Accessory nerve compression can cause weakness of the trapezius and sternocleidomastoid muscles, but can also cause cervical dystonia. Atlantoaxial (AAI) and craniocervical instability (CCI) are two potentially sinister diagnoses that cause damage to the segmental neurovascular structures due to overmobility of the upper cervical spine. Explore fellowships, residencies, internships and other educational opportunities. The surgeon may claim that because there is translational differences, meaning that the interval increases with movement, this is evidence of sinister CCI or AAI regardless of the measurement still being within normal limits. Now, for the record, I told the patient with 115 degrees that she does have CCI but that it is not causing her symptoms. Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. Atlas screws are generally placed in the lateral masses. J Bone Joint Surg Am. We can still treat it preventatively, but it wont resolve the symptoms. This conformation may be associated with thickening of the interarcuate ligament (atlantoaxial band), which has been interpreted as an indicator for instability in the atlantoaxial joint [79]. Does it matter whether these are done laying or sitting down? If not, does the patient actually have any significant symptom induction with rotation? 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. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. De Kleyn A, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes. The reason why AAI and CCI are potentially associated with so many symptoms such as headache, dizziness, etc., is due to the potential for neurovascular conflict. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. Or do you mean that there are positive improvement in symptoms despite the imaging being labeled as negative? 9/2017. 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. If this X-ray is repeated, the AAI might go away. It is better to let your doctor know if your son/daughter is having symptoms. Atlantoaxial rotary subluxations are overdiagnosed and often not measured properly. The ligaments supporting these joints are quite strong, but if they become Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. If combined with Chiari malformation, compression of the cerebellar tonsils can cooccur and will occur with lower measurements than normally needed to cause brainstem compression alone, due to filling of the space behind it (the descended cerebellum). As always, it is important to do a clinical radiological correlation to make an accurate assessment. PMID: 25210334; PMCID: PMC4158632. This can result in AAI where the bones are less stable and can damage the spinal cord. Foramen magnum decompression or syrinx manipulation was not performed in any patient. 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. Basilar invagination or dorsal migration of the dens, however, will mainly be evident in flexion but can (especially BI) also be seen in netural imaging. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. The atlantoaxial subluxation can occur isolated or can be found in cases in which there is also craniocervical instability. As mentioned initially in this article, craniocervical instability is mainly associated with jugular outlet obstruction and basilar invagination, whereas atlantoaxial instability can cause posteriorization of the dens and brainstem compression, or rotational dysfunction resulting in either bow hunters syndrome, Cock Robin syndrome or other variants of segmental luxations. 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). All conventional things like heart and lung problems, MS, cancer, infections etc. This website uses cookies to improve your experience while you navigate through the website. The same applies for conservative strategies to reduce internal jugular vein compression. The vast majority of these patients do NOT and this is important have clinical triggers suggestive of craniocervical or atlantoaxial instability, such as: LACK of symptoms when in neutral position (! And, of course, to determine whether or not the findings actually correlate with the patients symptoms and clinical exam. Anaesth Pain & Intensive Care 2018;22(2):238-242. Get the latest news, explore events and connect with Mass General. The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. A general neck MRI is usually a good idea and may show some arthritis in the atlantoaxial and atlanto-occipital joints along with minor intra-articular effusions, suggesting irritation of the joints. My poor baby has become completely lame and incontinent in the last 48 hours. Knattlia 2, 3038 All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. Thus, beware that a low clivo-axial angle (CXA) is often overinterpreted and abused as supportive evidence. 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. J Bone Joint Surg Am. A patient with positional brainstem compression due to TAL rupture, for example, will develop neurological (ie. 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. The procedure also comes with various inevitable side effects such as risk of screw failure, severe loss of neck mobility, risk of dural vein puncture as I have seen in several cases of c0-2 fusion, and more. 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. What muscles would need to be strengthened to prevent the ADI from opening up? JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. In other words, the vertical distance between the head and the spine. There can be, and are indeed many more potential explanations for these symptoms than just AAI and CCI. Rather, just like with the CXA, it is an indication of the present spinal health status and perhaps also an indicator as to non-surgical prognosis as well as an indicator of likely outcome if nothing is done. The patient will hinge back at their neck while simultaneously flexing the cranium. English +34 93 220 28 09 Espaol +34 93 198 34 24 We are committed to providing expert caresafely and effectively. KL TRENING & REHAB The ligaments involved are the transverse, alar and capsular ligaments. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. But if there is lots of space for the medulla, such invasive surgery simply is not warranted. Neurology. Atlanto-axial rotatory fixation. 2014). Atlantoaxial Instability Treatment. Care should be taken when positioning patients suspected of having this problem. For TOS CVH the patient will generally feel better when stress is reduced along with taking beta blockers (confer with your doctor). 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. This A 32 year-old female patient contacted me in 2019 as she had been diagnosed (by a radiologist alone) with craniocervical and atlantoaxial instability. 1978 Dec;37(6):525-8. doi: 10.1136/ard.37.6.525. Call 314-362-3577 for Patient Appointments. 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. Why do they have results tho when they correct the atlas/axis? 2008). Look for signs of retinal hypertension (subtle copper wiring, AV nicking, tortuosity of the arterioles, generalized vasospasm or papilledema. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. Dr. Gilete in Spain, although I often disagree with his diagnoses, tends to order beautiful dynamic CT scans and also good craniovascular scans. But opting out of some of these cookies may affect your browsing experience. 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. Some top offenders may suggest full craniocervical fusion, ie. 2011 Apr;15(1):41-47. Necessary cookies are absolutely essential for the website to function properly. More information about surgical treatment. Treatment, depending on the neurological symptoms and related pain, may be surgery. We'll assume you're ok with this, but you can opt-out if you wish. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. Elsevier Publishing. There are no exercises that can help an instability like that. Lack of signal change in the cord, and especially when it is not being compressed from both sides, is not a case of brainstem compression, Mild to moderate ligamentous compromise in cases where all measurements are normal or nearly normal, and there is no neurovascular compression, is generally NOT a surgical indication nor an indication for aggressive treatment. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. This can also promote anterior dissociation of the head which will lead to an abnormally high basion-axial interval (BAI Harris measurement) of more than 12mm (Ross & Moore, 2015). 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. In dogs with atlantoaxial subluxation, instability of the atlantoaxial joint results from a loss of ligamentous support of the axis, often with concurrent aplasia, hypoplasia or dysplasia of the dens. 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. 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. The reports I tend to get from these clinics are often laughable and full of guessing and overestimates. What is atlanto-axial instability? When Atlantoaxial instability occurs along with craniocervical instability, also known as occipitocervical instability (ie instability present also between skull and first cervical vertebra or Atlas), then fusion should consist of adding a fixation to the cranial bone through occipital or condylar screws which would give us as a whole C0 -C1-C2 posterior fusion. In less severe cases, physical therapy can also help. Call 314-362-3577forPatient Appointments. 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. Both patients had severe symptoms regardless of lying down, wearing a neck brace, etc., and did not get worse nor better when turning or moving their necks.