We examined 404 patients with this chromosome disorder and observed their atlanto-dens intervals and spinal canal widths to be significantly different from children without Down syndrome. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. 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. Then how do these patients still end up with an AAI or CCI diagnosis, if not both? 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. The patient had headache, dizziness, fatigue, pain in the arms and chest and often felt difficulty breathing. 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, Patients with genuine and symptomatic rotational vertebral artery compression will develop symptoms of vertebrobasilar insufficiency when they fully rotate their heads to one or both directions, and may be further worsened if done simultaneous with neck extension (DeKleyn 1927). 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. 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). to get a better impression of its actual thickness. 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. Upright cervical MRI in flexion, extension and maximal bi-directional rotation. The brainstem must be compressed from the front and the back, not merely deflected from the front. This, of course, must be evaluated on a case-to-case basis. 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. 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. 2014 Feb;11(1):75-82. ncbi.nlm.nih.gov/pubmed/24321024, Higgins JN et al. 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. 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. 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. I see massive amounts of patients with alleged AAI who have normal atlantoaxial facetal overlap, and of course, also lacking clinical correlation. KL TRENING & REHAB 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. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. Let us help you navigate your in-person or virtual visit to Mass General. Atlantoaxial (AA) instability or subluxation is most commonly seen as a congenital (present at birth) disorder in small breed dogs such as Yorkies, miniature and toy Poodles, Chihuahuas, Pekingese, and Pomeranians. Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. My experience is that most of these patients suffer from craniovascular pathologies, not CCI and AAI. Moreover, I have heard numerous similar stories from other patients. Something I often see reported as alleged evidence of sinister CCI, is a translational BDI or BAI (the basion-axial interval is the horizontal distance between the tip of the clivus and the posterior wall of the odontoid process. Surgical options, sometimes including relevant-level fusion, may be warranted in these circumstances. In other words, the vertical distance between the head and the spine. Kjetil Larsen is a Researcher and a injury rehabilitation specialist, and is the owner of MSK Neurology. 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. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. If your son/daughter does not need surgery, it is important for him/her to be very careful playing sports or doing other physical activities. The abnormal imaging findings will mainly be evident during extension of the head and neck. J Craniovertebr Junction Spine. TOS increases perfusion rates to the brain, to which the brain is very sensitive and may dysfunction depending on how high the pressures are (Larsen et al 2020), often resulting in severe fatigue, dizziness, headaches and especially occipital headaches/pain (these are hypertensive headaches, not an atlas problem). Therefore before proposing surgery, the evaluation of each case must be done really carefully. Her symptoms, however, did not at all change when changing her neck position and she had never had torticollis. The instability present between these vertebrae can cause the vertebrae to shift and injure the spinal cord. From the beginning, the patient doubted my diagnosis that this was a craniovascular problem because she felt pain in the suboccipital area, had cracking and clunking, and felt compatible with several things she had read online and on facebook forums. Clunking and popping that occurs in the upper neck can be scary, but is usually just a sign of facetal rigidity with reduction, meaning that they get stuck and then pop back into place. But if there is lots of space for the medulla, such invasive surgery simply is not warranted. It will rarely cause frank luxation, however where the facets dislocate and lock laterally. This site complies with the HONcode standard for trustworthy health information: verify here. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. To schedule an appointment, call one of the offices, or book an appointment online. With the increasing dependence on smartphones, computers, and other devices in our modern This, usually due to trauma, but can also occur gradually due to certain autoimmune disorders such as rheumatoid arthritis, gross congenital hypermobility (such as Ehler Danlos syndrome or Marfan syndrome), or certain congenital syndromes such as Downs syndrome (Yang et al. 1963;13(5):386396. 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. Upright MRI has very low quality and because of this, there is a lot of guesswork involved in its interpretation. 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. 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. Get the latest news, explore events and connect with Mass General. Gweon HM, Chung TS, Suh SH. Deliganis AV, Baxter AB, Hanson JA, 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. 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. I dont recommend MRA. Once in the Operating Room, surgery is performed under general anesthesia, with Neurophysiological monitoring (SSEP somatosensory evoked potentials), neuronavigation guidance and intraoperative fluoroscopy guidance. Training is done carefully twice per week. 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. If this X-ray is repeated, the AAI might go away. 1. At Mass General, the brightest minds in medicine collaborate on behalf of our patients to bridge innovation science with state-of-the-art clinical medicine. 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. Safe Care CommitmentGet the latest news on COVID-19, the vaccine and care at Mass General.Learn more. The term AAI can also be used in cases of transverse ligament rupture, in which the odontoid process (the axis of the C2) may, especially if there is also damage to the tectorial membrane, dislocate dorsally and compress the brainstem. Moderator. 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. Explore fellowships, residencies, internships and other educational opportunities. Second, because it is such a controversial topic that lacks medical consensus, poor understanding of the actual mechanism of pathology leads to misunderstandings. When the bones or ligaments of the atlantoaxial complex are injured, the spinal cord is at particular risk for injury, and surgical treatment is often indicated. 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. Call 314-362-3577 for Patient Appointments. 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. 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. But opting out of some of these cookies may affect your browsing experience. The most commonly used measures in the radiological evaluation of craniocervical instability and atlantoaxial instability are CXA, Grabb, BDI, BAI, ADI. Rather, it must be compressed by the dens ventrally, and flaval ligament and lamina posteriorly. Booking This pain tends to get worse with stress and with high heart rates, and are often also worse in the morning after lying down. This is what I said from the beginning; AAI is not the cause of these symptoms, the exam and triggers do not fit. 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. 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. To compress the brainstem it must be compressed from both sides, both infront and behind. Signs of ligamentous damage. This would depend on whether or not the compression of the brainstem is constant, which again would depend on several factors. 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. Rev. DOI: 10.3171/2015.1.FOCUS14791. 2000). 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. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. Apr 2, 2022 Any experience of Atlantoaxial instability? A CTV is preferable, but a general neck CT will also do if you have sensitive kidneys and would like to avoid contrast infusion. How is one supposed to know, if no one knows what you have in the first place? The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a posterior fusion of the first cervical vertebra (C1 or Atlas) and the second cervical vertebra (C2 or Axis). Adapted from Problems with the upper spine in children and adults with Down syndrome (DS) by E. Margolis, B. Henry, B. Sandella and M. Stephens. This iatrogenic practice must come to an end. In reality, in legitimate cases of atlantoaxial or craniocervical instability, the instability may cause a potentially dangerous neurovascular conflict, as mentioned initially, where the brainstem or vertebral arteries can get damaged. In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. These problems are much more constant than AAI CCI, which are, for the most part, positional problems. Because it doesnt work most of the time, and doesnt cause any lasting results. One is especially predisposed to this problem if the affected vertebral artery is highly dominant (much higher caliber than its contralateral counterpart) or if the contralateral artery is extremely hypoplastic, or, finally, the contralateral artery terminates as the posterior inferior cerebellar artery rather than at the basilar artery (Josy & Daily, 2015). 2012). My symptoms are mostly sitting or standing but better laying down, wont doing the CT angiogram then become useless if I do it laying down (my symptoms are dysautonomia-like when standing). 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. Elsevier Publishing. 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. The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. Although there were no current grounds for surgery? Search for condition information or for a specific treatment program. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. Another scenario could be that the patient has been diagnosed with atlantoaxial rotary subluxations, as little facetal overlap, lets say, 15%, is seen upon bidirectional rotation. Epub 2014 May 22. Copyright Dr Gilete Neurosurgery & Spine Surgery. PMID: 19769514. De Kleyn A, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes. PMID: 30805289; PMCID: PMC6383461. 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