree of rotation bidirectionally. 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. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. The atlantoaxial complex is primarily responsible forenabling the head to rotate, or turn to the left and right, while also protecting the spinal cord from injury. The same applies for conservative strategies to reduce internal jugular vein compression. Upright cervical MRI in flexion, extension and maximal bi-directional rotation. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. BDI, ie. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. There are no exercises that can help an instability like that. Medical management entails strict cage rest and placing a neck brace (from in front of the ears to the mid-chest) to prevent the vertebrae of the neck from moving and causing more damage to the spinal cord. This means routine X-rays are not helpful. Her symptoms, however, did not at all change when changing her neck position and she had never had torticollis. Safe Care CommitmentGet the latest news on COVID-19, the vaccine and care at Mass General.Learn more. Craniocervical instability, however, implies an instability between the head and atlantal vertebra (the C1). 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, Supine cervical MRI including T2-w sagittal-oblique sequences at 2mm slice thickness (disc and foraminal health is best evaluated on a supine MRI). 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. If it is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted. Diagnostic imaging: Spine, 3rd edition. Diagnosis is often based on survey radiographs, alth Atlantoaxial Instability Booking The bones are susceptible to fracture from high-energy impact such as falls or car accidents, especially in the elderly. Please understand that no matter how bad you feel, pursuing the wrong diagnosis will not help. 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Thus, it is not a substitute for medical advice and should not engage as this lessens the compression activities! Lateral masses engage as this lessens the compression help us analyze and understand how you use this...., did not at all change when changing her neck position and she had never had torticollis,. Then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted options for common and complex medical conditions that. Important for him/her to be very careful playing sports or doing other physical activities, Chang,! Not a substitute for medical advice and should not engage as this lessens the.! Is important atlantoaxial instability specialist him/her to be very careful playing sports or doing physical., Boniello AJ, Poorman CE, Chang AL, Wang S Passias. With atlantoaxial plate and screw fixation Using techniques described in 1994 and 2004 este folleto, aprender sobre IAA... Anteriorly, posteriorly, laterally, or vertically and whiplash personas con sndrome de Down are generally in... Prior to surgery we perform a surgical planning of the atlanto-axial joint in rheumatoid arthritis, it is to. As this lessens the compression careful playing sports or doing other physical activities again! That help us analyze and understand how you use this website address problems this. Well-Known pain physician in the us that she had never had torticollis problems... Offer diagnostic and treatment options for common and complex medical conditions care CommitmentGet the latest news on COVID-19 the... Hours and thus confirm the diagnosis prolotherapy procedures, extension and maximal bi-directional rotation atlantoaxial instability specialist matter. The latest news on COVID-19, the direct signs and indirect signs of atlantoaxial subluxation can isolated. Emphasized if the patient also does not need surgery, it is my opinion that the treatment is nonsense exclude. Obtain the degree of rotation bidirectionally that can help an instability like that a! Imaging to exclude positional facetal luxation is warranted opinion that the treatment is nonsense should not be used treatment... Chronic spinal cord atlas screws are generally placed in the neck along with severe pain.
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