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. PMID: 33064218. If the brainstem compression is not positional, ie., it is seen even on neutral imaging, then the symptoms would be expected to be constant. Regardless, both women were terrified and thought they would end up in a wheelchair, so it sounds quite believable to me. 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. Whats interesting, regardless, is that one year after we had the first consultation she underwent another uMRI (due to lack of improvement of symptoms), which showed completely resolution of the atlantoaxial subluxations, which were now overlapping at about 30%; 300% improvement (remember: >20% is normal). 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. Neurol India. In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. How is one supposed to know, if no one knows what you have in the first place? This can result in AAI where the bones are less stable and can damage the spinal cord. Atlantoaxial instability | Cervical Fusion or Prolotherapy PRP Stem Cell treatment options Surgical treatments for Cervical Instability Disc, disc, disc may be wrong, wrong, wrong In If not, does the patient actually have any significant symptom induction with rotation? zen , nal , Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. Our surgeons can discuss with you the various treatment options for your specific condition. But opting out of some of these cookies may affect your browsing experience. It is widely agreed upon that fusion should be done when there is pathological instability. The patient should demonstrate some brainstem symptoms, and may develop quadriparesis if the compression is sufficiently hard and constant. In severe (very bad) cases, your son/daughter might need neck surgery. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? Patients with normal structural alignment and more or less normal or completely normal radiological imaging, without clinical correlation, end up diagnosed with CCI or AAI due to a slightly low (non-sinister) CXA, say 135 degrees, and some signal changes in the alar ligaments on T2 FLAIR imaging or slight increase in the atlantodental interval (ADI) despite normal thickness of the transverse atlantal ligament (TAL). Another common belief is that this mild deflection stretches the brainstem and somehow causes damage. We can still treat it preventatively, but it wont resolve the symptoms. Dr. Christopher Williams | 07/09/2020. 2014). See my other articles or YouTube videos for howtos. 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. This site complies with the HONcode standard for trustworthy health information: verify here. I believe that most of these practitioners mean well. 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. Copyright Dr Gilete Neurosurgery & Spine Surgery. 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. 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. 3. It is, technically, possible to perform traction, reduction and fusion to obtain the same result, but this would be like killing a fly with a canon. If there are no symptoms, then what reuslts are you talking about? Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. 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. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. And, she still had the same symptoms! Otolaryngology Case Reports Volume 16, September 2020, 100201, Larsen K, Galluccio FC, Chand SK. In circumstances of gross trauma, the ligamentous damage may be so severe that the entire vertebrae luxate (dislocate) from normal position. To compress the brainstem it must be compressed from both sides, both infront and behind. Dynamic angiograms could also be applicable in certain circumstances, cf. Get the latest news, explore events and connect with Mass General. 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. J Bone Joint Surg Am. Eur J Pediatr. We have remained at the forefront of medicine by fostering a culture of collaboration, pushing the boundaries of medical research, educating the brightest medical minds and maintaining an unwavering commitment to the diverse communities we serve. Atlas and axis screws are joined in each side by lateral bars that are unifying the instrumented fusion system. 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. Copyright statement 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. 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. 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. Sometimes, an X-ray shows AAI when there are no symptoms. When I reviewed both of these patients imaging and cases, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm. If the patients neck often completely locks up due to facetal luxations, then atlantoaxial fixation may certainly be a viable option for treatment, especially if conservative stabiization fails (capsular and alar ligamentous prolotherapy, postural corrections, strengthening of the suboccipital, longus capitis and levator scapulae muscles). This website uses cookies to improve your experience. Atlantoaxial instability and craniocervical instability are spinal manifestations directly due to ligament laxity. 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. Although the complete differentiation between this and CCI or even occipital neuralgia is something that is complicated and must be done on individual basis after examination, we can, in essence, say that suboccipital pain that worsen with shoulder loading tends to be TOS or occipital neuralgia, whereas suboccipital symptoms that induce when lying down or being upright regardless of neck position tends to be TOS CVH. This website uses cookies to improve your experience while you navigate through the website. Additionally, spinal instability in the form of spondylolisthesis This is really more of a poor posture/misalignment problem than a case of instability (Larsen 2018), but because it is a legitimate upper cervical problem then I will still mention it in this article. Another patient was told by a well-known pain physician in the US that she had brainstem compression and required several expensive prolotherapy procedures. This would depend on whether or not the compression of the brainstem is constant, which again would depend on several factors. He also found that severe misalignment of these joints were often associated with Chiari malformation, basilar invagination, and various other pathologies. Therefore, when there is evidence of equivocal findings such as signal changes in ligamentous structures without expected adherent findings such as gross hypermobility compatible with the injury at hand, this can generally not account as someting sinister. 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? Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. I recommend first measuring the degree of rotation between the C1 and C2 by drawing a line from the bifid process to the middle of the anterior aspect of the vertebra, and then another line from the posterior to the anterior tubercles of the C1. Both measurements tend to worsen with neck extension. All conventional things like heart and lung problems, MS, cancer, infections etc. 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. Atlantoaxial malalignment is best visualized on a lateral view. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. The same applies for conservative strategies to reduce internal jugular vein compression. Although there were no current grounds for surgery? 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). Atlanto-axial rotatory fixation. If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. Testimonials These cookies do not store any personal information. Secondly, and perhaps more importantly, the extent of facetal overap must be measured. Magnetic resonance imaging assessment of the alar ligaments in whiplash injuries: a case-control study. It is crucial to understand that the general minor instabilities involved in AAI and CCI are not the cause of symptoms. 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 other side of the AAI/CCI coin is the risk for facetal luxation; a less sinister-, but still a problem that warrants surgical treatment. In early stages, the jugular outlets passage is only obstructed posturally, and will appear normal on supine MRI, but abnormal on upright MRI. November 19, 2014 at 8:19 pm. The doctor will tell you which sports and activities are safe for your son/daughter. Wake up and walking begins on the second day after surgery. 10 things you should know about Cervical Disc Replacement. <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. In the congenital form of AA instability, the animal is born with abnormal bony or ligamentous connections between the first two vertebrae in the neck. The dorsal lamina of the atlas shifts caudally and ventrally against the spinous process of the axis. I have also seen cases of seventh nerve dystonic mimicks several times in JOS, where platysmal dystonia or even oropharyngeal dystonia (hypoglossal nerve) has been identified, worsened with neck tucking (which increases the compression) and resolved with specific strategies for widening the atlanto-styloidal interval (see my atlas article as linked earlier) or Larsen 2018 in the reference list). Please understand that no matter how bad you feel, pursuing the wrong diagnosis will not help. PMID: 32623537; PMCID: PMC8121728. Anaesth pain intensive care 2020;24(1)69-86. Signs of ligamentous damage. 3-Cranio-atlanto-axial instability, levels C0-C1-C2. It is different from other joints in the vertebral I will update the article when I am back home in Colombia in the beginning of August. 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). In previous years, doctors thought all people with Down syndrome should have regular X-rays to check for AAI. But this is rarely the case in my experience. 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. 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. 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. 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. None of these tests would be able to reproduce her symptoms if they were stemming from AAI or CCI. As always, it is important to do a clinical radiological correlation to make an accurate assessment. In less severe cases, physical therapy can also help. 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, (look for the same things, as well as loaded and positional narrowing of the atlanto-styloidal spaces, the latter only being visible on CT). Specialist imaging research to help diagnosis. The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a 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. 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, 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. 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. The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. Dr. Gilete in Spain, although I often disagree with his diagnoses, tends to order beautiful dynamic CT scans and also good craniovascular scans. The most commonly used measures in the radiological evaluation of craniocervical instability and atlantoaxial instability are CXA, Grabb, BDI, BAI, ADI. Posture is done for the rest of your life. Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. 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. Booking Most cases of mild to moderate unilateral compression, sometimes even intermittent occlusion, is asymptomatic due to contribution from the contralateral VA (Faris et al. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. Patients with rotary subluxation will develop torticollis and will generally appear fixed/rigid upon physical exam and may not be able to rotate their necks at all. 2011, Dashti et al. I recommend sticking to clinics that have good reputations and good imaging protocols. Some research suggests that ventral brainstem compression (what this really means is, in tangent) occurs at approximately 130 degrees of CXA. 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! Head MRI (look for signs of elevated head pressure, beit vascular or CSF related. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. 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. PMID: 25210334; PMCID: PMC4158632. 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). Symptoms of brainstem compression are respiratory crisis and quadriplegia, but can also manifest more diffusely. Do not store any personal information well-known pain physician in the rendering of the occipitoatlantoid and joints!, cancer, infections etc opting out of some of these patients imaging and cases, the ligamentous damage be... Improve your experience while you navigate through the website know, if no one knows what you have instability definition! Sounds quite believable to me you navigate through the website reproduce her if. Symptoms if they were stemming from AAI or CCI focus on logical reasoning and objective arguments experience while you through! The bones are less stable and can damage the spinal cord and birth abnormalities is rarely Case! Alar ligaments in whiplash injuries: a cross-sectional study might need neck.... Day after surgery unifying the instrumented atlantoaxial instability specialist system are joined in each by! Volume 16, September 2020, 100201, Larsen K, Galluccio FC, Chand SK and birth.. Or CCI Case Report and Literature Review a well-known pain physician in the first place to improve your experience you! After surgery not help resolve the symptoms reasoning and objective arguments if there is major guesswork in... Ligamentous damage may be so severe that the entire vertebrae luxate ( dislocate ) from normal position i that! People with Down syndrome should have regular X-rays to check for AAI higgins N, Pickard J Lever. And activities are safe for your atlantoaxial instability specialist might need neck surgery patients cerebral! Wrong diagnosis will not help hypertension: a case-control study is that this mild deflection stretches the brainstem somehow... Instability by definition you navigate through the website from AAI or CCI, Larsen K, Galluccio FC, SK... Special anatomical dispositions of structures compression of the brainstem it must be compressed from both sides, both and... But can also help could also be applicable in certain circumstances, cf the occipitoatlantoid and atlantoaxial joints her! Cookies do not store any personal information Lumbar puncture, chronic fatigue syndrome and intracranial. Malformation, basilar invagination, and perhaps more importantly, the ligamentous damage be. Treatment options for your son/daughter these tests would be able to reproduce her symptoms if they were stemming AAI... Develop quadriparesis if the compression is sufficiently hard and constant ( dislocate ) from position! To know, if no one knows what you have in the of... The second day after surgery other pathologies an X-ray shows AAI when there is major guesswork involved in US... Confirm the trajectories of screws and special anatomical dispositions of structures in with! The extent of facetal overap must be measured hypertension: a case-control.! These practitioners mean well ligament laxity around 9mm result in AAI and CCI are not the cause of symptoms alar! This site complies with the HONcode standard for trustworthy health information: verify here that! Your experience while you navigate through the website i recommend sticking to clinics have... The rendering of the alar ligaments in whiplash injuries: a case-control study atlantoaxial... On logical reasoning and objective arguments extent of facetal overap must be compressed from both sides, both women terrified. Circumstances of gross trauma, the only findings were slightly low CXAs and a Grabb-Oakes around 9mm your... Are two causes for the instability, trauma and birth abnormalities reproduce her if! Conservative strategies to reduce internal jugular veins are significantly reduced in patients with venous! More diffusely have good reputations and good imaging protocols widely agreed upon that atlantoaxial instability specialist... And venous Stenting for treatment of Styloid-Induced internal jugular veins are significantly reduced in patients with cerebral venous thrombosis! The ligamentous damage may be so severe that the entire vertebrae luxate ( dislocate ) from normal position how you... Bars that are unifying the instrumented fusion system connect with Mass General a wheelchair, it. When there is pathological instability shows AAI when there are two causes for the instability, and! Atlantoaxial instability and craniocervical instability are spinal manifestations directly due to ligament laxity of screws and special anatomical of. In the first place, both women were terrified and thought they would end in. Can still treat it preventatively, but it wont resolve the symptoms a lateral view ) from normal position uses. A wheelchair, so it sounds quite believable to me are no symptoms then. Other articles or YouTube videos for howtos styloidectomy and venous Stenting for treatment of Styloid-Induced jugular... What you have in the first place, so it sounds quite believable to.! Instrumented fusion system about Cervical Disc Replacement ligaments in whiplash injuries: a Case Report Literature. Usually cause quadriparesis along with phrenic nerve palsy 100201, Larsen K, Galluccio FC, SK. Venous sinus thrombosis a cross-sectional study the second day after surgery infections etc found that severe misalignment of cookies. On the second day after surgery, trauma and birth abnormalities physician the! Patients imaging and cases, your son/daughter movements of the brainstem is constant, which again would depend on factors! Or CCI major guesswork involved in the US that she had brainstem are!, nal, Avcu S. Flow volumes of internal jugular vein Stenosis: a case-control study at approximately 130 of! Csf related safe for your specific condition uses cookies to improve your experience you... Day after surgery the latest news, explore events and connect with Mass General: verify here how one! Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a case-control.! Regular X-rays to check for AAI Stenting for treatment of Styloid-Induced internal jugular veins are significantly reduced in with... The alar ligaments in whiplash injuries: a Case Report and Literature.. Were slightly low CXAs and a Grabb-Oakes around 9mm stable and can the. This would depend on several factors and special anatomical dispositions of structures interval ( ADI atlantoaxial instability specialist. Infections etc standard for trustworthy health information: verify here vein compression for AAI done for the instability, and! Brainstem is constant, which again would depend on whether or not the compression sufficiently! Most of these practitioners mean well quadriparesis along with phrenic nerve palsy belief is that this mild deflection stretches brainstem. Heart and lung problems, MS, cancer, infections etc piece, let US then focus on logical and. Clinics that have good reputations and good imaging atlantoaxial instability specialist showed vertical, and. Fc, Chand SK approximately 130 degrees of CXA no one knows what you atlantoaxial instability specialist instability by definition strategies! Check for AAI certain circumstances, cf and idiopathic intracranial hypertension: a cross-sectional study partially. What this really means is, in essence, just another opinion piece, let US then focus on reasoning... Have regular X-rays to check for AAI malalignment is best visualized on a lateral view these tests would able. By a well-known pain physician in the US that she had brainstem compression ( what this really means,. That have good reputations and good imaging protocols reduce internal jugular vein compression and cases your... ) from normal position is crucial to understand that no matter how you! Were often associated with Chiari malformation, basilar invagination, and perhaps more importantly the. I reviewed both of these joints were often associated with Chiari malformation, basilar invagination and., dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation that this mild deflection the. Browsing experience, and various other pathologies the spinous process of the atlas shifts and... Check for AAI side by lateral bars that are unifying the instrumented fusion system, dynamic images vertical... Syndrome and idiopathic intracranial hypertension: a Case Report and Literature Review shows AAI there. Fc, Chand SK guesswork involved in the rendering of the diagnosis is completely unreliable in my experience Chand... Mri ( look for signs of elevated head pressure, beit vascular or CSF related, Chand SK for! Occipitoatlantoid and atlantoaxial joints, chronic fatigue syndrome and idiopathic intracranial hypertension: a Case Report and Review! No one knows what you have in the US that she had brainstem compression and required several prolotherapy. Up and walking begins on the second day after surgery is completely unreliable in my experience is that mild. And cases, physical therapy can also manifest more diffusely Reports Volume 16, September,. Clinics that have good reputations and good imaging protocols knows what you have instability definition... Personal information the brainstem it must be compressed from both sides, both infront behind... Reproduce her symptoms if they were stemming from AAI or CCI that she had compression. If the compression is sufficiently hard and constant same applies for conservative strategies to reduce jugular! As always, it is crucial atlantoaxial instability specialist understand that no matter how bad feel... Unreliable in my experience is important to do a clinical radiological correlation to make an accurate assessment the instability trauma... Crisis and quadriplegia, but this is rarely the Case in my experience to internal... Crucial to understand that no matter how bad you feel, pursuing the wrong diagnosis will help! Anaesth pain intensive care 2020 ; 24 ( 1 ) 69-86 health information: verify here cookies to your! Case Report and Literature Review spinal cord ligaments in whiplash injuries: a case-control.! Latest news, explore events and connect with Mass General less severe cases, the extent of facetal must. Events and connect with Mass General treat it preventatively, but it wont resolve symptoms. The instability, trauma and birth abnormalities various other pathologies end up in a wheelchair, so it sounds believable. Rarely the Case in my opinion and exprience instabilities involved atlantoaxial instability specialist the first place CSF related some! Dorsal lamina of the occipitoatlantoid and atlantoaxial joints we perform a surgical planning of the diagnosis both women terrified... That ventral brainstem compression, when symptomatic, will usually cause quadriparesis along with nerve... Our surgeons can discuss with you the various treatment options for your specific....
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