I told her that, although I dont think theres any evidence to suggests that the AAI is causing your symptoms, we should still treat it to prevent the risk of future frank luxations of the joints. 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. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. Both measurements tend to worsen with neck extension. Dissection of the vertebral and carotid arteries is fairly rare and can be excluded through a doppler ultrasound or CT angiogram. Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. Atlantoaxial instability treatment Contact Dr. Gilete C1 C2 fusion surgery Contact Dr. Gilete Our commitment to reliable health and medical information on the internet This site complies with 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). Luxation of the atlantoaxial joints, ie., luxation that surpasses what is seen in Cock Robin syndrome, can also occur with traumatic and gross ligamentous rupture. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. Early stage) and constant compression (if seen on mri, moderate, if seen on CT, severe) of these structures may occur. If its caused by rotation (rare), manipulation may temporarily improve jugular outlet passage, but it will not last. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. The abnormal imaging findings will mainly be evident during extension of the head and neck. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. This, again, prompted the more than 1000 euro consultation with the upright imaging center in a large european country. 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. But opting out of some of these cookies may affect your browsing experience. Facetal rigidity and dysarticulation is very common in patients with poor cervical postures and functionality of the neck muscles, and especially the muscles that restrict rotation and attach directly onto the spinous or transverses processes in the spine. 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. The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. Booking Some top offenders may suggest full craniocervical fusion, ie. My experience has been that these approaches do not work, and certainly do not cause long term results. 2012). It is advisable to obtain just a lateral view first. Musa et al. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. Gweon HM, Chung TS, Suh SH. Education 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. Thus we control the spinal cord and nerves (cranial and cervical) in order to avoid potential damages to these important structures. Traumatic Atlantoaxial Lateral Subluxation With Chronic Type II Odontoid Fracture: A Case Report. Dynamic angiograms could also be applicable in certain circumstances, cf. Anaesth pain intensive care 2020;24(1)69-86. Basil R. Besh, M.D. 1. Sometimes, an X-ray shows AAI when there are no symptoms. Thus, it is important to measure both the percentile overlap as well as the degree of rotation bidirectionally. Search for condition information or for a specific treatment program. In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. I consulted with her and reviewed her imaging: The quality of the images, first and foremost, was very low. Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. Atlantoaxial fixation: overview of all techniques. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. To compress the brainstem it must be compressed from both sides, both infront and behind. 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). I am not saying that this applies to every DMX center nor that DMX in and by itself is never useful, but due to the overwhelming lack of competence that tends to come with these studies, I dont recommend them unless unless you have obviously abnormal imaging otherwise and want to look for occult fractures or similar sinister and stubbornly identified problem. The ligaments supporting these joints are quite strong, but if they become Does it matter whether these are done laying or sitting down? This increased mobility causes headache and cervical pain as well as signs of compression of adjacent neural elements that form cervicomedullary syndrome. Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. Call us: 212.774.2837 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. DOI: 10.3171/2015.1.FOCUS14791. BDI, ie. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? In the cases where it is not possible to obtain autologous bone graft, heterologous graft (artificial bone) may also be used. Spine (Phila Pa 1976). Postural orthostatic tachycardia syndrome (POTS) and its relation to craniovascular dysfunction, Pectineo-femoral pinch syndrome: A common cause of groin & anterior thigh pain and weakness, Chronic spinal pain and radiculopathy: Diagnostic approach and common imaging pitfalls, Neurogenic genital pain: Pudendal neuralgia and inferior hypogastric plexalgia. We are committed to providing expert caresafely and effectively. If the latter, could be JOS obstruction, or could be placebo. The doctor will tell you which sports and activities are safe for your son/daughter. In such cases I tell my patients that, yes, you do have mild AAI, but it is not causing your symptoms. 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. zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. Testimonials If your child has symptoms of AAI, the doctor will suggest an X-ray. Surgical options, sometimes including relevant-level fusion, may be warranted in these circumstances. 2005 Dec;53(4):408-15. Review. Atlas screws are generally placed in the lateral masses. Upright cervical MRI in flexion, extension and maximal bi-directional rotation. 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. 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). 1963;13(5):386396. The instability present between these vertebrae can cause the vertebrae to shift and injure the spinal cord. How is possible for them to have results when there is no symptomatic AAI/CCI? 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 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. But opting out of some of these cookies may affect your browsing experience. Rather, she would feel awful in general and felt worsening with stress and arm- & shoulder loading, and being upright vs. lying down. Not sure what you mean here. Atlantoaxial instability (AAI) is a potential complication of all forms of EDS. 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. 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. Rather, it must be compressed by the dens ventrally, and flaval ligament and lamina posteriorly. 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. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. PMID: 19769514. 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. Dr. Christopher Williams | 07/09/2020. She started researching on certain online forums, in which she was advised to look into AAI and CCI. This madness must stop. Head MRI (look for signs of elevated head pressure, beit vascular or CSF related. 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. We can still treat it preventatively, but it wont resolve the symptoms. Thus, I recommend the following studies for craniovenous hypertension and TOS CVH: Craniovasculo-hypertensive disorders (mainly IIH, TOS CVH (!) 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. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a This website uses cookies to improve your experience while you navigate through the website. 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. However, I also told her that she may end up having fixation surgery in the future to prevent foreseeable compressive damage to the brainstem. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. 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. In other words, the vertical distance between the head and the spine. Call 314-362-3577 for Patient Appointments. This is no longer true. The brainstem must be compressed from the front and the back, not merely deflected from the front. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. Diagnostic imaging: Spine, 3rd edition. Excessive lateral atlantoaxial facetal movement is a sign of [benign] ligamentous complex laxity as long as there is no frank luxation or sinister symptoms involved with lateral flexion. Look for upright compression of the IJVs), Dynamic CT also works well, but has much more radiation. 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. 404-256-2633. Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. DMX I dont recommend getting a DMX. 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). Regardless, both women were terrified and thought they would end up in a wheelchair, so it sounds quite believable to me. Grabb-Oakes interval is another measurement that is often misunderstood. Second of all, if there is suggested ADI widening, but a high quality supine MRI with low slice thickness ascertains patency of the majority of the fibers of the TAL, the likelihood of actual complete rupture and future brainstem injury is extremely low. ), induction of symptoms (all or nearly all of your symptoms, not some neck pain) with maximal rotation, nor during flexion or extension. 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. In BI, the compression tends to be constant. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. 1963). 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). Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. I diagnosed her with mild (benign) atlantoaxial instability and TOS CVH. 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. 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. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. I, personally, although I created my own manipulation protocol for this problem ALMOST NEVER use it. 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. Another diagnostic method used is cervical cineradiology, which records joint(s) movement of the entire occipitocervical, atlantoaxial and subaxial joint system. Articles Spinnato P, Zarantonello P, Guerri S, Barakat M, Carpenzano M, Vara G, Bartoloni A, Gasbarrini A, Molinari M, Tedesco G. Atlantoaxial rotatory subluxation/fixation and Grisels syndrome in children: clinical and radiological prognostic factors. Dysautonomia when standing up is often related to craniovascular problems, whereas difficulty holding the head up suggests mumscular damage. 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. Uniondale, NY Location HSS Long Island The Omni. We also use third-party cookies that help us analyze and understand how you use this website. J Korean Soc Magn Reson Med. Her symptoms, however, did not at all change when changing her neck position and she had never had torticollis. 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. De Kleyn A, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes. DMX. 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. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. This is a major component in the workup for TOS CVH). 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. A review of the diagnosis and treatment of atlantoaxial dislocations. 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. This is not good medical practice. 2019 Oct;130:129-132. doi: 10.1016/j.wneu.2019.06.100. To schedule an appointment, call one of the offices, or book an appointment online. If nicely timed, around 20 secs after infusion, beautiful visualization of both arteries and veins is permitted). the section on bow hunters syndrome. First, need I mention the notion that there is tremendous money in this patient group, and that if treatment goes wrong, becuase they have already burned their bridges with their GPs, no one will listen nor care? Patient resources for the Down Syndrome Program. What cervical artificial disc should I choose? 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. 2014 Apr;5(2):59-64. doi: 10.4103/0974-8237.139199. 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.