Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, et al. 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). Our surgeons can discuss with you the various treatment options for your specific condition. Moderator. The BDI was 6mm and the BAI was 8mm, which are all farily normal. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. Thanks for your help! The most commonly used measures in the radiological evaluation of craniocervical instability and atlantoaxial instability are CXA, Grabb, BDI, BAI, ADI. This pain tends to get worse with stress and with high heart rates, and are often also worse in the morning after lying down. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. 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. Li M, Gao X, Rajah GB, Liang J, Chen J, Yan F, et al. Tambin conocer las causas, los signos y los sntomas de la IAA. Search for condition information or for a specific treatment program. to get a better impression of its actual thickness. When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. Washington University neurosurgeons have extensive experience treating problems in this area and are recognized nationally as experts in providing innovative treatments for this unique and complex area of the neck. It is important to understand that the size of the facets is what determines what degree of rotation would be excessive. These problems are much more constant than AAI CCI, which are, for the most part, positional problems. 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. PMID: 19769514. Radiographics 2000;20:S237-50. PMID: 25210334; PMCID: PMC4158632. See my youtube channel for appropriate training. For treatment of the facetal dysfunction I recommend postural correction for the head neck and shoulder blades, along with exercises for the trapezius, levator scapulae, suboccipital and deep neck flexor muscles. Lateral bowing of the inferior atlantal facets in netural position is a sign of transverse atlantal ligament laxity. In other words, the vertical distance between the head and the spine. Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. 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. 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. There is a growing trend, however, within the (or, at least, certain) alternative medical communities, where patients with normal or virtually normal imaging, and with the absence of clinical triggers that would suggest atlantoaxial or craniocervical instability, still end up diagnosed with these relatively sinister diagnoses. 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. In patients with Ehler Danlos syndrome, instability is present frequently in several segments, generally C0-C1-C2 (from occipital to axis). Knattlia 2, 3038 If this X-ray is repeated, the AAI might go away. 10 things you should know about Cervical Disc Replacement. 2014 Apr;5(2):59-64. doi: 10.4103/0974-8237.139199. 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. DMX I dont recommend getting a DMX. 2021 Feb;180(2):441-447. doi: 10.1007/s00431-020-03836-9. In less severe cases, physical therapy can also help. But opting out of some of these cookies may affect your browsing experience. DOI: https://doi.org/10.35975/apic.v24i1.1230. The ligaments holding the bones together can also be injured in trauma, or weakened in certain inflammatory conditions such as rheumatoid arthritis or Downsyndrome. 2014). Atlantoaxial instability will generally imply axial hypermobility of the atlantoaxial joint itself, which when symptomatic will result in Bow hunters syndrome (positional The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. PMID: 25083363; PMCID: PMC4111952. The brainstem must be compressed from the front and the back, not merely deflected from the front. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. The atlantoaxial subluxation may exist in patients neutral position (without neck movement) or may occur in relation to neck rotation movements (when the patient moves the neck to the right and left). Elsevier Publishing. Symptoms of brainstem compression are respiratory crisis and quadriplegia, but can also manifest more diffusely. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. 2. We offer diagnostic and treatment options for common and complex medical conditions. If unavailable, a CT angiogram can be used, but is less sensitive. 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, 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 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. One or 2 out of every 100 children with Down syndrome have symptoms of AAI, but doctors do not know the exact number yet. 3. We are committed to providing expert caresafely and effectively. 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. Both patients had severe symptoms regardless of lying down, wearing a neck brace, etc., and did not get worse nor better when turning or moving their necks. Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. Global Spine J. 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). Call 314-362-3577 for Patient Appointments. 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. 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). The most important risks involved in these injuries are concomitant arterial (especially vertebral artery) or brainstem injuries which can result in stroke or paralyis from the head and down or even death. This, seriously augmented by poor hinge neck postures (Larsen 2018). 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. The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. Craniocervical instability, however, implies an instability between the head and atlantal vertebra (the C1). J Neurol Surg B. DOI: 10.1055/s-0039-1677706, Perez MA, Bialer OY, Bruce BB, Newman NJ, Biousse V. Primary Spontaneous Cerebrospinal Fluid Leaks andIdiopathic Intracranial Hypertension. PMID: 18708935. Moreover, I have heard numerous similar stories from other patients. PMID: 30805289; PMCID: PMC6383461. She was also said to have ventral brainstem compression, which particularly scared her due to her difficulties with respiration. This, of course, must be evaluated on a case-to-case basis. Hopefully, this is the result of ignorance combined with poor clinical workup skills (incompetence) and not mere greed and malevolence. As touched upon in the beginning of this article, that prompted me to write this article, is a huge massive influx of patients over the last few years who have been illegitimately diagnosed with AAI or CCI. 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). Must be carefully evaluated and correlated with the patients symptoms). Neurosurgery. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. Most dogs with AA instability will develop clinical signs within the first 2 years of life, often after a seemingly mild traumatic event. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. 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. A lof patients have clicking and clunking in the neck along with severe suboccipital pain. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. 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 (! Epub 2014 May 22. Diagnostic markers for occult craniovascular congestion. Request Appointment. J Korean Soc Magn Reson Med. Copyright 2007-2023. But opting out of some of these cookies may affect your browsing experience. I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. Int J Spine Surg. A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. PMID: 749697; PMCID: PMC1000289. 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. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. Common findings: Ovalization of the orbitae, dilated optic nerve sheaths, pituitary concavity, Chiari malformation, tight brain appearance, jugular vein compression with or without white-vessel signs, dilation or narrowing of the lateral and possibly third ventricles, periventricular ependymal T2 FLAIR hyperintensities), Neck MRI (general evaluation of the neck integrity), CT angiogram of the head neck and subclavian arteries with the arms raised (contrast infusion via femoral vein. Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. Then, if there are not even sufficient findings for surgery, how can one possibly give such a fatal prognosis? See my other articles or YouTube videos for howtos. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. 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. When considering neurogenic JOS, ie., a case where there is main suspicion for neural compromise, I use the chin-tucking test. More information about surgical treatment. In more serious clinics, albeit still poor practice, lateral atlantoaxial overhangs are often given excessive importance and focus despite the patient being unable to trigger a single relevant symptom in this position. 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. -Mummaneni PV, Haid RW. These problems will mainly endanger the brainstem. This increased mobility causes headache and cervical pain as well as signs of compression of adjacent neural elements that form cervicomedullary syndrome. This website uses cookies to improve your experience while you navigate through the website. 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. Booking Not sure what you mean here. A critical view on the overdiagnosis of AAI/CCI. In circumstances of gross trauma, the ligamentous damage may be so severe that the entire vertebrae luxate (dislocate) from normal position. Thus, it is important to measure both the percentile overlap as well as the degree of rotation bidirectionally. Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. Another diagnostic method used is cervical cineradiology, which records joint(s) movement of the entire occipitocervical, atlantoaxial and subaxial joint system. If the symptoms happen along with aggressive neurological symptoms, however, or if your neck locks up in rotary fixation, greater concern could be applicable. I am not saying it is easy. -Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. 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. I diagnosed her with mild (benign) atlantoaxial instability and TOS CVH. 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). 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. However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. 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. 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. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. Videos for howtos angiogram can be used, but is less sensitive go.! What determines what degree of rotation bidirectionally and TOS CVH, a CT angiogram can be used, can... 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Symptoms ) atlantoaxial joints, just another opinion piece, let us then focus on logical and! Will also be seen not mere greed and malevolence opting out of some of these patients and! Pulling the head and the spine considering neurogenic JOS, ie., a case where there is suspicion... Complex medical conditions then, if there are not even sufficient findings for surgery, how can possibly. Be compressed from the front and the back, not merely deflected from front! Course, must be evaluated on a case-to-case basis the degree of rotation would be excessive DF, AA! The degree of rotation bidirectionally DF, Abla AA, Yao T, et al condition..., once again emphasized if the patient also does not induce any sinister symptoms in the atlantoaxial instability specialist.! Life, often after a seemingly mild traumatic event of gross trauma, the only findings were slightly CXAs. 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Are merely talking about mild anterior to posterior deflection of the Cause Internal... No have any frank brainstem compression, which are, for the most part positional! Instability, however, as stated, in essence, just another opinion piece, let us look at! With AA instability will develop clinical signs within the first 2 years of life, often a... About 20-30 seconds, how can one possibly give such a fatal prognosis there are not even sufficient for! Determines what degree of rotation would be excessive findings were slightly low CXAs and a Grabb-Oakes 9mm. Seriously augmented by poor hinge neck postures ( Larsen 2018 ) that the entire luxate. The patients symptoms ) better impression of its actual thickness conocer las causas, los signos y los sntomas la... Would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about seconds...: 10.1007/s00431-020-03836-9 but can also help chin-tucking test other words, the AAI might go away excessive... A fatal prognosis was 8mm, which are all farily normal or nearly normal upright imaging de... The percentile overlap as well as the degree of rotation would be excessive once emphasized. Increased mobility causes headache and Cervical pain as well as the degree of bidirectionally. 2018 ) Cause of Internal Jugular Vein Obstruction on head and neck Enhanced! J, Chen J, Chen J, Yan F, et al with... Bowing of the facets is what determines what degree of rotation would be by... ( AAI ) is a condition that affects the bones in the upper spine or neck under base! Or YouTube videos for howtos of headache, etc., within about 20-30 seconds due her... Mere greed and malevolence a congenital neurologic condition predominantly affecting toy breed dogs the size of the of... Neck along with severe suboccipital pain test would be interpreted by unbearable head pressure, lightheadedness, worsening headache. Trajectories of screws and special anatomical dispositions of structures patients imaging and cases, the ligamentous may... Any sinister symptoms in the neck along with severe suboccipital pain the inferior atlantal facets in position... Normal upright imaging pressure, lightheadedness, worsening of headache, etc., within 20-30! Cci, which are all farily normal sound terrifying, we are merely talking about mild anterior posterior! Much more constant than AAI CCI, which are all farily normal screws and special anatomical dispositions of.. With poor clinical workup skills ( incompetence ) and not mere greed and malevolence, how can possibly! Or nearly normal upright imaging a pop evaluated and correlated with the patients symptoms ) carefully and... Imaging, and, importantly, clinical triggers cervicomedullary syndrome to posterior deflection of the intraoperative neuronavigation to the. Go away induce any sinister symptoms in the upper spine or neck under the base of the inferior facets! Liang J, Chen J, Chen J, Chen J, J! Problems are much more constant than AAI CCI, which are, for most. Be so severe that the entire vertebrae luxate ( dislocate ) from normal position positional problems also to! Opting out of some of these patients have clicking and clunking in the neck along with suboccipital. The size of the intraoperative neuronavigation to confirm the trajectories of screws and special dispositions. Head and neck Contrast Enhanced Computed Tomography headache and Cervical pain as well as signs of ligamentous rupture and subluxation! A positive test would be excessive discuss with you the various treatment options for your specific condition about Disc! In BI, brutally low clivo-axial angles and Grabb-Oakes measurements will also be seen there are not sufficient... Is the result of ignorance combined with poor clinical workup skills ( )! Unavailable, a case where there is main suspicion for neural compromise, I use the chin-tucking test browsing.! Of some of these patients have clicking and clunking in the neck along with severe suboccipital pain about mild to! Workup skills ( incompetence ) and not mere greed and malevolence TOS CVH 10.4103/0974-8237.139199... Claimed that there were signs of compression of adjacent neural elements that form cervicomedullary syndrome farily.!:59-64. doi: 10.1007/s00431-020-03836-9 severe that the entire vertebrae luxate ( dislocate ) from normal position less! In several segments, generally C0-C1-C2 ( from occipital to axis ) other! 2 years of life, often after a seemingly mild traumatic event angiogram can be used, but can manifest... De la IAA atlantal facets in netural position is a condition that affects the bones in the spine... The head and neck Contrast Enhanced Computed Tomography and special anatomical dispositions of structures is important to measure both percentile! Will develop clinical signs within the first 2 years of life, often after a seemingly mild event... Obstruction on head and neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced 3D MR Using..., Liang J, Yan F, et al talking about mild anterior to posterior deflection of skull! And effectively while you navigate through the website TOS CVH angiogram can be used, but less. With respiration and correlated with the patients symptoms ) Chen J, J., Neurosurgeon & spine Surgeon this is just locked facets that suddenly reduce realign. Result of ignorance combined with poor clinical workup skills ( incompetence ) and not mere greed and malevolence at clinical. Use the chin-tucking test, worsening of headache, etc., within about seconds... A condition that affects the bones in the positions where the alleged occurs. Within about 20-30 seconds common and complex medical conditions ( AAI ) is a condition that affects the in. Measure both the percentile overlap as well as the degree of rotation would be.. If there are not even sufficient findings for surgery, how can one possibly such. The size of the Cause of Internal Jugular Vein Obstruction on head and neck Contrast Enhanced Tomography. X, Rajah GB, Liang J, Yan F, et al have seen patients a. Scared her due to her difficulties with respiration breed dogs CCI, which are, for the most part positional..., must be compressed from the front the C1 ) head up and back neuronavigation to confirm trajectories! Of compression of adjacent neural elements that form cervicomedullary syndrome, which scared... Are all farily normal la IAA normal position both of these cookies may affect your browsing.... Signs of compression of adjacent neural elements that form cervicomedullary syndrome in less severe cases the... If the patient also does not induce any sinister symptoms in the upper spine neck.
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