Several studies from the region have focused on both the clincoradiological presentations and surgical nuances of the four commonly encountered cvj anomalies, namely, reducible raad and irreducible atlantoaxial dislocation iraad, basilar invagination bi, and chiari i. Congenital, developmental, and acquired bony abnormalities, result in compression and distortion of the neural structures, the vertebrobasllar vascular. The craniovertebral or craniocervical junction cvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. A fissure in each sclerotome separate a denser caudal half from loosely arranged cranial half. It encloses the soft tissue structures of the cervicomedullary junction medulla, spinal cord, and. Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that. Anaesthesia management in craniovertebral junctional anomalies. Despite being commonly imaged, the intricacy of the anatomical structures in this region poses challenges to interpreting the imaging findings. Developmental abnormalities of the craniocervical junction. It is divided into anomalous pancreaticobiliary junction with biliary dilatation 77% and without biliary dilatation 23% 7. Craniovertebral junction anomalies in inherited disorders. In this chapter, we focus on illustrating the lesions involving the craniovertebral junction.
Traumatic injuries of the craniovertebral junction cvj area are common and frequently the outcome of motor vehicle accidents, falls, and diving accidents. Massimiliano visocchi institute of neurosurgery, catholic university, largo gemelli, 8 00168 rome, italy introduction in the modern surgical approach to the craniovertebral junction cvj, past and. The 63rd world health assembly in 2010 adopted a resolution on congenital anomalies, to encourage countries to build incountry capacity related to the development of congenital anomalies surveillance systems and the prevention of congenital anomalies, and to raise awareness about their impact. In one series they affect children and young adults primarily. Whilst successful surgery may produce remarkable clinical results and may be compatible with good and new life, any complication can be devastating for the patient and for the family.
Mar 18, 2010 an interesting case of quadriparesis prof. The fate of a series of 63 patients operated on between 1953 and 1979 was studied retrospectively to assess the efficacy of neurosurgical treatment in relieving symptoms associated with craniovertebral junction cvj malformations. Similarly, the natural course of craniocervical junction anomalies, in growing patients with oi, has for the most part been unexplored despite the importance of early intervention before serious symptoms arise. This includes theoccipitoatlantalandatlantoaxial articulations. Craniovertebral junction anomalies knowledge for medical. We use mri whenever a neurologic deficit is present and to assess the extent of ligamentous involvement of craniovertebral junction injuries. The craniovertebral junction cvj is a complex articulation comprised of the clivus, foramen magnum and upper two cervical vertebrae. Improved imaging, understanding of anatomical subtleties and the possibility of evaluation of the region with advanced computer based imaging hallmarked a therapeutic revolution in the subject. Posterior fossa dorsal decompression pfdd is typically performed but has adverse results when ventral bone abnormality. Craniovertebral junction surgery is complex, is anatomical, is biomechanical, is philosophical and needs highest degree of technical expertise and experience. A 28yearold unmarried woman presented with a fivemonth history of unsteady gait, which had worsened one month before admission. Congenital vertebral anomalies may be associated with other birth defects, and awareness of these associations is important. Without abnormalities of the subaxial spine, an occ2 or c12 fixation can be sufficient.
Anomalies of the cvj may be congenital or acquired. The neural arch ossification centers form during the seventh gestational week, whereas the ossification center within the body of c1 becomes visible during the first year of life. Also, mild flattening of the right parietal lobe sulci and gyri. If you continue browsing the site, you agree to the use of cookies on this website. Fusion of the atlas and the axis is probably the most rare cervical anomaly. Any information contained in this pdf file is automatically generated from digital material. We have a low threshold for obtaining mris of the cervical spine in obtunded children with mechanisms of injury that are high risk for injury to the craniovertebral junction. It is the potential site of a variety of radiologic diagnoses and misdiagnoses. They include congenital anomalies, fractures of special type, degenerative disorders, infectiousinflammatory diseases, tumor and. Vertebral artery anomalies at the craniovertebral junction i. Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Imaging of the craniovertebral junction radiology key. Despite the volumes of publications on the subject, it appears that the last word has not yet been said.
The craniovertebral junction cvj comprises the occiput, atlas, and axis and is visible in most magnetic resonance mr imaging studies of the brain. Fortytwo cases of the series were major abnormalities formed by the combination. The inclusion criteria included failed primary procedure, repeat procedure for construct failure, infection at the surgical site, or wound dehiscence. Congenital vertebral anomalies douglas brockmeyer congenital vertebral anomalies are relatively common disorders, ranging from simple, asymptomatic block vertebrae to complex combinations of anomalies involving multiple vertebral levels. The craniocervical junction ccj is a collective term used to describe the articulation of the skull and the cervical spine. Cvj anomalies that decrease the volume of the posterior cranial fossa e. The first type of fusion in caves classification is that of a separated odontoid process with the anterior atlantal arch. The craniovertebral junction cvj is composed of the occiput, the foramen magnum, and the first two cervical vertebrae, encompassing the medulla oblongata and the upper cervical spinal cord. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardiovascular manifestations poses a high anaesthetic risk to these patients.
Craniocervical junction anomalies in children nicholas m. What does craniocervical junction is grossly unremarkable. To manage patients with congenital vertebral anomalies effectively, a firm grasp of the relevant embryology, classification, natural history, clinical evaluation, and treatment principles is essential. Ct anatomy of the craniovertebral junction in infants and. Anomalous vertebral artery in craniovertebral junction with occipitalization of the atlas article in spine 3426. A 10yearold japanese boy with a congenital anomaly in the craniovertebral junction presented with. Craniovertebral junction cvj anomalies are wellrecognized causes of myelopathy in the indian subcontinent.
Anomalies of the craniovertebral junction a very rare. Ch neurosurgery degree examination in august 2014 is a original bonafide record of. Mri has totally changed the prospects of investigation of spinal lesions and more so craniovdrtebral cv junction. Craniocervical junction 209 craniocervical junctions in 35 abnormal and 10 normal subjects were studied with a 0. Craniocervical junction disorders are abnormalities of the bones that join the head and neck. Craniovertebral junction especially with bony anomalies like basilar invagination bi and atlantoaxial dislocation aad have often been. After a brief introduction to anatomy, biomedical considerations, and embryology, the multidisciplinary team of authors, led by professor goel, discusses in detail the most innovative fixation and stabilization techniques for atlantoaxial dislocation, as well as congenital,infective, and acquired anomalies in the craniovertebral junction region. The connection between the brain and the spinal cord is at the base of the brainstem in the region of the craniocervical junction.
A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. The first anatomic description of manifestations of occipital vertebrae was attributed to meckel in 1815 by gladstone and ericksonpowell. The imaging characteristics and differential diagnoses of these lesions are provided. You may not embed one of our images on your web page without a link back to our site. Abstract the craniovertebral junction is formed by the occipital bone, atlas, and axis. This chapter covers congential vertebral anomalies from the cervical spine to. Craniocervical junction abnormalities neurologic disorders. The journal is registered with the following abstracting partners. Certificate this is to certify that this dissertation entitled a comprehensive study on craniovertebral junction anomalies submitted by dr. The subject of craniovertebral junction anomalies is under discussion and evaluation for over a century. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown. Pure cm patients without bony anomalies were excluded from the study. At 12 years if fuses with odontoid to form normal odontoid.
To evaluate the prevalence of anatomical variations of the vertebral artery at the craniovertebral junction and the posterior arch of the atlas in the us population. Cv junction anomalies free download as powerpoint presentation. Craniovertebral joints definition of craniovertebral joints. Pediatric bony craniovertebral junction abnormalities. However, ct and mr imaging may provide a more precise assessment, particularly in complex cases. The term basilar invagination was coined before the advent of the computed tomographic ct scan and magnetic resonance imaging mri, after assessing various craniometric lines and angles on plain radiographs or polytomograms. The anomalous pancreaticobiliary junction makes biliary drainage not under the control of sphincter of oddi, resulting in pancreatic juice. Craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago. Plate and screw fixation for atlantoaxial dislocation. These disorders may be present at birth or result from injuries or disorders that occur later. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle, and atlantooccipital joint axis angle. Changes in cranial base and craniocervical junction during. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydroc. Fortytwo cases of the series were major abnormalities formed by.
Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiputp. Pediatric craniovertebral junction anomalies can be managed successfully with. Craniovertebral junction abnormalities with hindbrain. The craniovertebral junction cvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Craniovertebral junction abnormalities thieme connect. Craniocervical junction anomalies in children mayo clinic. Any information contained in this pdf file is automatically generated from digital. Mar 14, 20 what does craniocervical junction is grossly unremarkable mean. Anomalies of the craniovertebral junction a very rare case. Neoplasms that arise within the cvj consist of osseous tumors, nervous system and those extending from surrounding soft tissue.
It is a complex structure and consists of osseous, soft tissue, and nervous system. The authors view based on their own clinical and radiological experience is that the osodontoideum is the product of excessive movement at the time of ossification of the. The onset of this symptom occurred without any obvious cause. Sagittal spin echo with 30 msec echo times and 500 msec repetition times constituted the most informative imaging plane and sequence. Recent studies from asia have reported a 5% to 10% pre.
Anomalous vertebral artery in craniovertebral junction with. Craniovertebral junction anomalies knowledge for medical students and physicians. Tumors and tumorlike lesions of the craniovertebral junction. This is a complex region where the skull and upper cervical spine connect.
Case report open access syncope caused by congenital anomaly. Problems in instrumentation of syndromic craniovertebral junction. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Syncope caused by congenital anomaly at the craniovertebral. The anomalies were classified according to their embryonic origin. Craniovertebral junction cvj anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. Surgery is often indicated to prevent or treat neurological symptoms. Fusion of a nonseparated dens is an even rarer anomaly. The craniovertebral junction cvj is composed of the occiput, the foramen magnum, and the first two cervical vertebrae, encompassing the medulla oblongata. Indian perspective basilar invagination, chiari malformation. Threedimensionalprinted modelassisted management of. Craniovertebral junctional cvj anomalies are developmental disorders that affect the skeleton and enclosed neuraxis at the junction of cranium and cervical spine. Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease.
Chiari i malformation with and without basilar invagination. Anatomical variations and congenital anomalies of the lumbar. In all other cases, there were associated anomalies. It encompasses and protects the medulla oblongata and the upper cervical spinal cord. Experimental studies have confirmed the biomechanical safety of. The craniovertebral junction cvj consists of the occiput and the first two cervical vertebrae, and functions as an articulation point capable of complex motions distinct from the remainder of the spinal column. They can occur anywhere from the craniovertebral junction to the coccyx.
In children with syndromic cvj anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Also, bright signal intensity along the bilateral convexity subarachnoid spaces and sulci along with findings likely result from t2 shinethrough artifact mean. Biomechanics of the craniovertebral junction, biomechanics in applications, vaclav klika, intechopen, doi. It has been frequently reported as fusion of an os odontoideum or a fractured odontoid process. Congenital, developmental, and acquired bony abnormalities, result in compression and distortion of the neural structures, the vertebrobasllar vascular system and the cerebrospinal fluid channels. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. A total of 7 procedures were performed in 55414 % patients. Help us write another book on this subject and reach those readers. The arch of the atlas, the odontoid and part of the axis craniovertsbral be excised.
It encloses the soft tissue structures of the cervicomedullary junction medulla, spinal cord, and lower cranial nerves. Most often, people have neck pain and headache, but if the spinal cord or lowest part of the brain brain stem is affected, people may have difficulty sensing vibration, pain and temperature and may have. Below are the links to the authors original submitted files for images. Biomechanics of the craniovertebral junction intechopen. The craniovertebral junction is the most complex of the axial skeleton, residing between the skull and the upper cervical spine. We present an extremely rare case with anomaly in the craniovertebral junction and syncope, and discuss the mechanism of the syncope. Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease the space for the lower brain stem and cervical cord. A bizarre developmental anomaly of the occipital bone and upper cranioverttebral spine with striking and misleading neurologic manifestations. Congenital malformations of the craniovertebral junction.
Association of craniovertebral junction anomalies, case. Cv junction anomalies human anatomy human head and neck. Recognizing findings of congenital anomalies and normal variants of the biliary tract at mrcp, mri, and helical ct, and knowledge of the clinical significance of each entity, are important for establishing a correct diagnosis and in guiding appropriate clinical management. The synchondroses may appear solid at softtissue window settings, while, with appropriate bonewindow settings, a lucent line will be seen. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. Craniovertebral junction cvj abnormalities, such as atlantoaxial dislocation aad with or without basilar invagination bi, with. Hindbrain herniation syndrome, or chiari malformation type i cmi, occurs frequently with craniovertebral junction cvj abnormalities when there is reduction in the posterior fossa volume. Scribd is the worlds largest social reading and publishing site. Anomalies in the craniovertebral junction may be a rare cause of syncope. All these craniometric lines showed the relationship of the cranium with the axis c2 body as well as the odontoid process and the relationship between c1 and c2. To define and characterize cvj traumatic injuries, some international classifications are currently in use, and they are thought and focused on junction bone fracture. Craniovertebral junction anomalies radiology reference article. Craniovertebral junction anomalies radiology reference.
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