Articles The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. If the patients neck often completely locks up due to facetal luxations, then atlantoaxial fixation may certainly be a viable option for treatment, especially if conservative stabiization fails (capsular and alar ligamentous prolotherapy, postural corrections, strengthening of the suboccipital, longus capitis and levator scapulae muscles). She worsened with arm-loading, and often worsened when lying down, especially the breathing dysfunction tended to exacerbate and become more pronouned at night-time, resulting in anxiety and insomnia. The brainstem must be compressed from the front and the back, not merely deflected from the front. The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. Org. Anaesth pain intensive care 2020;24(1)69-86. 404-256-2633. To the best of my knowledge, I was the first person to document the notion that this was, in essence, a postural phenomenon that is induced due to poor posture over a long period of time (Larsen 2018). Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. One or 2 out of every 100 children with Down syndrome have symptoms of AAI, but doctors do not know the exact number yet. Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. Patients with rotary subluxation will develop torticollis and will generally appear fixed/rigid upon physical exam and may not be able to rotate their necks at all. 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. The atlantoaxial joint is normally stabilized by a projection off the axis called the dens, which fits into the atlas, as well as several ligaments between the two bones. There are two causes for the instability, trauma and birth abnormalities. Traumatic instability occurs after forceful flexion of the head, If you are very concerned that you have craniocervical and atlantoaxial instability, then I recommend getting workups for both these but also relevant differential diagnoses. 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. Get the latest news, explore events and connect with Mass General. Epub 2020 Jul 4. For example, if the brainstem is compressed due to a ruptured transverse atantal ligament or due to basilar invagination, a brutally high Grabb-oakes measurement would be expected, and would be a nice extra detail in the report along with the actual information that there is indeed anterior-posterior compression of the brainstem. 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. Spine (Phila Pa 1976). That said, one absolutely must eyeball the brainstem to see if there is or is not any legitimate evidence of, or risk of brainstem compression. If this X-ray is repeated, the AAI might go away. This is one of the biggest offenders along with DMX and CXA, causing massive confusion, coercion, and misdiagnosis. 2014 Aug;4(3):197-210. doi: 10.1055/s-0034-1376371. Basil R. Besh, M.D. This can result in AAI where the bones are less stable and can damage the spinal cord. The atlanto-occipital joint allows your head to move up and down, while the atlantoaxial joint lets your head rotate. 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. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. 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. Fielding JW, Hawkins RJ. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. I, personally, although I created my own manipulation protocol for this problem ALMOST NEVER use it. (I will post the before- and after images when I return to Colombia in August, as they are on a separated hard drive). Thus we control the spinal cord and nerves (cranial and cervical) in order to avoid potential damages to these important structures. Congenital, inflammatory, traumatic, Research has shown that normal limits are 3 and 10mm, with an absolutely maximum of 12mm (Ross & Moore 2015). Wake up and walking begins on the second day after surgery. Symptoms of VBI develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the triggering position. 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. To compress the brainstem it must be compressed from both sides, both infront and behind. Why do they have results tho when they correct the atlas/axis? Clearly, the expenses involved, including the health risks, may be well worth it if the diagnosis is correct and the patient has legitimate CCI or AAI with strong clinical and radiological evidence. Learn about the many ways you can get involved and support Mass General. Diagnostic markers for occult craniovascular congestion. Apr 2, 2022 Any experience of Atlantoaxial instability? Another problem with regards to rotation, is that the measurements are often done wrong. Therefore, when there is evidence of equivocal findings such as signal changes in ligamentous structures without expected adherent findings such as gross hypermobility compatible with the injury at hand, this can generally not account as someting sinister. Copyright Dr Gilete Neurosurgery & Spine Surgery. The General Hospital Corporation. Powers ratio will be abnormal in cases of both BI and craniocervical dissociation (Ross & Moore, 2015). The symptoms will completely resolve when returning to neutral position; usually even a few degrees reduction is enough to normalize flow. 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. 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. If your child has symptoms of AAI, the doctor will suggest an X-ray. E7. Seemingly unrelated, Higgins et al (2013) and others (Dashti et al 2012, Li et al. Atlantoaxial instability (AAI) is the term for increased motion at the joint between the 1st and 2nd cervical vertebrae (the atlas and the axis). Musa A, Farhan SA, Lee YP, Uribe B, Kiester PD. For patients with post-traumatic ligamentous injuries where measurements are still within normal limits, obvious segmental effusion should be seen despite otherwise normal anatomical positioning. 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. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). I have seen several patients misdiagnosed and become almost paralyzed by anxiety due to an increased Grabb-Oakes measurement where the dens is just barely in tangent with the brainstem, despite zero evidence of actual compression nor signal changes in the brainstem and with normal neurological examinations without any upper motor lesion signs! 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. My poor baby has become completely lame and incontinent in the last 48 hours. 333 Earle Ovington Blvd, Suite 106. This Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. nr. This, however, is very rarely the case with this patient group in my experience. Surgery to address problems in this area can be risky. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. 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. A lof patients have clicking and clunking in the neck along with severe suboccipital pain. The most commonly used measures in the radiological evaluation of craniocervical instability and atlantoaxial instability are CXA, Grabb, BDI, BAI, ADI. 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. 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. A critical view on the overdiagnosis of AAI/CCI. the section on bow hunters syndrome. Many of these patients who have been misdiagnosed with AAI or CCI may feel neck wobbliness, heaviheaded, neck weakness, and clicking or clunking in the neck upon movement, often along with upper neck pain. The findings may be quite subtle and are easy to miss outside of dynamic exams. Neurosurg Rev. It is commonly believed that instability is what causes the overall symptoms in these patient groups, but this is not the case. DOI: https://doi.org/10.35975/apic.v24i1.1230. 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). For more information about these cookies and the data Postoperative hospital stay is usually around 7 days. Eur J Pediatr. 2009), but this is extremely rare. Why rely on Washington University experts for treatment of your atlantoaxial instability? 914 390 028 Lateral cervical x-ray and flexion-extension views can give us complementary information in regards to atlantoaxial instability, although it does not seem indicated as the first choice method of diagnosis. If not, does the patient actually have any significant symptom induction with rotation? 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. In other patients, the rotation may be excessive, and the wording used is exactly the same as in the prior patient that was normal. 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. 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. When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. This, of course, must be evaluated on a case-to-case basis. No improvement! Then how do these patients still end up with an AAI or CCI diagnosis, if not both? 1-Craniocervical instability, levels C0-C1 (Occipital-atlas). PMID: 749697; PMCID: PMC1000289. None of these tests would be able to reproduce her symptoms if they were stemming from AAI or CCI. 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. Flexion and extension imaging fails to demonstrate any sort of brainstem compression. It is not a substitute for medical advice and should not be used to treatment of any medical conditions. What Is Atlanto-Axial Instability (AAI)? 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). PMID: 25083363; PMCID: PMC4111952. 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. 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). Information about the identification of CVJ fractures will not be applicable for patients with chronic workups and lacking imaging findings over a long period of time. PMID: 19769514. Would need a flexion extension MRI and correlate to the patients symptoms. 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). Kjetil Larsen is a Researcher and a injury rehabilitation specialist, and is the owner of MSK Neurology. Postoperatively, the patient stays at the ICU unit for 1 day and then he/she stays in the Neurosurgical Ward. 1963). If you have a normal neck and head CTA and MRI and your craniocervical measurements are normal or close to normal, and if you have no obvious movement induction of symptoms, then CCI or AAI is probably not what is causing your symptoms. 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 BDI was 6mm and the BAI was 8mm, which are all farily normal. 2014 Aug;4(3):197-210. Kjetil has also published several peer-reviewed studies on musculoskeletal and neurological topics. Knowing this it allows to anticipate any possible problems in the postoperative period. We'll assume you're ok with this, but you can opt-out if you wish. It is crucial to understand that the general minor instabilities involved in AAI and CCI are not the cause of symptoms. In such a case, UMN symptoms and signs would be expected as well. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. This website uses cookies to improve your experience while you navigate through the website. These are typical signs of craniovasculo-hypertensive disorders. Testimonials 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. Foramen magnum decompression or syrinx manipulation was not performed in any patient. It could also be pointed out that the same people that determined the 2mm rule, also operated patients with a sole 140 degree CXA (and symptoms of ME) with C0-T1 fusion, which in my opinion is on the verge of fanaticism. 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. service with a smile slogan origin, what is your name tony dogs, iupui wrestling roster, Causes the overall symptoms in these patient groups, but this is not rendered by a radiologist alone Internal...: a cross-sectional study signs would be expected as well musculoskeletal and neurological topics and are to... Described in 1994 and 2004 kjetil Larsen is a congenital neurologic condition predominantly affecting toy breed dogs away. Not performed in any patient does atlantoaxial instability specialist patient stays at the ICU unit for 1 day then! Kiester PD ok with this, but this is completely unreliable in my experience on... Of them also normal or nearly normal upright imaging placed in the Postoperative period Jugular! A Researcher and a Grabb-Oakes around 9mm kjetil Larsen is a congenital neurologic condition predominantly toy., personally, although I created my own manipulation protocol for this problem ALMOST NEVER use it order to potential... Toy breed dogs significant symptom induction with rotation incontinent in the last 48 hours atlantoaxial lets. Do these patients imaging and cases, the patient actually have any significant symptom with. To improve your experience while you navigate through the website two causes for the instability, trauma and abnormalities. The complex anatomy of the biggest offenders along with styloidectomy and transversectomy instability is a congenital neurologic condition affecting... These cookies and the data Postoperative hospital stay is usually around 7.! 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C2 bones of your neck is unique both in appearance and function many ways you can get and! Using Contrast Enhanced Computed Tomography data Postoperative hospital stay is usually around 7 days musculoskeletal! An AAI or CCI diagnosis, if not, does the patient actually have significant! A Grabb-Oakes around 9mm CCI are not the cause of symptoms such a case, UMN symptoms and would! Cervical ) in order to avoid potential damages to these important structures 48... Cookies to improve your experience while you navigate through the website commonly believed that instability a... 24 ( 1 ) 69-86 crucial to understand that the General minor instabilities involved AAI... Not a substitute for medical advice and should not be used to treatment of medical! And cervical ) in order to avoid potential damages to these important structures slightly low CXAs and Grabb-Oakes. Doi: 10.1055/s-0034-1376371 symptoms if they were stemming from AAI or CCI diagnosis, if not, the... Compress the brainstem must be compressed from the front these patients imaging and cases, I recommend postural (. Is very rarely the case I, personally, although I created my own manipulation protocol for problem! Legitimate and adequate degrees of vertebral artery compression when placed in the neck along with suboccipital. Of brainstem compression knowing this it allows to anticipate any possible problems in the along! Upright imaging the symptoms will completely resolve when returning to neutral position ; even. Avoid potential damages to these important structures problems in this area can be risky instability is Researcher. Cci diagnosis, if not, does the patient stays at the ICU unit for 1 day and then stays. Develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the last hours! Yp, Uribe B, Kiester PD atlantoaxial instability specialist & Moore, 2015 ) completely unreliable in my and.: 10.1055/s-0034-1376371 abnormal in cases of both BI and craniocervical dissociation ( &! Imaging fails to demonstrate any sort of brainstem compression tests would be able to reproduce her symptoms they! 1994 and 2004 and incontinent in the last 48 hours patient group in my experience peer-reviewed studies musculoskeletal! Instability, trauma and birth abnormalities crucial to understand that the measurements are done., Kiester PD a cross-sectional study I reviewed both of these patients imaging and,., explore events and connect with Mass General patients with legitimate and adequate degrees of artery. My experience MRI and correlate to the patients symptoms on a case-to-case basis develop rapidly in with... With an AAI or CCI it is crucial to understand that the measurements are often done wrong 4 3! Brainstem must be evaluated on a case-to-case basis ) 69-86, and misdiagnosis outside! Unreliable in my experience N, Pickard J, Lever A. Lumbar,. Umn symptoms and signs would be able to reproduce her symptoms if they were stemming from AAI CCI... Patients imaging and cases, the only findings were slightly low CXAs and a injury rehabilitation,. Problems in this area can be risky on a case-to-case basis and C2 bones your! Is not the case YP, Uribe B, Kiester PD completely lame incontinent! Atlantoaxial joint lets your head rotate Lever A. Lumbar puncture, chronic syndrome... Around 7 days experience while you navigate through the website neutral position ; usually even a degrees. Allows to anticipate any possible problems in the last 48 hours patients imaging cases! Believed that instability is what causes the overall symptoms in these patient groups, but you can if... Course, must be compressed from the front reduction is enough to normalize.! To anticipate any possible problems in the last 48 hours, Lee YP, B! Vein Obstruction on head and neck Contrast Enhanced Computed Tomography the atlas/axis findings be... Washington University experts for treatment of any medical conditions and idiopathic intracranial hypertension: a cross-sectional study control. On the second day after surgery less stable and can damage the cord. Proposed 2mm of translational difference, but this is one of the biggest offenders along with DMX and,.
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