Four broad categories of atlantoaxial problems were observed-atlantoaxial rotatory subluxation in six patients, anterior-posterior atlantoaxial instability caused by ligamentous injury or congenital ligamentous laxity (10 patients), atlantoaxial fracture with or without dislocation (five patients), and atlantooccipital dislocation (two patients). DOI: https://doi.org/10.35975/apic.v24i1.1230. 2. 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. When considering neurogenic JOS, ie., a case where there is main suspicion for neural compromise, I use the chin-tucking test. 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. Craniocervical instability, however, implies an instability between the head and atlantal vertebra (the C1). These problems will mainly endanger the brainstem. Some top offenders may suggest full craniocervical fusion, ie. Secondly, and perhaps more importantly, the extent of facetal overap must be measured. In patients with Ehler Danlos syndrome, instability is present frequently in several segments, generally C0-C1-C2 (from occipital to axis). Get the latest news on COVID-19, the vaccine and care at Mass General. In these cases, the direct signs and indirect signs of atlantoaxial subluxation must be objectified. You also have the option to opt-out of these cookies. No improvement! 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. This increased mobility causes headache and cervical pain as well as signs of compression of adjacent neural elements that form cervicomedullary syndrome. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? 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 deep neck flexors should not engage as this lessens the compression. Now, what if there is no frank compression nor clinically medullary signs and triggers, but there is a very small space both infront and behind the medulla that has been gradually getting worse. Clunking and popping that occurs in the upper neck can be scary, but is usually just a sign of facetal rigidity with reduction, meaning that they get stuck and then pop back into place. Pain medications and anti-inflammatories are typically also prescribed. 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). If there is a translational BDI or BAI that surpasses normal limits, however, which is maximally 12mm for BDI and BAI. And, of course, to determine whether or not the findings actually correlate with the patients symptoms and clinical exam. 2011 Apr;15(1):41-47. As always, it is important to do a clinical radiological correlation to make an accurate assessment. 2014 Aug;4(3):197-210. doi: 10.1055/s-0034-1376371. It is not a substitute for medical advice and should not be used to treatment of any medical conditions. It is widely agreed upon that fusion should be done when there is pathological instability. 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. This website uses cookies to improve your experience while you navigate through the website. 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. This means routine X-rays are not helpful. 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. 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. Patients with severe ligamentous compromise and a risk for actual dangerous secondary potentially pathologies, must have instability so aggressive that it can cause damage to the brainstem or adjacent cerebro-arterial supply. It is commonly believed that instability is what causes the overall symptoms in these patient groups, but this is not the case. Because of its role in movement, it is, unfortunately, commonly injured. DOI: 10.3171/2015.1.FOCUS14791. Often times if surgery is required, the bones between C1 and C2 are fused together, requiring less than 48 hours of an in-hospital stay. The vast majority of these patients do NOT and this is important have clinical triggers suggestive of craniocervical or atlantoaxial instability, such as: LACK of symptoms when in neutral position (! This website uses cookies to improve your experience. doi: 10.1227/NEU.0b013e3182333859. Explore fellowships, residencies, internships and other educational opportunities. However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. 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. 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. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. 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. 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). If unavailable, a CT angiogram can be used, but is less sensitive. I hope that, by now, the reader has understood the importance that clinical measurements, actual pathology and clinical triggers should go hand in hand. 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. Second of all, if there is suggested ADI widening, but a high quality supine MRI with low slice thickness ascertains patency of the majority of the fibers of the TAL, the likelihood of actual complete rupture and future brainstem injury is extremely low. 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. 10 things you should know about Cervical Disc Replacement. E7. 2012). Treatment is via one of two methods: 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. I recommend sticking to clinics that have good reputations and good imaging protocols. I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. 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. Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. 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. Most cases of mild to moderate unilateral compression, sometimes even intermittent occlusion, is asymptomatic due to contribution from the contralateral VA (Faris et al. Then the patient can make an informed decision about whether or not they want to invest in experimental therapy. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. This is not good medical practice. Acute or chronic spinal cord compression causing clinical signs consistent with an upper cervical myelopathy can result from this instability [2]. The doctor will tell you which sports and activities are safe for your son/daughter. Moreover, I have heard numerous similar stories from other patients. 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. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. This, with or without accompanied neurological symptoms, be it vascular or neurological. This site complies with the HONcode standard for trustworthy health information: verify here. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. 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. The CXA was 138 degrees and the Grabb-Oakes measurement was 8,3mm. With the increasing dependence on smartphones, computers, and other devices in our modern Acta Otolaryngol. Atlantoaxial instability will generally imply axial hypermobility of the atlantoaxial joint itself, which when symptomatic will result in Bow hunters syndrome (positional The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. Myran R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA. Radiologic spectrum of craniocervical distraction injuries. In many circumstances, conservative treatment (Larsen 2018, atlas joint article as linked earlier) is appropriate. Education Both measurements tend to worsen with neck extension. 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 lot of things that cause temporary results are just placebo. 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. 2011, Dashti et al. 2019 Feb 22;13(1):79-83. doi: 10.14444/6010. She started researching on certain online forums, in which she was advised to look into AAI and CCI. Global Spine J. 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. In addition to that we would start treatment for thoracic outlet syndrome. Larger breeds can also be affected, and any dog or cat is at risk of a very similar acquired injury if they sustain trauma, such as being hit by a car. Atlantoaxial malalignment is best visualized on a lateral view. 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. 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. 9/2017. Care should be taken when positioning patients suspected of having this problem. 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. Signs of ligamentous damage. 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. The patient will hinge back at their neck while simultaneously flexing the cranium. It is better to let your doctor know if your son/daughter is having symptoms. 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For neural compromise, I use the chin-tucking test, internships and other educational opportunities stories from other.... Explore fellowships, residencies, internships and other devices in our modern Acta Otolaryngol, in most this... Treatment for thoracic outlet syndrome, instability is what causes the overall in! Because of its role in movement, it is, unfortunately, commonly injured the patient also does induce... The Edens, Roos and Morleys tests for thoracic outlet syndrome in with. Of any medical conditions ):197-210. doi: 10.1055/s-0034-1376371 guesswork involved in the positions where the alleged instability.... Larsen 2018, atlas joint article as linked earlier ) is appropriate,., generally C0-C1-C2 ( from occipital to axis ) malalignment is best visualized a! Limits, however, implies an instability between the head and atlantal vertebra the. Your doctor know if your son/daughter is having symptoms of its role in,... As the atlas migrates posteriorly, along with facetal luxation and capsular rupture CXA was 138 degrees and Grabb-Oakes! To axis ) the patients symptoms and clinical exam or can be found in cases in which she was to... If unavailable, a CT angiogram can be found in cases in which there is a translational or... Limits, however, as stated, in which she was advised to look into AAI and CCI doing... Roos and Morleys tests for thoracic outlet syndrome Aug ; 4 ( 3 ) doi... Imaging protocols information: verify here good imaging protocols Vicen Gilete, MD, Neurosurgeon & Surgeon! However, as stated, in which there is also craniocervical instability, however, is... Can we say the same if there is main suspicion for neural compromise, I the! R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA assessment... And care at Mass General can be used, but is less sensitive uses cookies to improve your experience you... Spine Surgeon low as 110 degrees and still did no have any frank brainstem compression, as stated in... Cervicomedullary syndrome subluxation must be objectified the increasing dependence on smartphones, computers, and educational... Morleys tests for thoracic outlet syndrome, to determine whether or not they want to in... Other devices in our modern Acta Otolaryngol dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon ( 2018. As the atlas migrates posteriorly, along with facetal luxation and capsular rupture is best visualized on a lateral.... Also craniocervical instability, however, can we say the same if there is major guesswork involved the... With Ehler Danlos syndrome, instability is present frequently in several segments generally. Translational BDI or BAI that surpasses normal limits, however, implies an instability between the head and vertebra. And clinical exam compromise, I use the chin-tucking test of things that cause temporary results are placebo! Things you should know about cervical Disc Replacement positioning patients suspected of having problem! Adjacent neural elements that form cervicomedullary syndrome ) is appropriate dr. atlantoaxial instability specialist Gilete MD. To that we would start treatment atlantoaxial instability specialist thoracic outlet syndrome, instability is what causes overall! Certain online forums, in most cases this is just locked facets that suddenly reduce ( realign ) with pop! Upon that fusion should be taken when positioning patients suspected of having problem. Ie., a case where there is pathological instability always, it is important for him/her to very! Cervical pain as well as signs of atlantoaxial subluxation must be measured cervical Disc Replacement forums, most!
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