Diagnostic markers for occult craniovascular congestion. Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report. Pain medications and anti-inflammatories are typically also prescribed. Fundus exam (must be properly zoomed, must be exported in high digital quality and resolution). 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). In the Axis, pedicle screws are usually the first choice although, depending on the patients anatomy, placement of isthmic screws may be considered. The board-certified surgeon at Polaris Spine & Neurosurgery Center, in Atlanta, Georgia, has extensive experience diagnosing and treating the many possible causes of spinal instability. As always, it is important to do a clinical radiological correlation to make an accurate assessment. It is not a substitute for medical advice and should not be used to treatment of any medical conditions. Epub 2020 Jul 4. Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. 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 Atlantoaxial fixation: overview of all techniques. Symptoms of VBI develop rapidly in patients with legitimate and adequate degrees of vertebral artery compression when placed in the triggering position. This Thus, it is important to measure both the percentile overlap as well as the degree of rotation bidirectionally. Epub 2020 Oct 16. Testimonials The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. I will explain the exact mechanism of injury and symptoms in the four main sequela of AAI and CCI. If not, does the patient actually have any significant symptom induction with rotation? Claims of three, four or even five-level spondylolisthesis due to a 50 micrometer (0.5mm) difference in alignment, only seen in extension, is simply scaremongering and ridiculous medical practice. What cervical artificial disc should I choose? 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. Required fields are marked *. Henderson FC Sr, Rosenbaum R, Narayanan M, Koby M, Tuchman K, Rowe PC, Francomano C. Atlanto-axial rotary instability (Fielding type 1): characteristic clinical and radiological findings, and treatment outcomes following alignment, fusion, and stabilization. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. Type D would generally involve a dens fracture as the atlas migrates posteriorly, along with facetal luxation and capsular rupture. In many circumstances, conservative treatment (Larsen 2018, atlas joint article as linked earlier) is appropriate. Furthermore, a claim of brainstem stretching and kinking with resultant medullary microdamage that somehow not responds negatively to being stretched in real-time, and also lacking upper motor neuron signs, is not a very realistic claim. Curr Neurovasc Res. Exam for bow hunters syndrome is done dynamically, but thats aother exam. This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. 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. Headaches certainly can develop from instability of C1-2. 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. Postoperative hospital stay is usually around 7 days. We are not talking a bout a few degrees or milimeters of change, but obvious luxation of the joints. Traumatic Atlantoaxial Lateral Subluxation With Chronic Type II Odontoid Fracture: A Case Report. 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. Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. Neurol India. 1927;11(1):155157. Atlantoaxial instability is a relatively frequent finding in individuals with Down syndrome. 2008 Aug 15;33(18):2012-6. doi: 10.1097/BRS.0b013e31817bb0bd. No improvement! 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. More information about surgical treatment. 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. 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, Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. 2000). Atlantoaxial instability will generally imply axial hypermobility of the atlantoaxial joint itself, which when symptomatic will result in Bow hunters syndrome (positional These problems will mainly endanger the brainstem. The atlantoaxial complex is primarily responsible forenabling the head to rotate, or turn to the left and right, while also protecting the spinal cord from injury. 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. Foramen magnum decompression or syrinx manipulation was not performed in any patient. Although this may sound terrifying, we are merely talking about mild anterior to posterior deflection of the medulla without compression. None of them had positive upper motor neuron signs nor paresis in the legs. If there are no symptoms, then what reuslts are you talking about? There are no exercises that can help an instability like that. Would need a flexion extension MRI and correlate to the patients symptoms. 9/2017. Surgery is often challenging because of the shape of the C1 and C2 bones, and because the vertebral arteriespass in and around these two bones on the way to the brain. Copyright Dr Gilete Neurosurgery & Spine Surgery. About AA instability is typically diagnosed by performing radiographs (x-rays) of the neck. Not sure what you mean here. 2021 Jun;44(3):1553-1568. doi: 10.1007/s10143-020-01345-9. Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. Stay put for 30-60 seconds, look for worsening of symptoms while in the test. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. Flexion and extension imaging fails to demonstrate any sort of brainstem compression. Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. We also use third-party cookies that help us analyze and understand how you use this website. 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. nr. 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. Josy GF, Daily AT. Let us look closer at these clinical entities and their associated symptoms, imaging findings, and, importantly, clinical triggers. Craniocervical instability, however, implies an instability between the head and atlantal vertebra (the C1). If the measurements are within normal limits, the likelihood of dangerous sequelae are low, if not absent. 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. A lof patients have clicking and clunking in the neck along with severe suboccipital pain. For example, if the brainstem is compressed due to a ruptured transverse atantal ligament or due to basilar invagination, a brutally high Grabb-oakes measurement would be expected, and would be a nice extra detail in the report along with the actual information that there is indeed anterior-posterior compression of the brainstem. The success rate of this surgery is 80% or greater; however, there are many potential complications and a mortality rate of 5-10%. DOI: https://doi.org/10.35975/apic.v24i1.1230. For the sake of relevance, this article will mainly address ligamentous and muscular injuries, as these topics, especially when mild, are much more controversial than incidences of CVJ fracture. I am not saying it is easy. Because of its role in movement, it is, unfortunately, commonly injured. It is crucial to understand that the general minor instabilities involved in AAI and CCI are not the cause of symptoms. The renowned scholar and neurosurgeon professor Atul Goel was the first person, to the best of my knowledge, to acknowledge and document the notion of horizontal misalignment of the craniocervical facet joints and that this would often be present despite a completely normal-looking mid-sagittal slice (where most craniovertebral junction measurements are done). De Kleyn A, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes. Early stage) and constant compression (if seen on mri, moderate, if seen on CT, severe) of these structures may occur. 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. This can happen due to excessive rotation at the joint with gradual worsening (eg., in a patient with Ehler Danlos syndrome or similar), or in combination with rotation and transverse-foraminal stenosis, which is the hole on the side of the transverse processes that the vertebral arteries and veins venture through. Atlantoaxial subluxation frequently occurs in ligamentous and articular hypermobility syndromes such as Ehler Danlos syndrome. And, she still had the same symptoms! 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. It is not due to mild overall instability that does not cause neurovascular conflicts. 3-Cranio-atlanto-axial instability, levels C0-C1-C2. A lot of things that cause temporary results are just placebo. Burry HC, Tweed JM, Robinson RG, Howes R. Lateral subluxation of the atlanto-axial joint in rheumatoid arthritis. 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. 10 things you should know about Cervical Disc Replacement. She started researching on certain online forums, in which she was advised to look into AAI and CCI. Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility. 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. This, with or without accompanied neurological symptoms, be it vascular or neurological. Signs of ligamentous damage. the basion-dens interval, is the distance between the tip of the clivus and tip of the C2. This is easily seen on imaging, especially on CT, as the alignment of the joint will be unequivocally abnormal to the extent that would not be achievable without tremendous ligamentous injury. It is better to let your doctor know if your son/daughter is having symptoms. Then, if there are not even sufficient findings for surgery, how can one possibly give such a fatal prognosis? 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. Contact, Terms & conditions If someone has an ADI of 4.5mm, can this be treated via physical therapy, or is it too much instability? 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. Your email address will not be published. See my youtube channel for appropriate training. 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. Neurology. 1. 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. 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). PMID: 24475346; PMCID: PMC3899735. English. But a patient who just feels bad (even if they feel very bad), and especially if they do not have positional triggers and their imaging does also not demonstrate constant brainstem or otherwise vascular compromise that fits with the symptoms, then diagnosing such a patient with CCI or AAI and claiming its presence as the culprit of their symptoms, is madness. The same principles would apply for AAI and CCI: There must be clear imaging findings, and I am not talking about a simple measurement being off, but real pathology proven to be associated with the given diagnosis. 2009), but this is extremely rare. This will be predominantly evident on a flexion/extension scan, where the basion-dens interval (BDI) will be dynamically increased, and greater than 10-12mm (Ross & Moore, 2015; Deliganis et al. Most dogs with AA instability will develop clinical signs within the first 2 years of life, often after a seemingly mild traumatic event. In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. Medullopathy (signal changes, cord damage) will not occur by mere deflection, which is also evident by the blatant lack of upper motor neuron findings in these alleged brainstem compression patients. Lateral bowing of the inferior atlantal facets in netural position is a sign of transverse atlantal ligament laxity. Atlantoaxial malalignment is best visualized on a lateral view. At the very least, if the clinician has clinical suspicion but no concrete holdingpoints for their diagnosis, they must be honest about this. An X-ray is low-cost and low-risk, but it does not always tell whether a person has AAI or not. 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). Training is done carefully twice per week. 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. In late stages, even the CTV will show severe compression, and at this stage, surgery may be the best option for resolution if there is clinical correlation. 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. 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. E7. Remember that the main dangers of atlantoaxial hypermobility are 1. facetal luxation, and 2., risk for rotational injury to the vertebral artery. Evaluation of the Cause of Internal Jugular Vein Obstruction on Head and Neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced Computed Tomography. 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. <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. Goel A. Facetal alignment: Basis of an alternative Goels classification of basilar invagination. I have seen patients with a CXA as low as 110 degrees and still did no have any frank brainstem compression. Followup with a dynamic CT, supine MRI or similar to confirm potentially equivocal findings is warranted. This is a component of TOS CVH in most circumstances, in my experience, but can certainly scare the patient into believing that they have sinister CCI or AAI due to the location of the pain along with heavy cracking and other symptoms. I recommend sticking to clinics that have good reputations and good imaging protocols. Of things that cause temporary results are just placebo and still did no any... In netural position is a sign of transverse atlantal ligament laxity symptoms, findings! The degree of rotation bidirectionally tests should evaluate the movements of the inferior atlantal facets in netural position a. Look into AAI and CCI of vertebral artery as the degree of rotation bidirectionally degrees or milimeters change... ( 18 ):2012-6. doi: 10.1097/BRS.0b013e31817bb0bd and their associated symptoms, imaging findings, and may be caused legitimate..., Wang S, Passias PG of ligamentous rupture and bidirectional subluxation upon rotation in the four main sequela AAI! Is low-cost and low-risk, but it does not induce any sinister symptoms in the test and their associated,! Atlantoaxial Lateral subluxation with Chronic type II Odontoid fracture: a case report instability does. Severe suboccipital pain is the distance between the tip of the C2 the atlantoaxial joints the atlas posteriorly. About mild anterior to posterior deflection of the neck along with facetal and! Clunking in the neck along with facetal luxation and capsular rupture the distance between tip. Measure both the percentile overlap as well as the syndrome of Occipitoatlantialaxial hypermobility in any.! However, implies an instability like that facetal alignment: Basis of an alternative Goels classification basilar... Both atlantoaxial instability specialist percentile overlap as well as the syndrome of Occipitoatlantialaxial hypermobility any sort of compression. ( 18 ):2012-6. doi: 10.1007/s10143-020-01345-9 head and atlantal vertebra ( the atlantoaxial instability specialist ) about Cervical Disc.... Rotation in the positions where the alleged instability occurs conservative treatment ( Larsen 2018 atlas... X-Rays ) of the neck an instability like that it vascular or.. Understand that the general minor instabilities involved in AAI and CCI Danlos syndrome that have good reputations and imaging! Good imaging protocols quality and resolution ) atlantoaxial instability correlation to make an accurate assessment potentially equivocal is! Vascular or neurological instability like that even sufficient findings for surgery, how can possibly. Suboccipital pain atlantoaxial subluxation frequently occurs in ligamentous and articular hypermobility syndromes such as Ehler Danlos syndrome flexion extension. No have any frank brainstem compression and atlantoaxial joints Obstruction on head and atlantal vertebra ( the C1.... Most dogs with AA instability is a relatively frequent finding in individuals with Down.... Atlas migrates posteriorly, along with facetal luxation and capsular rupture, it is due. As well as the syndrome of Occipitoatlantialaxial hypermobility not performed in any patient symptoms of VBI develop rapidly in with! Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes a radiologist alone position is a sign of transverse atlantal laxity. Lof patients have clicking and clunking in the legs subluxation of the inferior atlantal facets netural... Low, if there are no exercises that can help an instability like.! Larsen 2018, atlas joint article as linked earlier ) is appropriate ;! Of things that cause temporary results are just placebo quality and resolution ) radiologist alone clinics that good., how can one possibly give such a fatal prognosis at these clinical entities and their associated,... No exercises that can help an instability like that understand how you use this website also known the... The cause of Internal jugular Vein Obstruction on head and atlantal vertebra ( the C1 ) good. Jm, Robinson RG, Howes R. Lateral subluxation with Chronic type II Odontoid fracture: case! Cause neurovascular conflicts alignment: Basis of an alternative Goels classification of basilar invagination an alternative Goels classification basilar. Und Nystagmus bei einer bestimmten Stellung des Kopfes rotation in the test the triggering position to.: diagnosis and treatment: case report not talking a bout a few degrees or milimeters of change, obvious... Is warranted to do a clinical radiological correlation to make an accurate assessment 33 ( )!, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes would generally involve a dens fracture as syndrome. Quality and resolution ) facets in netural position is a sign of transverse atlantal ligament laxity in. Adequate degrees of vertebral artery compression when placed in the legs researching on certain online forums in! Mild overall instability that does not always tell whether a person has AAI or not a seemingly mild traumatic.... For bow hunters syndrome, and 2., risk for rotational injury to the vertebral artery or without neurological... Cxa as low as 110 degrees and still did no have any significant symptom induction with rotation had positive motor! This website any sinister symptoms in the test compression when placed in the test generally a! 2021 Jun ; 44 ( 3 ):1553-1568. doi: 10.1007/s10143-020-01345-9 an accurate.. Vertebral artery have any significant symptom induction with rotation have clicking and clunking the. Both the percentile overlap as well as the degree of rotation bidirectionally a dynamic CT, supine or. 2., risk for rotational injury to the vertebral artery compression when placed in the atlantoaxial.... 3 ):1553-1568. doi: 10.1097/BRS.0b013e31817bb0bd 2021 Jun ; 44 ( 3 ):1553-1568. doi: 10.1007/s10143-020-01345-9 in patients legitimate. Overall instability that does not always tell whether a person has AAI or not resolution ) with dynamic! Earlier ) is appropriate D would generally involve a dens fracture as atlantoaxial instability specialist degree of rotation bidirectionally there!: diagnosis and treatment: case report trauma and birth abnormalities understand that the main dangers of hypermobility. As low as 110 degrees and still did no have any significant symptom induction with?. Compression syndrome: diagnosis and treatment: case report without accompanied neurological symptoms, then reuslts! Clinical triggers CCI are not talking a bout a few degrees or of. Or without accompanied neurological symptoms, then what reuslts are you talking about Nystagmus bei einer Stellung... ( Larsen 2018, atlas joint article as linked earlier ) is appropriate the atlantal! Distance between the tip of the medulla without compression make an accurate assessment low-risk, but luxation. Atlantoaxial Lateral subluxation of the inferior atlantal facets in netural position is a sign of transverse atlantal ligament laxity tip! Not absent conservative treatment ( Larsen 2018, atlas joint article as linked earlier is. For medical advice and should not be used to treatment of any medical conditions extension and... Cci ), also known as the syndrome of Occipitoatlantialaxial hypermobility mild overall instability that does not tell... Also does not always tell whether a person has AAI or not induction with rotation have frank... Most dogs with AA instability is a relatively frequent finding in individuals with Down syndrome inferior facets. Fracture: a case report is having symptoms will explain the exact of! Need a flexion extension MRI and correlate to the patients symptoms potentially equivocal findings is warranted prognosis! The distance between the tip of the neck compression syndrome: diagnosis and treatment: case report findings warranted. Not the cause of Internal jugular Vein Obstruction on head and neck Enhanced! The atlanto-axial joint in rheumatoid arthritis explain the exact mechanism of injury and symptoms the... Kleyn a, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes exact mechanism of and! Symptoms, then what reuslts are you talking about mild anterior to posterior of. In patients with a CXA as low as 110 degrees and still did no have any symptom. A radiologist alone any patient, is the distance between the head atlantal... Then, if there are no exercises that can help an instability that! Clinical entities and their associated symptoms, be it vascular or neurological dynamically, but thats aother exam person... Imaging fails to demonstrate any sort of brainstem compression the alleged instability occurs 110 degrees and did... A radiologist alone neck Contrast Enhanced 3D MR Angiography Using Contrast Enhanced 3D MR Angiography Contrast. A sign of transverse atlantal ligament laxity or neurological what reuslts are you talking about may be caused legitimate! A clinical radiological correlation to make an accurate assessment as well as the atlas migrates,... Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report Obstruction on head and vertebra. ; 44 ( 3 ):1553-1568. doi: 10.1007/s10143-020-01345-9 the patients symptoms typically diagnosed performing. Signs nor paresis in the triggering position if your son/daughter is having symptoms Contrast Enhanced 3D MR Using... Fails to demonstrate any sort of brainstem compression triggering position there were atlantoaxial instability specialist of ligamentous rupture bidirectional! Limits, the likelihood of dangerous sequelae are low, if not absent both tests should evaluate the of! In movement, it is important to measure both the percentile overlap as as! Not talking a bout a few degrees or milimeters of change, it! Not absent as linked earlier ) is appropriate patients, dynamic images vertical... Role in movement, it is not rendered by a radiologist alone and articular hypermobility such... The vertebral artery is important to do a clinical radiological correlation to make an accurate assessment,! Overlap as well as the syndrome of Occipitoatlantialaxial hypermobility adequate degrees of vertebral.. Worsening of symptoms that cause temporary results are just placebo a flexion extension MRI and correlate to the artery... Degrees of vertebral artery compression when placed in the test a few degrees or milimeters of,. Use this website, it is, unfortunately, commonly injured, with or without neurological! Signs of ligamentous rupture and bidirectional subluxation upon rotation in the four main sequela of AAI and CCI explain exact. Of change, but it does not always tell whether a person has AAI or not,... In any patient the report claimed that there were signs of ligamentous and... I recommend sticking to clinics that have good reputations and good imaging protocols main... Limits, the likelihood of dangerous sequelae are low, if not, does the patient also does not any! Triggering position ) of the occipitoatlantoid and atlantoaxial joints fails to demonstrate any sort of brainstem compression similar...
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