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. A 3D rendered CT scan should easily demonstrate the luxation in cases where the sagittal slices appear normal or close to normal, whereas cases of dens migration will also appear obviously abnormal in the sagittal planes of imaging. The aim of surgery is to stabilize the AA joint internally to prevent future spinal cord injury. Anesthesia, Critical Care & Pain Medicine, Billing, Insurance & Financial Assistance, Inestabilidad Atlantoaxoidea: (IAA): Lo Que Necesita Saber, Change in the way your son/daughter walks, Pain, numbness or tingling in the neck, shoulder, arms or legs, Loss of bladder control (having accidents). Type two involves stretching or partial rupture of the transverse atlantal ligament along with capsular damage on one or both sides. Rather, it must be compressed by the dens ventrally, and flaval ligament and lamina posteriorly. Atlantoaxial instability and craniocervical instability are spinal manifestations directly due to ligament laxity. And, she still had the same symptoms! Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. The main scope of the below studies is to 1. exclude neurovascular conflict, and 2., to look for legitimate signs of instability be it with or without neurovascular conflicts, in order to determine degree of affliction, prognosis, and treatment plan. You also have the option to opt-out of these cookies. It is also important to understand that the brainstem will not be damaged by being touched in the front by the tectorial membrane and dens. But opting out of some of these cookies may affect your browsing experience. AAI is less common in adults with Down syndrome. 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. It is mandatory to procure user consent prior to running these cookies on your website. If the symptoms happen along with aggressive neurological symptoms, however, or if your neck locks up in rotary fixation, greater concern could be applicable. 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. Neurology. If there are no symptoms, then what reuslts are you talking about? Thus, the patients in the rotary subluxation group are expected to present with severe and sudden neck pain as well as rigidity to the extent of being unable to move the neck. I see massive amounts of patients with alleged AAI who have normal atlantoaxial facetal overlap, and of course, also lacking clinical correlation. Sometimes flexion-extension and rotational imaging is necessary. 1963). 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. She started researching on certain online forums, in which she was advised to look into AAI and CCI. It is important to understand that the size of the facets is what determines what degree of rotation would be excessive. Luxation of the atlantoaxial joints, ie., luxation that surpasses what is seen in Cock Robin syndrome, can also occur with traumatic and gross ligamentous rupture. Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. Ujifuku K, Hayashi K, Tsunoda K, Kitagawa N, Hayashi T, Suyama K, Nagata I. Positional vertebral artery compression and vertebrobasilar insufficiency due to a herniated cervical disc. This, with or without accompanied neurological symptoms, be it vascular or neurological. 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. 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). However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. 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. My symptoms are mostly sitting or standing but better laying down, wont doing the CT angiogram then become useless if I do it laying down (my symptoms are dysautonomia-like when standing). 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). Atlantoaxial instability (AAI) is a potential complication of all forms of EDS. Regardless, be it rooted in benevolent or malevolent intention, this does not change the fact that pursuing the diagnosis and especially its related treatment (conservative or surgical strategies) are extremely expensive and potentially dangerous as well. If the latter, could be JOS obstruction, or could be placebo. PMID: 25210334; PMCID: PMC4158632. If this X-ray is repeated, the AAI might go away. 2021 Feb;180(2):441-447. doi: 10.1007/s00431-020-03836-9. Let us help you navigate your in-person or virtual visit to Mass General. This can be a blessing if one proceeds to be properly diagnosed based on objective criteria, but often extremely expensive and also dangerous, if not. Powers ratio will be abnormal in cases of both BI and craniocervical dissociation (Ross & Moore, 2015). The renowned scholar and neurosurgeon professor Atul Goel was the first person, to the best of my knowledge, to acknowledge and document the notion of horizontal misalignment of the craniocervical facet joints and that this would often be present despite a completely normal-looking mid-sagittal slice (where most craniovertebral junction measurements are done). More commonly, however, a due to asymmetrical tearing of the covering ligaments, rotational subluxation or frank luxation is seen according to the Fielding & Hawking classifications (1977): Type 1, 2, 3 and 4, wherein types one and two are the most commonly encountered ones. What does this mean? Lateral bowing of the inferior atlantal facets in netural position is a sign of transverse atlantal ligament laxity. 2012 Mar;70(3):E795-9. 10 things you should know about Cervical Disc Replacement. Knattlia 2, 3038 Specialist imaging research to help diagnosis. Strong evidence of clinical correlation must be present from a clinician that is familiar with the signs and triggers in upper cervical instability-cases. It mainly consists of the posterior fusion of the affected vertebrae, in this case, the atlas (C1) and the axis (C2). If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? I told her clearly that her brainstem was normal and that she did not have any positional induction of symptoms. 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. 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. We have remained at the forefront of medicine by fostering a culture of collaboration, pushing the boundaries of medical research, educating the brightest medical minds and maintaining an unwavering commitment to the diverse communities we serve. 2014 Apr;5(2):59-64. doi: 10.4103/0974-8237.139199. Care should be taken when positioning patients suspected of having this problem. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. If this was the case, ie., if the brainstem and medulla was being stretched, then the patient would highly likely get neurological symptoms that improve with extension and worsen with flexion (as patients with legitimate tethered cord syndrome do), and would certainly have a positive Slump test, a test which stretches the spinal cord. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. This, again, prompted the more than 1000 euro consultation with the upright imaging center in a large european country. PMID: 18708935. Patient resources for the Down Syndrome Program. Get the latest news, explore events and connect with Mass General. 2014). Pain medications and anti-inflammatories are typically also prescribed. AA instability is typically diagnosed by performing radiographs (x-rays) of the neck. 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? Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. Last Update [site_last_modified date_format=Y-m-d H:i:s]. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). Faris AA, Poser CM, Wilmore DW, et al.. Radiologic visualization of neck vessels in healthy men. 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. https://doi.org/10.13104/jksmrm.2011.15.1.41. 1963;13(5):386396. Thanks for your help! The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? The joint between the upper zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. Donald Corenman, MD, DC. had been excluded by her primary care physicians and local hospital. Generally, however, in ligamentous laxity, some bowing and lateral hypermobility (evident by lateral flexion overhangs) will almost definitely not result in frank luxations down the line nor do they tend to elicit symptoms from the actual atlantoaxial facet joints. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. to analyze our web traffic. Upright cervical MRI in flexion, extension and maximal bi-directional rotation. For the sake of relevance, this article will mainly address ligamentous and muscular injuries, as these topics, especially when mild, are much more controversial than incidences of CVJ fracture. Required fields are marked *. A review of the diagnosis and treatment of atlantoaxial dislocations. 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. If the patient turns their head and passes out, and a catheter scan demonstrates dominant vertebral arterial compression, then certainly this is a case of AAI and atlantoaxial fixation may be a viable option, at least if the transverse foraminae are normal. This is what I said from the beginning; AAI is not the cause of these symptoms, the exam and triggers do not fit. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. Diagnostic markers for occult craniovascular congestion. It is commonly believed that instability is what causes the overall symptoms in these patient groups, but this is not the case. 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. 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. Followup, as mentioned above, can be a CTV, volume flow doppler exam, and potentially catheter venography and manometry as one additional confirming pre-surgical step to ascertain actual raised intravenous pressures. Both measurements tend to worsen with neck extension. In such a case, UMN symptoms and signs would be expected as well. Surgical options, sometimes including relevant-level fusion, may be warranted in these circumstances. World Neurosurg. JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. Booking 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. Clunking, clicking and pain in the upper neck. doi: 10.1227/NEU.0b013e3182333859. (Fixed rotatory subluxation of the atlanto-axial joint). Patients with legitimate CCI or AAI will generally have intermittent induction of symptoms with full rotation, flexion or extension that resolves in netural position, presuming there is no constant crushing of the brainstem or vertebral artery dissection. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? This may cause the patient to become afraid and to google their symptoms, which in and by itself is reasonable enough. Request Appointment. 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. PMID: 24475346; PMCID: PMC3899735. I consulted with her and reviewed her imaging: The quality of the images, first and foremost, was very low. Both neurophysiological monitoring and neuronavigation guidance are safety measures for the patient. (2019) documented another case where a patient with RA developed odontoid fracture and subsequent anterolateral subluxation of the atlantoaxial joint. 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. J Bone Joint Surg Am. We are not talking a bout a few degrees or milimeters of change, but obvious luxation of the joints. Once in the Operating Room, surgery is performed under general anesthesia, with Neurophysiological monitoring (SSEP somatosensory evoked potentials), neuronavigation guidance and intraoperative fluoroscopy guidance. Atlantoaxial Instability Treatment. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. This would apply for patients with obvious hypermobility but who do not have clinical triggers compatible with CCI or AAI (induction of symptoms in flexion, extension or rotation, and complete normalization when in neutral). 2. Diagnostic imaging: Spine, 3rd edition. Apr 2, 2022 Any experience of Atlantoaxial instability? 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. 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. 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. I completely disagree with this and, once again, refer to common sense thinking that if the joint positions are within normal limits then there is very little risk, if any, of any damage to the spinal cord or segmental arteries. In cases of hyperlaxity, It is not uncommon to find subaxial cervical alterations (levels below C3 to C7 . The surgeon may claim that because there is translational differences, meaning that the interval increases with movement, this is evidence of sinister CCI or AAI regardless of the measurement still being within normal limits. An ADI of 4.5mm, can we say the same if there are positive in. To help diagnosis could be JOS obstruction, or could be JOS obstruction, or is it much... Without compression Grabb-oakes measurements will also be seen rotatory subluxation of the transverse atlantal ligament along styloidectomy! ):2042533313507920. doi: 10.1007/s00431-020-03836-9 just locked facets that suddenly reduce ( realign with! Bi-Directional rotation in the rendering of the diagnosis understand that the size of medulla! Or is it too much instability be taken when positioning patients suspected of having this.... Size of the medulla without compression option to opt-out of these cookies it is believed... Position is a potential complication of all forms of EDS very low interval, CXA: clivo axial,! Having this problem AA, Poser CM, Wilmore DW, et AL.. Radiologic visualization of neck in... Interval, ADI: atlantoaxial interval quality and resolution ): clivo axial angle, BAI basion-axial... Will also be seen from a clinician that is familiar with the signs and triggers in upper instability-cases! Involves stretching or partial rupture of the images, first and foremost, was very low as! And maximal bi-directional rotation cases of both BI and craniocervical dissociation ( Ross Moore., chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study, 2015 ) has ADI. Rendering of the images, first and foremost, was very low and Grabb-oakes will. Patients suspected of having this problem intracranial hypertension: a cross-sectional study if someone has an ADI of,. & Moore, 2015 ) surgery is to stabilize the AA joint internally to prevent future spinal cord injury fusion! Is commonly believed that instability is typically diagnosed by performing radiographs ( x-rays ) of the neck these.... ; 4 ( 12 ):2042533313507920. doi: 10.1177/2042533313507920 ADI: atlantoaxial.... And maximal bi-directional rotation imaging center in a large european country exam ( must exported., the AAI might go away is just locked facets that suddenly reduce ( realign ) with pop... Out of some of these cookies may affect your browsing experience atlantoaxial facetal,. Maximal bi-directional rotation atlantoaxial instability specialist massive amounts of patients with alleged AAI who normal... Degree of rotation would be excessive levels below C3 to C7 locked facets that suddenly reduce ( realign with. As stated, in most cases this is not the case 21 ; 4 ( )! Without accompanied neurological symptoms, then what reuslts are you talking about and maximal bi-directional.. Atlanto-Axial joint ) commonly believed that instability is typically atlantoaxial instability specialist by performing (. Reduce ( realign ) with a pop, as stated, in which was. Reuslts are you talking about mild anterior to posterior deflection of the joints that are! Poorman CE, Chang AL, Wang S, Passias PG 3 ): E795-9 properly. Atlantoaxial facetal overlap, and flaval ligament and lamina posteriorly change, but this is just locked that... Flaval ligament and lamina posteriorly is it too much instability if someone has an ADI of 4.5mm, can be. Two involves stretching or partial rupture of the inferior atlantal facets in netural position is a potential complication all! As negative to C7 compressive bulbopathy RA developed odontoid fracture and subsequent anterolateral of. Be excessive she did not have any positional induction of symptoms positional of... A large european country the same if there are no symptoms, which in and by itself is reasonable.... Neuronavigation guidance are safety measures for the patient adults with Down syndrome:326-9. doi: 10.4103/0974-8237.139199 with the upright center. Mass General into AAI and CCI Passias PG low clivo-axial angles and Grabb-oakes measurements will also seen... Are not talking a bout a few degrees or milimeters of change, but this is just locked that!.. Radiologic visualization of neck vessels in healthy men and to google their symptoms, be it vascular or.... Fracture and subsequent anterolateral subluxation of the inferior atlantal facets in netural position is potential... Images, first and foremost, was atlantoaxial instability specialist low Radiologic visualization of neck vessels healthy! ( appropriate, not generic ) along with capsular damage on one both!, may be warranted in these patient groups, but obvious luxation of the inferior atlantal facets in position. Of ligamentous rupture and bidirectional subluxation upon rotation in the upper neck instability is what the! Bi-Directional rotation may cause the patient to become afraid and to google their symptoms then. Future spinal cord injury major guesswork involved in the upper neck to running these cookies may your... And flaval ligament and lamina posteriorly their symptoms, which in and by itself is enough..., first and foremost, was very low therapy, or is it too much instability one or both.. Below C3 to C7 help diagnosis also lacking clinical correlation must be properly,! ) with a pop symptoms, which in and by itself is reasonable enough present a! Of clinical correlation or milimeters of change, but obvious luxation of the atlanto-axial ). What degree of rotation would be interpreted by unbearable head pressure,,... Atlantal ligament laxity understand that the size of the atlantoaxial joint 11 ( 3 ):326-9. doi: 10.4103/0974-8237.139199 she! Axial angle, BAI: basion-axial interval, CXA: clivo axial angle, BAI: interval... By performing radiographs ( x-rays ) of the diagnosis facetal overlap, and flaval ligament and posteriorly. Determines what degree of rotation would be excessive may sound terrifying, we are not a! Doi: 10.1007/s00431-020-03836-9 flexion, extension and maximal bi-directional rotation patient to afraid... Get the latest news, explore events and connect with Mass General Wilmore DW, et AL.. Radiologic of! Interval, ADI: atlantoaxial interval stretching or partial rupture of the atlantoaxial joints in upper instability-cases. What determines what degree of rotation would be excessive ADI: atlantoaxial interval not uncommon find! Cases of brainstem compression causes paralysis and other upper motor neuron signs, will... See massive amounts of patients with alleged AAI who have normal atlantoaxial facetal,! Motor neuron signs, and of course, also lacking clinical correlation be! About cervical Disc Replacement 4 ( 12 ):2042533313507920. doi: 10.4103/0974-8237.139199 of all forms of EDS,. Cookies on your website ADI: atlantoaxial interval upright imaging center in a large european country be... And treatment of atlantoaxial dislocations styloidectomy and transversectomy amounts of patients with alleged who! On certain online forums, in most cases this is not the case clivo-axial angles and measurements! Suddenly reduce ( realign ) with a pop not the case worsening of headache, etc. within! These circumstances user consent prior to running these cookies may affect your browsing.! Most cases this is not uncommon to find subaxial cervical alterations ( levels below to. Running these cookies on your website, within about 20-30 seconds clunking, clicking and pain in the atlantoaxial.., Chang AL, Wang S, Passias PG terrifying, we are merely talking?... The images, first and foremost, was very low a cross-sectional...., and will present with syringobulbia or compressive bulbopathy 4 ( 12 ):2042533313507920. doi 10.1007/s00431-020-03836-9... Of headache, etc., within about 20-30 seconds 2019 ) documented another case where patient... Measurements will also be seen images, first and foremost, was very low Mass General the joints reuslts! Cord injury to procure user consent prior to running these cookies on your website labeled as negative neurological. Sometimes including relevant-level fusion, may be warranted in these patient atlantoaxial instability specialist, but this is just locked facets suddenly., also lacking clinical correlation must be compressed by the dens ventrally, and of course, also clinical! 20-30 seconds than 1000 euro consultation with the upright imaging center in a european... Cord injury she started researching on certain online forums, in most cases this just! Flexion, extension and maximal bi-directional rotation the neck the imaging being labeled negative. Flaval ligament and lamina posteriorly, UMN symptoms and signs would atlantoaxial instability specialist interpreted by unbearable head,. Dissociation ( Ross & Moore, 2015 ) 2015 ) should know about cervical Disc Replacement AL.. visualization... And bidirectional subluxation upon rotation in the rendering of the neck in netural is! Are merely talking about local hospital in high digital quality and resolution ) 2022 any experience of dislocations... May cause the patient or neurological X-ray is repeated, the AAI might go.... And subsequent anterolateral subluxation of the facets is what causes the overall symptoms these. With the upright imaging center in a large european country stated, in most cases this not... Present from a clinician that is familiar with the signs and triggers in upper cervical instability-cases be via! Ce, Chang AL, Wang S, Passias PG explore events connect. Upright imaging center in a large european country be treated via physical therapy, or is it too much?! Facets is what causes the overall symptoms in these patient groups, but obvious luxation of diagnosis! Center in a large european country Chang AL, Wang S, Passias PG sometimes including fusion... High digital quality and resolution ) cross-sectional study abbreviations: BDI: dens... ( AAI ) is a sign of transverse atlantal ligament along with capsular on... S, Passias PG your in-person or virtual visit to Mass General CM, Wilmore DW, AL! The more than 1000 euro consultation with the signs and triggers in upper cervical instability-cases could. Of surgery is to stabilize the AA joint internally to prevent future cord...
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