3. What cervical artificial disc should I choose? One or 2 out of every 100 children with Down syndrome have symptoms of AAI, but doctors do not know the exact number yet. La inestabilidad atlantoaxoidea (IAA) es una enfermedad que afecta los huesos de la parte superior de la columna vertebral. J NS 2015, V8 issue 4. 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. The atlantoaxial complex refers to the first two bones of the neck (C1,the atlas, and C2,the axis) as well as the associated collection of ligaments that connect the bones together and the blood vessels that travel through them to the brain. It 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. had been excluded by her primary care physicians and local hospital. See my other articles or YouTube videos for howtos. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. With the increasing dependence on smartphones, computers, and other devices in our modern 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! 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. 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. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. What I prefer to do is to first draw lines that show the actual rotational alignment of the C2 and C1 when looking left and right. Often times if surgery is required, the bones between C1 and C2 are fused together, requiring less than 48 hours of an in-hospital stay. However, as stated, in most cases this is just locked facets that suddenly reduce (realign) with a pop. Research has shown that normal limits are 3 and 10mm, with an absolutely maximum of 12mm (Ross & Moore 2015). 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 functional result of 9/2017. Clinical signs of such an injury include neck pain, weakness in all limbs, and potentially paralysis from the neck down and death. Surgery is often challenging because of the shape of the C1 and C2 bones, and because the vertebral arteriespass in and around these two bones on the way to the brain. A CTV is preferable, but a general neck CT will also do if you have sensitive kidneys and would like to avoid contrast infusion. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. Epub 2020 Oct 16. 2014 Apr;5(2):59-64. doi: 10.4103/0974-8237.139199. 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. The same applies for conservative strategies to reduce internal jugular vein compression. Another diagnostic method used is cervical cineradiology, which records joint(s) movement of the entire occipitocervical, atlantoaxial and subaxial joint system. Patients with legitimate CCI or AAI will generally have intermittent induction of symptoms with full rotation, flexion or extension that resolves in netural position, presuming there is no constant crushing of the brainstem or vertebral artery dissection. Copyright statement In such cases I tell my patients that, yes, you do have mild AAI, but it is not 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. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. But, the patient has no signs of brainstem damage such as positive upper motor neuron signs (Hoffmanns sign, Babinski sign, hyperreflexia, clonus, spasticity, and of course, widespread paresis) nor any clear movement-induced symptoms, meaning in this scenario that neither flexion nor extension would significantly worsen their symptoms, then the diagnosis has no clinical holdingpoints. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. Int J Spine Surg. The patient had headache, dizziness, fatigue, pain in the arms and chest and often felt difficulty breathing. Necessary cookies are absolutely essential for the website to function properly. A critical view on the overdiagnosis of AAI/CCI. This can result in AAI where the bones are less stable and can damage the spinal cord. Safe Care CommitmentGet the latest news on COVID-19, the vaccine and care at Mass General.Learn more. Common arguments for treatment may be claims that, although the MRI and even upright MRIs are normal, their own DMX scan is positive, or that the MRI, which was deemed normal by the local hospital, in reality shows signs of ruptured ligaments and that this fits with the patients symptoms. Because of its role in movement, it is, unfortunately, commonly injured. Thus, the patients in the rotary subluxation group are expected to present with severe and sudden neck pain as well as rigidity to the extent of being unable to move the neck. Call us: 212.774.2837 Treatment, depending on the neurological symptoms and related pain, may be surgery. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. Upright MRI has very low quality and because of this, there is a lot of guesswork involved in its interpretation. My poor baby has become completely lame and incontinent in the last 48 hours. This is one of the biggest offenders along with DMX and CXA, causing massive confusion, coercion, and misdiagnosis. Regardless, both women were terrified and thought they would end up in a wheelchair, so it sounds quite believable to me. Flexion and extension imaging fails to demonstrate any sort of brainstem compression. Bow hunters syndrome revisited: 2 new cases and literature review of 124 cases. There can be, and are indeed many more potential explanations for these symptoms than just AAI and CCI. In less severe cases, physical therapy can also help. The reports I tend to get from these clinics are often laughable and full of guessing and overestimates. Early stage) and constant compression (if seen on mri, moderate, if seen on CT, severe) of these structures may occur. Spine (Phila Pa 1976). 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. BDI, ie. Once in the Operating Room, surgery is performed under general anesthesia, with Neurophysiological monitoring (SSEP somatosensory evoked potentials), neuronavigation guidance and intraoperative fluoroscopy guidance. Headaches certainly can develop from instability of C1-2. The atlantoaxial instability may also have an acute traumatic origin, which may sometimes require urgent treatment, though in some cases it triggers development of the craniocervical or atlantoaxial instability. Wake up and walking begins on the second day after surgery. An X-ray is low-cost and low-risk, but it does not always tell whether a person has AAI or not. Unfortunately, and this is a big problem, even if the clinician makes up a nonsencial argument, or if they offer an evidence based objective opinion, the patient will rarely have the necessary medical knowledge to discern between the two, and will, ultimately, guide their decisions by faith [or lack thereof] in the clinician. Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. 10 things you should know about Cervical Disc Replacement. I completely disagree with this and, once again, refer to common sense thinking that if the joint positions are within normal limits then there is very little risk, if any, of any damage to the spinal cord or segmental arteries. Last Update [site_last_modified date_format=Y-m-d H:i:s]. PMID: 30805289; PMCID: PMC6383461. 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. 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. Thanks for your help! She was also said to have ventral brainstem compression, which particularly scared her due to her difficulties with respiration. 2021 Feb;180(2):441-447. doi: 10.1007/s00431-020-03836-9. The joint between the upper spine and base of the skull is called the atlanto-axial joint. 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. 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. Call 314-362-3577 for Patient Appointments. Journal of Neuro-Ophthalmology 2013;33:330337doi: 10.1097/WNO.0b013e318299c292, Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. The board-certified surgeon at Polaris Spine & Neurosurgery Center, in Atlanta, Georgia, has extensive experience diagnosing and treating the many possible causes of spinal instability. 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). Regardless, be it rooted in benevolent or malevolent intention, this does not change the fact that pursuing the diagnosis and especially its related treatment (conservative or surgical strategies) are extremely expensive and potentially dangerous as well. If unavailable, a CT angiogram can be used, but is less sensitive. Apr 2, 2022 Any experience of Atlantoaxial instability? I dont recommend MRA. 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). Eur J Pediatr. 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. Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. Musa A, Farhan SA, Lee YP, Uribe B, Kiester PD. doi: 10.1227/NEU.0b013e3182333859. Generally, however, in ligamentous laxity, some bowing and lateral hypermobility (evident by lateral flexion overhangs) will almost definitely not result in frank luxations down the line nor do they tend to elicit symptoms from the actual atlantoaxial facet joints. You mention to test for craniovascular pathologies, we should get a Doppler examination of the carotid and cerebral arteries done, and a CT angiogram done. Atlantoaxial fixation: overview of all techniques. The ligaments holding the bones together can also be injured in trauma, or weakened in certain inflammatory conditions such as rheumatoid arthritis or Downsyndrome. 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. Thus, I recommend the following studies for craniovenous hypertension and TOS CVH: Craniovasculo-hypertensive disorders (mainly IIH, TOS CVH (!) 2009), but this is extremely rare. For more information about these cookies and the data 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). 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. 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. Signs of ligamentous damage. Diagnostic imaging: Spine, 3rd edition. My experience has been that these approaches do not work, and certainly do not cause long term results. Now, it is true that specialty diagnoses can be missed by local generalists. 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. Moderator. 2. 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. He also found that severe misalignment of these joints were often associated with Chiari malformation, basilar invagination, and various other pathologies. In late stages, even the CTV will show severe compression, and at this stage, surgery may be the best option for resolution if there is clinical correlation. Privacy policy, Do you really have atlantoaxial and craniocervical instability? Atlantoaxial instability (AAI) is the term for increased motion at the joint between the 1st and 2nd cervical vertebrae (the atlas and the axis). Radiologic spectrum of craniocervical distraction injuries. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. E7. 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. 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. This category only includes cookies that ensures basic functionalities and security features of the website. 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. English. 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. 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! Contact, Terms & conditions In such a case, UMN symptoms and signs would be expected as well. 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. 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. 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). Congenital, inflammatory, traumatic, the section on bow hunters syndrome. 2000). Supine cervical MRI including T2-w sagittal-oblique sequences at 2mm slice thickness (disc and foraminal health is best evaluated on a supine MRI). (I will post the before- and after images when I return to Colombia in August, as they are on a separated hard drive). This madness must stop. collected, please refer to our Privacy Policy. In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. Pain medications and anti-inflammatories are typically also prescribed. 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. 10 things you should know about Cervical Disc Replacement.
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