atlantoaxial instability specialist

Neuronavigation assistance guides us all through the surgery, thus it diminishes (though it does not eliminate) the risks while placing the screws for the fusion. Unfortunately, she was not compliant to the treatment that I prescribed (TOS, TOS CVH) other than the treatment for AAI, which she was convinced that was her problem. 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. Rather, she would feel awful in general and felt worsening with stress and arm- & shoulder loading, and being upright vs. lying down. 1978 Dec;37(6):525-8. doi: 10.1136/ard.37.6.525. 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. This is no longer true. Then, if there are not even sufficient findings for surgery, how can one possibly give such a fatal prognosis? Beware that suboccipital pain, espeically if your imaging is normal, is a very common sympton in thoracic outlet syndrome, and is actually a migraine variant. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. J Neurosurg Spine. An X-ray is low-cost and low-risk, but it does not always tell whether a person has AAI or not. 10 things you should know about Cervical Disc Replacement. In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. It is not a substitute for medical advice and should not be used to treatment of any medical conditions. The atlas can sublux anteriorly, posteriorly, laterally, or vertically. If it is, however then flexion/extension and rotational imaging to exclude positional facetal luxation is warranted. Musa A, Farhan SA, Lee YP, Uribe B, Kiester PD. I, personally, although I created my own manipulation protocol for this problem ALMOST NEVER use it. 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 I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. <9mm), which overestimate the pathologies and are much misunderstood due to unrealistic consensus of what is normal) will clearly be abnormal, such as the Harris measurement (BAI), basion dens interval (BDI), or Powers ratio. Atlantoaxial instability | Cervical Fusion or Prolotherapy PRP Stem Cell treatment options Surgical treatments for Cervical Instability Disc, disc, disc may be wrong, wrong, wrong In These problems are much more constant than AAI CCI, which are, for the most part, positional problems. Early stage) and constant compression (if seen on mri, moderate, if seen on CT, severe) of these structures may occur. 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. It is imperative to understand that patients with dagerous craniovertebral junction injuries, although one may sometimes require a dynamic CT or x-ray to identify them, will have clear imaging findings combined with clear clinical triggers in the utmost majority of incidences. How is one supposed to know, if no one knows what you have in the first place? In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. Atlantoaxial instability is a relatively frequent finding in individuals with Down syndrome. Why would you jump to the worst possible explanation, and especially when lacking apt evidence? 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). How is possible for them to have results when there is no symptomatic AAI/CCI? Treatment, depending on the neurological symptoms and related pain, may be surgery. The diagnosis can be made by means of an Upright MRI (magnetic Resonance Imaging) or with a cervical CT scan with 3D reconstruction. PMID: 25210334; PMCID: PMC4158632. TOS is also a common cause of dyspnea (respiratory difficulty), although these patients will have normal blood oxygen levels, which was also the case here. This category only includes cookies that ensures basic functionalities and security features of the website. If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? For example, although the medical literature (almost exclusively biased reports written by people considered experts on the topics (I am also biased on the topic; all experts are) may suggest a clivo-axial angle lower than 150 degrees as abnormal, this is still a measurement used to associate concrete craniocervical angles with medullary compression. But opting out of some of these cookies may affect your browsing experience. Traumatic Atlantoaxial Lateral Subluxation With Chronic Type II Odontoid Fracture: A Case Report. doi: 10.1227/NEU.0b013e3182333859. Surgical reduction and fixation would be the only appropriate treatment. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. 2012). Therefore, when I hear about patients being operated on with no other abnormality than a CXA of 140 degrees, my opinion is that this is reckless butchery. 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. The most commonly used measures in the radiological evaluation of craniocervical instability and atlantoaxial instability are CXA, Grabb, BDI, BAI, ADI. The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. 2-Atlantoaxial instability, levels C1-C2 (atlas-axis). Not sure what you mean here. Basil R. Besh, M.D. The patient should demonstrate some brainstem symptoms, and may develop quadriparesis if the compression is sufficiently hard and constant. It is important to understand that the size of the facets is what determines what degree of rotation would be excessive. 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. He also found that severe misalignment of these joints were often associated with Chiari malformation, basilar invagination, and various other pathologies. After the preoperative analysis of the Magnetic Resonance Imaging (MRI) and CT scan of each patient, we perform a thin sliced preoperative CT oriented towards neuronavigation that will be carried out during surgery. the section on bow hunters syndrome. English +34 93 220 28 09 Espaol +34 93 198 34 24 This website uses cookies to improve your experience while you navigate through the website. And, although there was zero evidence of brainsstem compression, she did indeed have subluxation of atlantoaxial joints with around 10% of overlap when turning to the side. Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, et al. It is, technically, possible to perform traction, reduction and fusion to obtain the same result, but this would be like killing a fly with a canon. Lateral bowing of the inferior atlantal facets in netural position is a sign of transverse atlantal ligament laxity. and craniovenous outflow obstruction) will frequently cause severe fatigue, migraine, headache, dizziness, tinnitus, pain in the upper neck/back of the head (this is hypertensive migraine, not atlas pain Larsen et al 2020), POTS, memory loss, cognitive decline or fluctuating cognitive ability, syncopal event, seizures, and even, sometimes, hemi or paraparesis and other stroke-like symptoms. PMID: 24475346; PMCID: PMC3899735. Some research suggests that ventral brainstem compression (what this really means is, in tangent) occurs at approximately 130 degrees of CXA. Due to the instability in the craniocervical junction deformation can occur to the brainstem, upper spinal cord, and cerebellum. Surgery to address problems in this area can be risky. PMID: 749697; PMCID: PMC1000289. In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. It is different from other joints in the vertebral Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. This may not apply for all of them, but it is a common problem which makes this patient group especially susceptible to become perfect victims of medical vulturism. Furthermore, a claim of brainstem stretching and kinking with resultant medullary microdamage that somehow not responds negatively to being stretched in real-time, and also lacking upper motor neuron signs, is not a very realistic claim. J Bone Joint Surg Am. 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. 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. 2021 Feb;180(2):441-447. doi: 10.1007/s00431-020-03836-9. 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. You can read more about these problems in my Myalgic encepalitis (link) and intracranial hypertension (linked earlier) articles as well as my 2018 and 2020 papers (Larsen 2018, Larsen et al 2020) in the reference lists if you think this may be you. The patient may seek out their GP or a local neurosurgeon who will, usually, and usually rightfully so, dismiss these claims, as the patients imaging is normal and also lack neurological signs that would fit with neurovascular compromise. 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. Otolaryngology Case Reports Volume 16, September 2020, 100201, Larsen K, Galluccio FC, Chand SK. I have seen countless reports from DMX centers where the patient, despite having normal or virtually normal conventional imaging, the patient is delivered reports of laughable quality, typically deeming the whole neck as unstable, despite the images being virtually normal. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. And if yes, do they completely normalize when resuming neutral position? The joint between the upper Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report. Atlantoaxial (AAI) and craniocervical instability (CCI) are two potentially sinister diagnoses that cause damage to the segmental neurovascular structures due to overmobility of the upper cervical spine. That said, yes, it is my opinion that the treatment is nonsense. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. This category only includes cookies that ensures basic functionalities and security features of the website. For more information about these cookies and the data I will update the article when I am back home in Colombia in the beginning of August. When rotated to the right, making sure that the axial alignment of the imaging program is aligned with the spinal column longitudinally, compare the anterior aspect of the right facet vs. the facet of the C2, and the posterior aspect of the left facet vs. the facet of the C2 and calculate the actual percentile of overlap. (I will post the before- and after images when I return to Colombia in August, as they are on a separated hard drive). Followup with a dynamic CT, supine MRI or similar to confirm potentially equivocal findings is warranted. Seemingly unrelated, Higgins et al (2013) and others (Dashti et al 2012, Li et al. Why do they have results tho when they correct the atlas/axis? Uniondale, NY Location HSS Long Island The Omni. Another common belief is that this mild deflection stretches the brainstem and somehow causes damage. 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. However, I also told her that she may end up having fixation surgery in the future to prevent foreseeable compressive damage to the brainstem. To compress the brainstem it must be compressed from both sides, both infront and behind. Int J Spine Surg. -Mummaneni PV, Haid RW. 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. But, if a specialist points something out that is not conventionally considered, he should either 1. make sure to emphasize the notion that it is a subtle finding with unsure actual clinical applicability or 2. make sure to prove his points through objective findings. 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. Aggressive craniovertebral junction ligamentous injuries can also result in vertical displacements. It is mandatory to procure user consent prior to running these cookies on your website. Education Upright cervical MRI in flexion, extension and maximal bi-directional rotation. DRAMMEN, NORWAY, Home Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. Atlantoaxial instability (AAI) is a potential complication of all forms of EDS. Neurologic signs of a cranial cervical myelopathy typically present at a young age and can range from cervical pain (hyperesthesia) to paralysis. Acta Otolaryngol. However, if there is obvious compromise of a ligament but there is no evidence of sinister hypermobility or structural displacement (eg., very high ADI), the ligamentous should be further examined with high-resolution T2 FLAIR imaging with low slice thickness (supine imaging!) Lack of signal change in the cord, and especially when it is not being compressed from both sides, is not a case of brainstem compression, Mild to moderate ligamentous compromise in cases where all measurements are normal or nearly normal, and there is no neurovascular compression, is generally NOT a surgical indication nor an indication for aggressive treatment. 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. 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. I believe that most of these practitioners mean well. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. Be sure to understand the mechanism of induction of symptoms in AAI and CCI before jumping on this potentially dangerous, and often financially devastating bandwagon! 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. PMID: 33064218. 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. Atlantoaxial and craniocervical instability are both real and potentially sinister diagnoses that require treatment. What cervical artificial disc should I choose? What cervical artificial disc should I choose? In addition to reproducible clinical triggers (positions), the patient should preferably undergo a dynamic catheter angiography of the neck. Finally, beware that many of these uMRI clinics render horrible images that barely show any anatomy, yet somehow still manage to determine various complicated diagnoses from them. Would need a flexion extension MRI and correlate to the patients symptoms. About In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. 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. Copyright Dr Gilete Neurosurgery & Spine Surgery. 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. 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. If you or your veterinarian is concerned that your Knattlia 2, 3038 2020). In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. There are no exercises that can help an instability like that. You can also get these images done to get peace of mind if you do not have strong neurological sequelae related to the popping, but beware that many of these specialist clinics diagnose AAI CCI no matter what your imaging looks like, and therefore I generally recommend working with larger hospitals. Booking Most dogs with AA instability will develop clinical signs within the first 2 years of life, often after a seemingly mild traumatic event. Because of its role in movement, it is, unfortunately, commonly injured. 333 Earle Ovington Blvd, Suite 106. 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. Learn about career opportunities, search for positions and apply for a job. Grabb-Oakes interval is another measurement that is often misunderstood. Last Update [site_last_modified date_format=Y-m-d H:i:s]. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. Now, the I was told is clearly second-hand information, and I cannot guarantee its accuracy. If the patient is indeed positionally symptomatic, however, and there is compatible imaging evidence, either atlantoaxial fusion, transverse foraminotomy or certain physical therapies may be warranted depending on how severe the findings and symptoms are. 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. The problem, in the patients eyes, may be a lacking reasonable counter-argument and counter-diagnosis that would explain his or her symptoms, which then prompts the patient to seek out alternative health care. 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). Dr. Gilete in Spain, although I often disagree with his diagnoses, tends to order beautiful dynamic CT scans and also good craniovascular scans. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. Instability in the hip can result in dislocation, ligament tears, muscle damage and wear of the joint. 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. -Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. Our surgeons can discuss with you the various treatment options for your specific condition. J Bone Joint Surg Am. In many circumstances, conservative treatment (Larsen 2018, atlas joint article as linked earlier) is appropriate. 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). Advanced Surgical Neuro-oncology Fellowship, Complex and Minimally Invasive Spine Deformity Fellowship, Endovascular Surgical Neuroradiology Fellowship, Neurosurgical Spine Innovation Fellowship, Neurosurgical Peripheral Nerve and Spine Fellowship. 1963;13(5):386396. Sometimes, the symptoms may trigger within a few minutes after the test as well, depending on various factors which exceed the scope of this article. Articles This conformation may be associated with thickening of the interarcuate ligament (atlantoaxial band), which has been interpreted as an indicator for instability in the atlantoaxial joint [79]. Another patient was told by a well-known pain physician in the US that she had brainstem compression and required several expensive prolotherapy procedures. And, of course, to determine whether or not the findings actually correlate with the patients symptoms and clinical exam. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. 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. E7. When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. Another diagnostic method used is cervical cineradiology, which records joint(s) movement of the entire occipitocervical, atlantoaxial and subaxial joint system. Risk in asymptomatic patients: If the patient has craniovertebral dissociation either due to anterior or superior migration of the head in relation to the cervical column, one may argue that there is a risk for traction injury to the brains blood supply even in cases where the patient has no obvious induction of symptoms upon flexion-, extension or rotation, and has no imaging that demonstrates neurovascular conflict (eg., BHS or positional brainstem compression). 2019 Feb 22;13(1):79-83. doi: 10.14444/6010. Look for signs of retinal hypertension (subtle copper wiring, AV nicking, tortuosity of the arterioles, generalized vasospasm or papilledema. He specializes in the treatment of chronic pain and has developed several distinctive protocols both with regards to diagnosis and conservative rehabilitation of difficult conditions. This can result in AAI where the bones are less stable and can damage the spinal cord. Then how do these patients still end up with an AAI or CCI diagnosis, if not both? 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. 2014 Aug;4(3):197-210. Presuming the central venous pressure being normal, then I am not so interested in the pre and post-stenotic gradients as they tend to be unreliable. Maybe they temporary fix some compression? This is a major component in the workup for TOS CVH). Secondly, and perhaps more importantly, the extent of facetal overap must be measured. AA instability is typically diagnosed by performing radiographs (x-rays) of the neck. Treatment depends on your son/daughters symptoms. The problem has received various names such as mere jugular vein compression, venous eagles syndrome, but I have called it jugular outlet syndrome (JOS), as it is a problem that not only affects the craniovenous outflow, but also several cranial nerves, and can be culpable in various strange neurological disorders (Read my atlas article (link) I also have an upcoming paper on this topic that I hope to release this or next year). Postoperatively, the patient stays at the ICU unit for 1 day and then he/she stays in the Neurosurgical Ward. The other side of the AAI/CCI coin is the risk for facetal luxation; a less sinister-, but still a problem that warrants surgical treatment. 3-Cranio-atlanto-axial instability, levels C0-C1-C2. I consulted with her and reviewed her imaging: The quality of the images, first and foremost, was very low. It is crucial to understand that the general minor instabilities involved in AAI and CCI are not the cause of symptoms. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. As stated, although rooted in postural dysfunction, this is not really a problem of pathological instability, and therefore I dont recommend neck fusion to treat this problem. 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). JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. Uniondale, NY 11553. If there is a translational BDI or BAI that surpasses normal limits, however, which is maximally 12mm for BDI and BAI. DMX. To schedule an appointment, call one of the offices, or book an appointment online. These cookies do not store any personal information. 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. But opting out of some of these cookies may affect your browsing experience. Org. PMID: 19769514. AAI and CCI are diagnoses that mainly cause the risk for either brainstem damage or injury to the arteries that supply the brain with blood, and this can cause paralysis or stroke if left untreated in cases where there is legitimate evidence for pathology. 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. 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. The problem begins when certain nonsensical articles about CCI and AAI, that do not properly explain relevant clinical correlation nor imaging requirements, but rather, just lists a set of associated symptoms, finds favor in the patient. Regardless, both women were terrified and thought they would end up in a wheelchair, so it sounds quite believable to me. PMID: 18708935. Atlanto-axial instability is a potentially dangerous condition where the ligament between the atlas (C1`) and axis (C2) vertebrae at the top of your neck is partially torn. Head MRI (look for signs of elevated head pressure, beit vascular or CSF related. This website uses cookies to improve your experience while you navigate through the website. Myran R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA. This is what I said from the beginning; AAI is not the cause of these symptoms, the exam and triggers do not fit. 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. Training is done carefully twice per week. J NS 2015, V8 issue 4. In such a case, UMN symptoms and signs would be expected as well. This may cause the patient to become afraid and to google their symptoms, which in and by itself is reasonable enough. Atlantoaxial fixation: overview of all techniques. The bones are susceptible to fracture from high-energy impact such as falls or car accidents, especially in the elderly. Congenital, inflammatory, traumatic, At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. Either way, if positive, move on to confirm narrowing of the jugular passage between the styloid process and C1 transverse process on a CT scan. She was also said to have ventral brainstem compression, which particularly scared her due to her difficulties with respiration. 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. 1977;59 (1): 37-44. Get the latest news, explore events and connect with Mass General. Both measurements tend to worsen with neck extension. What is atlanto-axial instability? This is important to understand, because maximal rotation will induce, and neutral position will stop the symptoms in patients with legitimate vascular conflict in AAI. Spine (Phila Pa 1976). 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. Once the diagnosis of atlantoaxial instabilityis made, one should consult the neurologist, neurosurgeon, and a geneticist if the patient is a child. Pearls and Other Issues The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. Surgical management is recommended for those with severe signs and for those who have tried and failed medical management. After hospital discharge, doctors usually control patients at least once a week after discharge on an outpatient basis, to make sure everything is correct before flying back home, thus we recommend to stay in Barcelona after discharge for 10-15 days. Why rely on Washington University experts for treatment of your atlantoaxial instability? Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. If the symptoms happen along with aggressive neurological symptoms, however, or if your neck locks up in rotary fixation, greater concern could be applicable. Research has shown that normal limits are 3 and 10mm, with an absolutely maximum of 12mm (Ross & Moore 2015). Get the latest news on COVID-19, the vaccine and care at Mass General. This article will take a critical look at these diagnoses and elaborate upon the factual structural risks that are seen in atlantoaxial- and craniocervical instabilities, as well as their expected realistic symptoms and triggers. Accessory nerve compression can cause weakness of the trapezius and sternocleidomastoid muscles, but can also cause cervical dystonia. This is easily seen on imaging, especially on CT, as the alignment of the joint will be unequivocally abnormal to the extent that would not be achievable without tremendous ligamentous injury. Medullopathy (signal changes, cord damage) will not occur by mere deflection, which is also evident by the blatant lack of upper motor neuron findings in these alleged brainstem compression patients. to get a better impression of its actual thickness. The BDI was 6mm and the BAI was 8mm, which are all farily normal. The atlanto-occipital joint allows your head to move up and down, while the atlantoaxial joint lets your head rotate. Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility. In the cases where it is not possible to obtain autologous bone graft, heterologous graft (artificial bone) may also be used. 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. This can also promote anterior dissociation of the head which will lead to an abnormally high basion-axial interval (BAI Harris measurement) of more than 12mm (Ross & Moore, 2015). If combined with Chiari malformation, compression of the cerebellar tonsils can cooccur and will occur with lower measurements than normally needed to cause brainstem compression alone, due to filling of the space behind it (the descended cerebellum). Posture is done for the rest of your life. But this is rarely the case in my experience. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. Patients with hyperrotation of the atlantoaxial joints can also develop Bow hunters syndrome (BHS). Headaches certainly can develop from instability of C1-2. The reason why AAI and CCI are potentially associated with so many symptoms such as headache, dizziness, etc., is due to the potential for neurovascular conflict. If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. The atlantoaxial complex is primarily responsible forenabling the head to rotate, or turn to the left and right, while also protecting the spinal cord from injury. Privacy policy, Do you really have atlantoaxial and craniocervical instability? However, appropriate inclusive criteria must be used to render the diagnoses; subtle findings and the lack of a strong clinical correlation is not enough, and will easily lead to misdiagnosis and related anxiety and suffering. Due to the poor practice integrity that is often associated with DMX imaging, despite these modalities indeed having some utility in certain cases, I cannot recommend having them done unless done in a serious hospital without a financial incentive (ie., without financial connections to the clinician ordering them), and without a very obvious scope of investigation that could not already be seen in MR or CT imaging. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? This iatrogenic practice must come to an end. Sometimes flexion-extension and rotational imaging is necessary. The mission of FORM Ortho is to be the preferred provider of orthopedic care and occupational health amongst our community, case managers and primary care physicians. Atlantoaxial subluxation frequently occurs in ligamentous and articular hypermobility syndromes such as Ehler Danlos syndrome. Save my name, email, and website in this browser for the next time I comment. Pain medications and anti-inflammatories are typically also prescribed. It is, as we say, in tangent with the dens and tectoral ventrally alone. Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. In less severe cases, physical therapy can also help. Copyright 2007-2023. Patient resources for the Down Syndrome Program. 2014 Aug;4(3):197-210. doi: 10.1055/s-0034-1376371. Explore fellowships, residencies, internships and other educational opportunities. Moreover, I have heard numerous similar stories from other patients. 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. Although the complete differentiation between this and CCI or even occipital neuralgia is something that is complicated and must be done on individual basis after examination, we can, in essence, say that suboccipital pain that worsen with shoulder loading tends to be TOS or occipital neuralgia, whereas suboccipital symptoms that induce when lying down or being upright regardless of neck position tends to be TOS CVH. Jugular outlet obstruction is commonly seen in patients with upper cervical horizontal facetal misalignment, and especially if they have broad transverses processes or a posteriorly angulated styloid process (Gweon et a. A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. This site complies with the HONcode standard for trustworthy health information: verify here. These cookies will be stored in your browser only with your consent. Neurology. Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. Thus, it is important to measure both the percentile overlap as well as the degree of rotation bidirectionally. A CTV is preferable, but a general neck CT will also do if you have sensitive kidneys and would like to avoid contrast infusion. 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. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. 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). The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. I recommend first measuring the degree of rotation between the C1 and C2 by drawing a line from the bifid process to the middle of the anterior aspect of the vertebra, and then another line from the posterior to the anterior tubercles of the C1. Apr 2, 2022 Any experience of Atlantoaxial instability? Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. When Atlantoaxial instability occurs along with craniocervical instability, also known as occipitocervical instability (ie instability present also between skull and first cervical vertebra or Atlas), then fusion should consist of adding a fixation to the cranial bone through occipital or condylar screws which would give us as a whole C0 -C1-C2 posterior fusion. Call 314-362-3577 for Patient Appointments. 2008). This webpage is intended to provide health information so that you can be better informed. 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. 1927;11(1):155157. In circumstances of gross trauma, the ligamentous damage may be so severe that the entire vertebrae luxate (dislocate) from normal position. A 32 year-old female patient contacted me in 2019 as she had been diagnosed (by a radiologist alone) with craniocervical and atlantoaxial instability. In such cases I tell my patients that, yes, you do have mild AAI, but it is not causing your symptoms. Must be carefully evaluated and correlated with the patients symptoms). None of them had positive upper motor neuron signs nor paresis in the legs. Tambin conocer las causas, los signos y los sntomas de la IAA. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. Both neurophysiological monitoring and neuronavigation guidance are safety measures for the patient. In cases of hyperlaxity, It is not uncommon to find subaxial cervical alterations (levels below C3 to C7 . Bow hunters syndrome revisited: 2 new cases and literature review of 124 cases. Neurosurgery. 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. Contact, Terms & conditions We did the Edens, Roos and Morleys tests for thoracic outlet syndrome, which were all positive. 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). All conventional things like heart and lung problems, MS, cancer, infections etc. 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. I hope that, by now, the reader has understood the importance that clinical measurements, actual pathology and clinical triggers should go hand in hand. Additionally, spinal instability in the form of spondylolisthesis That is why they are much less affected by actual neck position than legitimate CCI AAI patients are, and certainly do not become symptom free in neutral positions. If the measurements are within normal limits, the likelihood of dangerous sequelae are low, if not absent. This Case Rep Neurol 2019;11:295298, Waldock WJ, Higgins NJ, Axon P. A case report of gastroparesis resolved by styloidectomy. Epub 2020 Oct 16. I very often receive upright MRI reports where the rotation is completely normal, and the patient is still diagnosed with AAI. For example, if the patient blacks out every time she turns her head to the left, a followup dynamic catheter angiography could be done, and may demonstrate high-grade stenosis of the vertebral artery when turning to the left. 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. The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. It is not due to mild overall instability that does not cause neurovascular conflicts. Let us help you navigate your in-person or virtual visit to Mass General. Exam for bow hunters syndrome is done dynamically, but thats aother exam. 2019 Oct;130:129-132. doi: 10.1016/j.wneu.2019.06.100. If a gliding is causing it (it is usually a glide or, a glide combined with mild rotation), no manipulation can fix it. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. Second, because it is such a controversial topic that lacks medical consensus, poor understanding of the actual mechanism of pathology leads to misunderstandings. It should be stressed that C1-C2 fusion, indicated by symptomatology, results in the natural cancellation of C1 over C2 movement so it results in approximately a deficit of 50% of the rotation of the neck. 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. I recommend doing this with a neuro-ophthalmologist, not a general ophthalmologist or opticician, as the findings are often missed. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. Her symptoms, however, did not at all change when changing her neck position and she had never had torticollis. Call 314-362-3577forPatient Appointments. zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. This, with or without accompanied neurological symptoms, be it vascular or neurological. Mild to moderate cases tend to respond well to appropriate conservative therapy (not general therapy), cf., once again, my atlas joint article from 2017 linked several times earlier. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. And, fair enough, I do not expect blind trust nor compliance. In the Axis, pedicle screws are usually the first choice although, depending on the patients anatomy, placement of isthmic screws may be considered. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). PMID: 30805289; PMCID: PMC6383461. The BDI indicates vertical-, and the BAI horizontal structural integrity. Global Spine J. (Fixed rotatory subluxation of the atlanto-axial joint). BHS implies rotational compression of the vertebral arteries, which are two out of four arteries that supply the brain (two internal carotid and two vertebral arteries). Traumatic ligamentous ruptures or gradual deterioration of joint stability may cause basilar invagination, which is a degenerative process causing the odontoid process to graduall migrate into the head via the foramen magnum. Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. In the congenital form of AA instability, the animal is born with abnormal bony or ligamentous connections between the first two vertebrae in the neck. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. ADI laxity is mainly caused by head and neck trauma, so as long as you avoid future collisions, it will probably not deteriorate. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. Horizontal misalignment of the facet joints often cause dorsal migration of the C0 and C1 facets which cause approximation of the styloid process and the C1 transverse processes. Strong evidence of clinical correlation must be present from a clinician that is familiar with the signs and triggers in upper cervical instability-cases. But this measurement in and by itself, when it is 9 or 10 or even higher, but there is no brainstem compression not even in flexion-extension imaging this cannot be interpreted as a surgical indicator. 2000). Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. Copyright Dr Gilete Neurosurgery & Spine Surgery. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. Dynamic angiograms could also be applicable in certain circumstances, cf. And, she still had the same symptoms! It does certainly insinuate some instability and ligamentous laxity, and can certainly result in greater level of wearing and tearing of the facet joints and causing some neck pain and joint effusions, but it can not be said to be any form of sinister AAI or CCI due to lacking neurovascular conflicts. These problems will mainly endanger the brainstem. Symptoms of VBI develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the triggering position. What muscles would need to be strengthened to prevent the ADI from opening up? Kjetil has also published several peer-reviewed studies on musculoskeletal and neurological topics. 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. My experience is that most of these patients suffer from craniovascular pathologies, not CCI and AAI. The natural anatomic C1-C2 movement is basically rotation and approximately implies 50% of necks total rotation movement. Therefore before proposing surgery, the evaluation of each case must be done really carefully. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. The joint between the upper spine and base of the skull is called the atlanto-axial joint. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy.

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