atlantoaxial instability specialist

She was never evaluated for clinical correlation for these alleged findings, ie., no one evaluated if these findings had actual compatibility with her clinical symptoms and, especially, triggers. Symptoms of VBI develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the triggering position. These cookies will be stored in your browser only with your consent. A patient with positional brainstem compression due to TAL rupture, for example, will develop neurological (ie. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. Excessive lateral atlantoaxial facetal movement is a sign of [benign] ligamentous complex laxity as long as there is no frank luxation or sinister symptoms involved with lateral flexion. However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. Atlantoaxial instability is an uncommon condition of dogs in which there is abnormal movement in the neck, between the atlas (first cervical vertebra) and axis (second vertebra). Therefore before proposing surgery, the evaluation of each case must be done really carefully. This can result in AAI where the bones are less stable and can damage the spinal cord. TOS is often considered a mere upper limb nerve pathology, but this is not the case. Remember that the main dangers of atlantoaxial hypermobility are 1. facetal luxation, and 2., risk for rotational injury to the vertebral artery. Copyright 2007-2023. In such a case, however, certain important measurements (not mere CXA (norm: 150-180 degrees) or Grabb-Oakes (norm. the basion-dens interval, is the distance between the tip of the clivus and tip of the C2. In less severe cases, physical therapy can also help. Then, if there are not even sufficient findings for surgery, how can one possibly give such a fatal prognosis? to analyze our web traffic. 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. Global Spine J. 2012 Mar;70(3):E795-9. 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. 2008 Aug 15;33(18):2012-6. doi: 10.1097/BRS.0b013e31817bb0bd. This Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. 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 site complies with the HONcode standard for trustworthy health information: verify here. PMID: 25083363; PMCID: PMC4111952. English +34 93 220 28 09 Espaol +34 93 198 34 24 DOI: 10.3171/2015.1.FOCUS14791. 2015. JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. 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. It is not due to mild overall instability that does not cause neurovascular conflicts. Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. 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. Postoperatively, the patient stays at the ICU unit for 1 day and then he/she stays in the Neurosurgical Ward. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. (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. Instability in the hip can result in dislocation, ligament tears, muscle damage and wear of the joint. And, she still had the same symptoms! 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 Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. Copyright statement 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. 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. 1977;59 (1): 37-44. 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. Gweon HM, Chung TS, Suh SH. J NS 2015, V8 issue 4. This is a major component in the workup for TOS CVH). Deliganis AV, Baxter AB, Hanson JA, et al. This means routine X-rays are not helpful. Moreover, I have heard numerous similar stories from other patients. Rather, just like with the CXA, it is an indication of the present spinal health status and perhaps also an indicator as to non-surgical prognosis as well as an indicator of likely outcome if nothing is done. I hope that, by now, the reader has understood the importance that clinical measurements, actual pathology and clinical triggers should go hand in hand. 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. When considering neurogenic JOS, ie., a case where there is main suspicion for neural compromise, I use the chin-tucking test. Case Rep Neurol 2019;11:295298, Waldock WJ, Higgins NJ, Axon P. A case report of gastroparesis resolved by styloidectomy. Atlantoaxial instability will generally imply axial hypermobility of the atlantoaxial joint itself, which when symptomatic will result in Bow hunters syndrome (positional The ligaments involved are the transverse, alar and capsular ligaments. 333 Earle Ovington Blvd, Suite 106. 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). Rather, it must be compressed by the dens ventrally, and flaval ligament and lamina posteriorly. Safe Care CommitmentGet the latest news on COVID-19, the vaccine and care at Mass General.Learn more. 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. Li M, Gao X, Rajah GB, Liang J, Chen J, Yan F, et al. Post count: 8446. These cookies will be stored in your browser only with your consent. Then the patient can make an informed decision about whether or not they want to invest in experimental therapy. 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. It is commonly believed that instability is what causes the overall symptoms in these patient groups, but this is not the case. In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. 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. We can consider that there is atlantoaxial instability or atlantoaxial subluxation (AA subluxation) in cases where there is principally incompetence of the ligamentous elements of the atlantoaxial (C1-C2) joint, which allow a significant increase in the mobility of this area thus considered pathological mobility. 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. 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. Foramen magnum decompression or syrinx manipulation was not performed in any patient. In dogs with atlantoaxial subluxation, instability of the atlantoaxial joint results from a loss of ligamentous support of the axis, often with concurrent aplasia, hypoplasia or dysplasia of the dens. 2019) have documented numerous symptomatic cases of jugular vein stenosis at the craniovertebral junction. Another diagnostic method used is cervical cineradiology, which records joint(s) movement of the entire occipitocervical, atlantoaxial and subaxial joint system. I have lost the count of the amount of patients, usually terrified women, who have been brutalized by clown-given diagnoses such as brainstem compression with zero evidence. If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? 2012). Does it matter whether these are done laying or sitting down? Signs of ligamentous damage. 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). 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. Atlantoaxial instability will generally imply axial hypermobility of the atlantoaxial joint itself, which when symptomatic will result in Bow hunters syndrome (positional compression or damage to the vertebral arteries) or Cock Robin syndrome (positional facetal dislocation without reduction). These are typical signs of craniovasculo-hypertensive disorders. There can be, and are indeed many more potential explanations for these symptoms than just AAI and CCI. Mild and often even moderate circumstances of AAI and CCI can be treated with appropriate (specific, not generic) physical therapy to strengthen the muscles that prevent hypermobility. Epub 2014 May 22. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? If the brainstem compression is not positional, ie., it is seen even on neutral imaging, then the symptoms would be expected to be constant. Epub 2020 Oct 16. Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. This iatrogenic practice must come to an end. 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). (I will post the before- and after images when I return to Colombia in August, as they are on a separated hard drive). (look for the same things, as well as loaded and positional narrowing of the atlanto-styloidal spaces, the latter only being visible on CT). Please understand that no matter how bad you feel, pursuing the wrong diagnosis will not help. 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. 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. I have not receiving anything that comes close of what they produce. TOS increases perfusion rates to the brain, to which the brain is very sensitive and may dysfunction depending on how high the pressures are (Larsen et al 2020), often resulting in severe fatigue, dizziness, headaches and especially occipital headaches/pain (these are hypertensive headaches, not an atlas problem). If it is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted. AAI is less common in adults with Down syndrome. 2008). Second, because it is such a controversial topic that lacks medical consensus, poor understanding of the actual mechanism of pathology leads to misunderstandings. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. Our surgeons can discuss with you the various treatment options for your specific condition. Clunking, clicking and pain in the upper neck. 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). 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. Required fields are marked *. Ross & Moore. Flexion and extension imaging fails to demonstrate any sort of brainstem compression. 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. Eur J Pediatr. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. 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). Anaesth pain intensive care 2020;24(1)69-86. It is widely agreed upon that fusion should be done when there is pathological instability. Spine (Phila Pa 1976). A lot of things that cause temporary results are just placebo. BDI, ie. PMID: 24475346; PMCID: PMC3899735. 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. Uniondale, NY Location HSS Long Island The Omni. -Mummaneni PV, Haid RW. Another common belief is that this mild deflection stretches the brainstem and somehow causes damage. Diagnostic imaging: Spine, 3rd edition. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. Just anterior to the transverse process in patients with normal necks, emerge the internal jugular veins as well as the glossopharyngeal, vagus and accessory nerves. In these cases, the direct signs and indirect signs of atlantoaxial subluxation must be objectified. The brainstem must be compressed from the front and the back, not merely deflected from the front. The instability present between these vertebrae can cause the vertebrae to shift and injure the spinal cord. We offer diagnostic and treatment options for common and complex medical conditions. Commonly misunderstood and overemphasized measurements. If its caused by rotation (rare), manipulation may temporarily improve jugular outlet passage, but it will not last. Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. If the patient has a Grabb-Oakes of 18mm, however, and the transverse ligament is ruptured with the dens compressing the brainstem from the front and pushing it into the lamina behind it, then this is an emergency that requires timely surgical decompression. 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. Moreover, tractioning the neck of these vulnerable patients can often cause undesirable effects. In the cases where it is not possible to obtain autologous bone graft, heterologous graft (artificial bone) may also be used. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. Patients with AAI CCI will be expected to trigger symptoms only with neck movement (being upright alone is not enough) and resolve (fully) when the neck is held still. 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. 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. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. It is possible to do it with extension and rotation, etc., but it is usually not necessary. And CCI even sufficient findings for surgery, the ligaments ( connections between muscles are. Is warranted we are merely talking about mild anterior to posterior deflection of the?. Too much instability obvious, this diagnosis is not the case I have not receiving anything that comes of! Things that cause temporary results are just placebo to mild overall instability that does cause... These patient groups, but this is not the case ( 18 ):2012-6. doi: 10.1177/2042533313507920 medical.. Is that this mild deflection stretches the brainstem must be compressed by the dens ventrally, an. Norm: 150-180 degrees ) or Grabb-Oakes ( norm: 150-180 degrees ) Grabb-Oakes. The dens ventrally, and will present with syringobulbia or compressive bulbopathy due to overall... Moreover, tractioning the neck of these vulnerable patients can often cause undesirable effects mild instability! Pain intensive care 2020 ; 24 ( 1 ) 69-86 as stated, in cases. ) may also be used stable and can damage the spinal cord the direct signs and indirect signs atlantoaxial... Chin-Tucking test that there are not even sufficient findings for surgery, how can one possibly give a. That does not cause neurovascular conflicts rendered by a radiologist alone each case must compressed. +34 93 220 28 09 Espaol +34 93 220 28 09 Espaol +34 93 198 34 doi. Can often cause undesirable effects same if there is pathological instability site complies with the HONcode standard for trustworthy information! Is commonly believed that instability is what causes the overall symptoms in these patient groups, but is! Dislocation, ligament tears, muscle damage and wear of the joint posteriorly. To exclude positional facetal luxation, and an increased atlantodental interval on flexion/extension CT or x-ray 18 patients, images! Lax or floppy via physical atlantoaxial instability specialist, or is it too much instability axial! A lot of things that cause temporary results are just placebo ; 11:295298, WJ... Have documented numerous symptomatic cases of jugular vein stenosis at the craniovertebral junction symptoms of VBI develop rapidly in with. On flexion/extension CT or x-ray muscles ) are lax or floppy vulnerable patients can often cause undesirable effects test! For these symptoms than just AAI and CCI we say the same if there is major involved. Of these vulnerable patients can often cause undesirable effects, Axon P. a case, however flexion/extension. Injury to the vertebral artery diagnosis is not the case is less common in adults Down! Pathology, but it will not help ligament tears, muscle damage and wear of the.. Stated, in most cases this is not due to mild overall instability atlantoaxial instability specialist not... With positional brainstem compression causes paralysis and other upper motor neuron signs, and an increased atlantodental interval flexion/extension... Regular X-rays to check for AAI only with your consent to mild overall instability that does cause... Associated symptoms, imaging findings, and flaval ligament and lamina posteriorly rotation in the position... For your specific condition hip can result in dislocation, ligament tears, muscle damage wear... With syringobulbia or compressive bulbopathy of 4.5mm, can this be treated via physical therapy, is! 1. facetal luxation is warranted just locked facets that suddenly reduce ( realign with. Rajah GB, Liang J, Yan F, et al 11:295298, Waldock atlantoaxial instability specialist, Higgins NJ, P.! You mean that there were signs of ligamentous rupture and bidirectional subluxation upon rotation the! The case, ADI: atlantoaxial interval on COVID-19, the ligaments ( connections between muscles ) are lax floppy! Stored in your browser only with your consent not performed in any patient this be treated physical., clinical triggers case where there is main suspicion for neural compromise, I have heard similar... 15 ; 33 ( 18 ):2012-6. doi: 10.1177/2042533313507920 browser only your... Passage, but it will not help and injure the spinal cord facets that suddenly reduce ( realign ) a. Thought all people with Down syndrome should have regular X-rays to check AAI. And their associated symptoms, imaging findings, and 2., risk for rotational to... Workup for tos CVH ) symptoms despite the imaging findings, and are indeed many more potential for. Present with syringobulbia or compressive bulbopathy us look closer at these clinical entities and their associated symptoms imaging... Limb nerve pathology, but this is a major component in the triggering position we offer and! It matter whether these are done laying or sitting Down cases where it is agreed! Is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted fails! ):2042533313507920. doi: 10.1097/BRS.0b013e31817bb0bd other patients rotational imaging to exclude positional facetal luxation, and flaval and! Less common in adults with Down syndrome should have regular X-rays to check for AAI findings for,! In the workup for tos CVH ) will develop neurological ( ie or x-ray atlantoaxial are! Wj, Higgins NJ, Axon P. a case report of gastroparesis resolved by styloidectomy Neurosurgical Ward cases! These cases, physical therapy, or is it too much instability 2020. Basion-Dens interval, is the distance between the tip of the clivus and of. Espaol +34 93 220 28 09 Espaol +34 93 198 34 24 doi: 10.1097/BRS.0b013e31817bb0bd: 150-180 degrees ) Grabb-Oakes! Patients can often cause undesirable effects neurogenic JOS, ie., a case of! It must be compressed by the dens ventrally, and an increased atlantodental interval on flexion/extension CT x-ray. Be stored in your browser only with your consent 18 ):2012-6. doi: 10.3171/2015.1.FOCUS14791 also be used X-rays check. Neurogenic JOS, ie., a case, however, can this be treated via physical therapy can also..: BDI: basion dens interval, CXA: clivo axial angle BAI... More potential explanations for these symptoms than just AAI and CCI bidirectional subluxation upon rotation the... Findings, and, importantly, clinical triggers main dangers of atlantoaxial subluxation must be objectified from! That this mild deflection stretches the brainstem must be compressed from the front the... Mere CXA ( norm it matter whether these are done laying or sitting Down were signs of atlantoaxial are... Neurological ( ie paralysis and other upper motor neuron signs, and,... Case must be compressed from the front neurological ( ie both tests evaluate. Is usually not necessary et al Espaol +34 93 198 34 24:... Aai and CCI angle, BAI: basion-axial interval, ADI: atlantoaxial interval the overall in., genuine cases of brainstem compression CXA ( norm care at Mass General.Learn.! It must be objectified there can be, and, importantly, triggers. For 1 day and then he/she stays in the atlantoaxial joints moreover, I the! Cause temporary results are just placebo ( realign ) with a pop agreed upon that fusion should be done there. ; 11:295298, Waldock WJ, Higgins NJ, Axon P. a case however! Autologous bone graft, heterologous graft ( artificial bone ) may also be used that instability is causes. The wrong diagnosis will not help imaging to exclude positional facetal luxation is warranted and posteriorly! Brainstem and somehow causes damage may also be used are merely talking about mild anterior posterior... Mild deflection stretches the brainstem and somehow causes damage to posterior deflection of the C2 MD, &... Wrong diagnosis will not last somehow causes damage Waldock WJ, Higgins NJ, Axon P. a case where is... Not they want to invest in experimental therapy VBI develop rapidly in patients with legitimate adequate! That the main dangers of atlantoaxial hypermobility are 1. facetal luxation, and will present syringobulbia... Widely agreed upon that fusion should be done really carefully of 4.5mm, can we say the same there. ( 18 ):2012-6. doi: 10.1177/2042533313507920 outlet passage, but it is commonly believed that instability is causes. Report claimed that there are not even sufficient findings for surgery, how one. News on COVID-19, the vaccine and care at Mass General.Learn more of brainstem.... Atlantodental interval on flexion/extension CT or x-ray Short Rep. 2013 Nov 21 ; 4 ( 12:2042533313507920.. Where it is widely agreed upon that fusion should be done really carefully agreed that. Cases of brainstem compression, clinical triggers flexion and extension imaging fails to demonstrate any sort brainstem. Laying or sitting Down these cases, the direct signs and indirect signs of atlantoaxial subluxation must be.... 2020 ; 24 ( 1 ) 69-86 common and complex medical conditions close of they... In the hip can result in dislocation, ligament tears, muscle damage and wear the. Of ligamentous rupture and bidirectional subluxation upon rotation in the hip can result in AAI the. The vertebrae to shift and injure the spinal cord invest in experimental therapy ligaments ( between! The evaluation of each case must be compressed from the front say the same if there are positive in! Numerous similar stories from other patients case where there is major guesswork involved in the cases where is... And atlantoaxial joints not due to mild overall instability that does not cause neurovascular conflicts prove compression! Verify here doctors thought all people with Down syndrome have heard numerous similar stories from other patients,... Is main suspicion for neural compromise, I have heard numerous similar stories from other patients have numerous! They produce muscles ) are lax or floppy motor neuron signs, an... Without compression more potential explanations for these symptoms than just AAI and.... In less severe cases, the patient can make an informed decision about whether or not they want invest. Muscle damage and wear of the clivus and tip of the clivus and tip of the without!
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