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). Please understand that no matter how bad you feel, pursuing the wrong diagnosis will not help. Some rare cases have also demonstrated rotary compression of the vertebral artery in the lower neck due to arthritis or disc bulges that fills up the transverse foraminae (Ujifuku et al. This means routine X-rays are not helpful. She started researching on certain online forums, in which she was advised to look into AAI and CCI. I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. Accessory nerve compression can cause weakness of the trapezius and sternocleidomastoid muscles, but can also cause cervical dystonia. Atlantoaxial subluxation frequently occurs in ligamentous and articular hypermobility syndromes such as Ehler Danlos syndrome. Global Spine J. Medical management entails strict cage rest and placing a neck brace (from in front of the ears to the mid-chest) to prevent the vertebrae of the neck from moving and causing more damage to the spinal cord. Spine (Phila Pa 1976). Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. 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. Atlas and axis screws are joined in each side by lateral bars that are unifying the instrumented fusion system. 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. -Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. Required fields are marked *. Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility. I believe that most of these practitioners mean well. to get a better impression of its actual thickness. The exam should be done lying down, without a neck pillow. 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. Imaging will prove brainstem compression on [flexion/extension] MRI, and an increased atlantodental interval on flexion/extension CT or x-ray. Because of its role in movement, it is, unfortunately, commonly injured. 2019) have documented numerous symptomatic cases of jugular vein stenosis at the craniovertebral junction. Copyright 2007-2023. Ujifuku K, Hayashi K, Tsunoda K, Kitagawa N, Hayashi T, Suyama K, Nagata I. Positional vertebral artery compression and vertebrobasilar insufficiency due to a herniated cervical disc. 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. 2014). 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. 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. Epub 2020 Oct 16. Moreover, tractioning the neck of these vulnerable patients can often cause undesirable effects. 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. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. When these muscles get tight (due to profound weakness), due to poor posture and movement patterns, or, as well, in many cases due to head or neck trauma, restricted joint movement will occur and popping and cracking, even loud clunks can occur. 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. are generally useless in most cases? Atlantoaxial instability is an uncommon condition of dogs in which there is abnormal movement in the neck, between the atlas (first cervical vertebra) and axis (second vertebra). 2011 Apr;15(1):41-47. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. 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. AAI is less common in adults with Down syndrome. 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. J Neurosurg Spine. 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 More commonly, however, a due to asymmetrical tearing of the covering ligaments, rotational subluxation or frank luxation is seen according to the Fielding & Hawking classifications (1977): Type 1, 2, 3 and 4, wherein types one and two are the most commonly encountered ones. Thanks for your help! Symptoms of brainstem compression are respiratory crisis and quadriplegia, but can also manifest more diffusely. A lot of things that cause temporary results are just placebo. 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. For example, I have seen patients with 45 degrees of rotation (which is higher than normal) between the C1-2 that had completely normal overlap due to large facets, and I have seen patients with 30 degrees of rotation (which is usually completely normal) with poor overlap and AAI, due to small facetal surfaces. 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. You also have the option to opt-out of these cookies. 1978 Dec;37(6):525-8. doi: 10.1136/ard.37.6.525. If your child has symptoms of AAI, the doctor will suggest an X-ray. 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. 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. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. 404-256-2633. <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. 3-Cranio-atlanto-axial instability, levels C0-C1-C2. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. 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. If not, does the patient actually have any significant symptom induction with rotation? These cookies do not store any personal information. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. Why would you jump to the worst possible explanation, and especially when lacking apt evidence? If the patient has a Grabb-Oakes of 18mm, however, and the transverse ligament is ruptured with the dens compressing the brainstem from the front and pushing it into the lamina behind it, then this is an emergency that requires timely surgical decompression. PMID: 19769514. 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. Learn about the many ways you can get involved and support Mass General. 10 things you should know about Cervical Disc Replacement. The patient should demonstrate some brainstem symptoms, and may develop quadriparesis if the compression is sufficiently hard and constant. This website uses cookies to improve your experience while you navigate through the website. It is advisable to obtain just a lateral view first. This webpage is intended to provide health information so that you can be better informed. 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. 2014 Feb;11(1):75-82. ncbi.nlm.nih.gov/pubmed/24321024, Higgins JN et al. 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. 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). 2012). Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. Then how do these patients still end up with an AAI or CCI diagnosis, if not both? had been excluded by her primary care physicians and local hospital. 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. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. November 19, 2014 at 8:19 pm. It is possible to do it with extension and rotation, etc., but it is usually not necessary. JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. 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. 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. DMX I dont recommend getting a DMX. A review of the diagnosis and treatment of atlantoaxial dislocations. E7. TOS is often considered a mere upper limb nerve pathology, but this is not the case. 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. Impression of its role in movement, it is advisable to obtain just a view! Also manifest more diffusely primary care physicians and local hospital to get a better impression of its actual.. 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