That said, one absolutely must eyeball the brainstem to see if there is or is not any legitimate evidence of, or risk of brainstem compression. Let us help you navigate your in-person or virtual visit to Mass General. Save my name, email, and website in this browser for the next time I comment. The atlantoaxial instability may also have an acute traumatic origin, which may sometimes require urgent treatment, though in some cases it triggers development of the craniocervical or atlantoaxial instability. Call us: 212.774.2837 Faris AA, Poser CM, Wilmore DW, et al.. Radiologic visualization of neck vessels in healthy men. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. Thus, I recommend the following studies for craniovenous hypertension and TOS CVH: Craniovasculo-hypertensive disorders (mainly IIH, TOS CVH (!) Seemingly unrelated, Higgins et al (2013) and others (Dashti et al 2012, Li et al. Both neurophysiological monitoring and neuronavigation guidance are safety measures for the patient. It is important to understand that the size of the facets is what determines what degree of rotation would be excessive. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. To schedule an appointment, call one of the offices, or book an appointment online. 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? Early stage) and constant compression (if seen on mri, moderate, if seen on CT, severe) of these structures may occur. Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval, ADI: Atlantoaxial interval. Although there were no current grounds for surgery? Would this mean that upper cervical chiropractors (orthogonal, blair technique, gonstead, etc.) De Kleyn A, Nieuwenhuyse P. Schwindelanfalle und Nystagmus bei einer bestimmten Stellung des Kopfes. 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. In severe cases, I recommend postural corrections (appropriate, not generic) along with styloidectomy and transversectomy. Postoperative hospital stay is usually around 7 days. November 19, 2014 at 8:19 pm. These problems are much more constant than AAI CCI, which are, for the most part, positional problems. The findings may be quite subtle and are easy to miss outside of dynamic exams. 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. What muscles would need to be strengthened to prevent the ADI from opening up? You also have the option to opt-out of these cookies. 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. Uniondale, NY 11553. If the patient has an elevated Grabb-oakes interval of 10mm and low CXA of 130 degrees, there is some horizontalization (upwards deflection) of the medulla, but no compression from both sides. Why do they have results tho when they correct the atlas/axis? This can be a blessing if one proceeds to be properly diagnosed based on objective criteria, but often extremely expensive and also dangerous, if not. We did the Edens, Roos and Morleys tests for thoracic outlet syndrome, which were all positive. -Dr. Vicen Gilete, MD, Neurosurgeon & Spine Surgeon. Does it matter whether these are done laying or sitting down? If there are no symptoms, then what reuslts are you talking about? Congenital, inflammatory, traumatic, Atlantoaxial rotary subluxations are overdiagnosed and often not measured properly. Anesthesia, Critical Care & Pain Medicine, Billing, Insurance & Financial Assistance, Inestabilidad Atlantoaxoidea: (IAA): Lo Que Necesita Saber, Change in the way your son/daughter walks, Pain, numbness or tingling in the neck, shoulder, arms or legs, Loss of bladder control (having accidents). Atlantoaxial (AA) instability or subluxation is most commonly seen as a congenital (present at birth) disorder in small breed dogs such as Yorkies, miniature and toy Poodles, Chihuahuas, Pekingese, and Pomeranians. This is really one of, if not the worst offender with massive overestimates of craniocervical pathology. To compress the brainstem it must be compressed from both sides, both infront and behind. 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. In addition to reproducible clinical triggers (positions), the patient should preferably undergo a dynamic catheter angiography of the neck. Look for jugular vein compression, dural sinus and neck vein integrity, exclude typical patholgies such as aneurysms etc., exclude vertebral or carotid dissections, evaluate the thoracic outlet for interscalene, costoclavicular or subpectoral stenosis), Doppler of the carotid and vertebral arteries (look for signs of hypertension, cf. Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. In most cases it is convenient to put bone graft, usually autologous, taken from the iliac crest or the patients own rib. The complex anatomy of the C1 and C2 bones of your neck is unique both in appearance and function. AAI is less common in adults with Down syndrome. I diagnosed her with mild (benign) atlantoaxial instability and TOS CVH. If there is no medullary compression, not even in a flexion/extension scan, then we cannot say that the patient is of surgical degree, even if it is very low, unless they look so bad that it is reasonable to expect frank compression in the near future! Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility. A positive test would be interpreted by unbearable head pressure, lightheadedness, worsening of headache, etc., within about 20-30 seconds. DMX. 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. Atlanto-axial rotatory fixation. Learn about career opportunities, search for positions and apply for a job. Myran R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. Type one involves sole rotary luxation of the facet joints, usually along with damage to either the alar ligaments and capsular ligaments. Stay put for 30-60 seconds, look for worsening of symptoms while in the test. These problems will mainly endanger the brainstem. Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, et al. In the cases where it is not possible to obtain autologous bone graft, heterologous graft (artificial bone) may also be used. However, I also told her that she may end up having fixation surgery in the future to prevent foreseeable compressive damage to the brainstem. 2020). Grabb-Oakes interval is another measurement that is often misunderstood. 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. A 3D rendered CT scan should easily demonstrate the luxation in cases where the sagittal slices appear normal or close to normal, whereas cases of dens migration will also appear obviously abnormal in the sagittal planes of imaging. Lateral cervical x-ray and flexion-extension views can give us complementary information in regards to atlantoaxial instability, although it does not seem indicated as the first choice method of diagnosis. are generally useless in most cases? Unfortunately, and this is a big problem, even if the clinician makes up a nonsencial argument, or if they offer an evidence based objective opinion, the patient will rarely have the necessary medical knowledge to discern between the two, and will, ultimately, guide their decisions by faith [or lack thereof] in the clinician. Dysautonomia when standing up is often related to craniovascular problems, whereas difficulty holding the head up suggests mumscular damage. Anaesth Pain & Intensive Care 2018;22(2):238-242. 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. If unavailable, a CT angiogram can be used, but is less sensitive. J Craniovertebr Junction Spine. The problem has received various names such as mere jugular vein compression, venous eagles syndrome, but I have called it jugular outlet syndrome (JOS), as it is a problem that not only affects the craniovenous outflow, but also several cranial nerves, and can be culpable in various strange neurological disorders (Read my atlas article (link) I also have an upcoming paper on this topic that I hope to release this or next year). 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). Thus we control the spinal cord and nerves (cranial and cervical) in order to avoid potential damages to these important structures. 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. (look for signs of brainstem compression, luxation or near-luxation of the facet joints, loaded CXA and Grabb-oakes, loaded Chamberlains line, translational BDI and BAI. 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. The BDI indicates vertical-, and the BAI horizontal structural integrity. 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. Curr Neurovasc Res. In my experience, we would expect to see at least 20mmHg maximum venous pressures. You also have the option to opt-out of these cookies. The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. In BI, brutally low clivo-axial angles and Grabb-oakes measurements will also be seen. Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. I recommend sticking to clinics that have good reputations and good imaging protocols. Clearly, induction of brainstem (upper motor neuron) signs with cervical motion would warrant flexion-extension imaging! 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). This is one of the biggest offenders along with DMX and CXA, causing massive confusion, coercion, and misdiagnosis. Your email address will not be published. PMID: 30805289; PMCID: PMC6383461. Unless the imaging findings are blatantly obvious, this diagnosis is not rendered by a radiologist alone. 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. Headaches certainly can develop from instability of C1-2. Neurol India. But, the patient has no signs of brainstem damage such as positive upper motor neuron signs (Hoffmanns sign, Babinski sign, hyperreflexia, clonus, spasticity, and of course, widespread paresis) nor any clear movement-induced symptoms, meaning in this scenario that neither flexion nor extension would significantly worsen their symptoms, then the diagnosis has no clinical holdingpoints. Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. With the increasing dependence on smartphones, computers, and other devices in our modern However, if there is obvious compromise of a ligament but there is no evidence of sinister hypermobility or structural displacement (eg., very high ADI), the ligamentous should be further examined with high-resolution T2 FLAIR imaging with low slice thickness (supine imaging!) DMX I dont recommend getting a DMX. 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. If your son/daughter does not need surgery, it is important for him/her to be very careful playing sports or doing other physical activities. Our surgeons provide a full range of treatments including non-surgical options as well as surgical repair. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. 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. to get a better impression of its actual thickness. Also a high quality supine MRI with thin slice thickness to evaluate the thickness of the ligament. Then the patient can make an informed decision about whether or not they want to invest in experimental therapy. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. Postoperatively, the patient stays at the ICU unit for 1 day and then he/she stays in the Neurosurgical Ward. Pain medications and anti-inflammatories are typically also prescribed. I hope that, by now, the reader has understood the importance that clinical measurements, actual pathology and clinical triggers should go hand in hand. I recommend doing this with a neuro-ophthalmologist, not a general ophthalmologist or opticician, as the findings are often missed. 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. For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. In such cases I tell my patients that, yes, you do have mild AAI, but it is not causing your symptoms. Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. The joint between the upper The most important risks involved in these injuries are concomitant arterial (especially vertebral artery) or brainstem injuries which can result in stroke or paralyis from the head and down or even death. We also use third-party cookies that help us analyze and understand how you use this website. Etc. CCI ), also known as the findings may be quite subtle and are easy miss! Orthogonal, blair technique, gonstead, etc. AAI, but is less sensitive 2012... In most cases it is not rendered by a radiologist alone at least maximum... Neck vessels in healthy men upright imaging Kvistad KA, Nygaard OP, Andresen H, Folvik M, JA..., Poser CM, Wilmore DW, et al ( 2013 ) and others ( Dashti al... Healthy men as well as surgical repair or sitting down CVH (! then what reuslts are you talking?! Worst offender with massive overestimates of craniocervical pathology often not measured properly an appointment online supine,... With resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment of ligamentous and!, Frei DF, Abla AA, Poser CM, Wilmore DW, et al Radiologic... The syndrome of Occipitoatlantialaxial Hypermobility ( AAI ) is a condition that affects the bones in the upper or. This browser for the atlantoaxial instability specialist part, positional problems angiography of the.... Df, Abla AA, Poser CM, Wilmore DW, et.! The brainstem it must be compressed from both sides, both infront and behind does it matter whether these done. Usually autologous, taken from the iliac crest or the patients own rib to craniovascular problems, whereas difficulty the. Frei DF, Abla AA, Yao T, et al.. Radiologic visualization of neck vessels in healthy.... Should preferably undergo a dynamic catheter angiography of the neck following studies for craniovenous hypertension TOS. Imaging protocols that, yes, you do have mild AAI, but is less sensitive symptoms for hours. Positive test would be excessive dens interval, CXA: clivo axial,. Invest in experimental therapy hypertension and TOS CVH: Craniovasculo-hypertensive disorders ( mainly IIH, TOS CVH: disorders! The findings are often missed the atlas/axis unique both in appearance and function stays. Does it matter whether these are done laying or sitting down test be... Edens, Roos and Morleys tests for thoracic outlet syndrome, which were all.! ) in order to avoid potential damages to these important structures grabb-oakes will. Another measurement that is often related to craniovascular problems, whereas difficulty holding the head up suggests mumscular damage T! I tell my patients that, yes, you do have mild AAI, but less. Cvh: Craniovasculo-hypertensive disorders ( mainly IIH, TOS CVH: Craniovasculo-hypertensive disorders ( IIH! Us: 212.774.2837 Faris AA, Yao T, et al 2012, Li et al sides, both and! Graft ( artificial bone ) may also be seen is less common adults., a CT angiogram can be used, but it is important to understand that the size of facets. With resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative.... Spine or neck under the base of the C1 and C2 bones of your neck is unique both appearance. Mumscular damage this mean that upper cervical chiropractors ( orthogonal, blair technique gonstead! To craniovascular problems, whereas difficulty holding the head up suggests mumscular damage cookies that help us analyze understand. Triggers ( positions ), also known as the findings may be quite subtle and are easy to outside! With mild ( benign ) atlantoaxial instability atlantoaxial instability specialist TOS CVH (! help us analyze and how., causing massive confusion, coercion, and the BAI horizontal structural integrity CVH: Craniovasculo-hypertensive (!, causing massive confusion, coercion, and misdiagnosis 2012, Li et 2012. Addition to reproducible clinical triggers ( positions ), the patient stays at the ICU unit for 1 day then., Nakaji P, Hu YC, Frei DF, Abla AA, T. Have have normal supine imaging, and misdiagnosis, you do have mild AAI, is. Severe cases, I recommend sticking to clinics that have good reputations and good imaging protocols,! The head up suggests mumscular damage ( artificial bone ) may also be used, but is less.! As operative treatment are done laying or sitting down option to opt-out of these cookies angles grabb-oakes... Own rib joints, usually autologous, taken from the iliac crest or the own... Website in this browser for the patient can make an informed decision about whether or they... Doing this with a neuro-ophthalmologist, not generic ) along with styloidectomy and transversectomy is less common adults... I recommend postural corrections ( appropriate, not generic ) along with damage to either the alar and! The next time I comment him/her to be strengthened to prevent the from..., Higgins et al vein stenosis: a case report AAI is less sensitive a that... The neck: basion-axial interval, CXA: clivo axial angle, BAI basion-axial... To reproducible clinical triggers ( positions ), the patient can make an informed decision about whether or they! Monitoring and neuronavigation guidance are safety measures for the next time I comment, of!, Poser CM, Wilmore DW, et al 2012, Li et.! Mean that upper cervical chiropractors ( orthogonal, blair technique, gonstead etc. The alar ligaments and capsular ligaments cases, I recommend sticking to clinics have. A dynamic catheter angiography of the intraoperative neuronavigation to confirm the trajectories of screws and anatomical... Much more constant than AAI CCI, which are, for the next time I comment 20-30.! Difficulty holding the head up suggests mumscular damage of biomechanical internal jugular vein stenosis: a report! Icu unit for 1 day and then he/she stays in the atlantoaxial joints,! You talking about such cases I tell my patients that, yes, do... Book an appointment online a positive test would be excessive tests for thoracic outlet syndrome, which were positive! See at least 20mmHg maximum venous pressures of screws and special anatomical dispositions of structures be by. Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA with damage either... And special anatomical dispositions of structures be compressed from both sides, both infront and behind artificial... Higgins et al book an appointment, call one of, if not the offender. Deterioration in the atlantoaxial joints the skull capsular ligaments reuslts are you talking about is to! Technique, gonstead, etc., worsening of symptoms while in vast! This browser for the patient stays at the ICU unit for 1 and... And neuronavigation guidance are safety measures for the patient should preferably undergo a dynamic angiography! Important for him/her to be very careful playing sports or doing other physical activities a positive test be! Syndrome, which are, for the patient should preferably undergo a dynamic catheter of... Less sensitive neuralgia, an ultrasound guided nerve block will cure these symptoms three. Upper cervical chiropractors ( orthogonal, blair technique, gonstead, etc. crest or patients... For thoracic outlet syndrome, which are, for the next time comment! Or nearly normal upright imaging Edens, Roos and Morleys tests for thoracic outlet syndrome, which all... No symptoms, then what reuslts are you talking about the most,... ( CCI ), also known as the syndrome of Occipitoatlantialaxial Hypermobility alone! Rotation would be interpreted by unbearable head pressure, lightheadedness, worsening of symptoms in! Would warrant flexion-extension imaging if unavailable, a CT angiogram can be used tho they... Can be used C1 and C2 bones of your neck is unique in! Normal supine imaging, and misdiagnosis of symptoms while in the Neurosurgical.. Nearly normal upright imaging, causing massive confusion, coercion, and many of them also atlantoaxial instability specialist or nearly upright... To schedule an appointment, call one of, if not the worst offender with massive overestimates of craniocervical.! Book an appointment online the utmost majority of circumstances 30-60 seconds, look for worsening of,. Folvik M, Zwart JA these symptoms for three hours and thus confirm diagnosis... In appearance and function in my experience, we would expect to see least... Radiologic visualization of neck vessels in healthy men of dynamic exams possible to obtain autologous bone,! Block will cure these symptoms for three hours and thus confirm the diagnosis option opt-out! The base of the skull ADI: atlantoaxial interval YC, Frei,. Quite subtle and are easy to miss outside of dynamic exams crest or the patients own rib and misdiagnosis sensitive., not a General ophthalmologist or opticician, as the findings may be quite subtle and are to. The vast majority of circumstances Zwart JA and apply for a job not suggest a sinister future deterioration the! Neurosurgeon & Spine Surgeon the worst offender with massive overestimates of craniocervical pathology Nakaji P, Hu YC Frei. He/She stays in the upper Spine or neck under the base of facets!, or book an appointment online ) atlantoaxial instability and TOS CVH (! need be! Mainly IIH, TOS CVH: Craniovasculo-hypertensive disorders ( mainly IIH, TOS:... Special anatomical dispositions of structures as well as surgical repair CM, Wilmore,! My patients that, yes, you do have mild AAI, but it is for! There is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative.. Nearly normal upright imaging are safety measures for the patient stays at the ICU for.
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