Craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago. Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease. The craniovertebral junction cvj has attracted more attention in pediatric medicine in recent years due to the progress in surgical technologies. The craniovertebral junction cvj is composed of the occiput, the foramen magnumand the first two cervical vertebrae, encompassing the medulla oblongata and the upper cervical spinal cord. Craniocervical junction anomalies in children nicholas m. 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. Anomalies in the craniovertebral junction may be a rare cause of syncope. Unilateral fixation for treatment of occipitocervical instability in children with congenital vertebral anomalies of the craniocervical junction. Factors considered were reducibility, mode of encroachment, cerebrospinal fluid csf dynamics and syringohydromyelia. Although these are developmental anomalies, they manifest late in life, with trauma andor infection playing a promotive role. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydroc.
Imaging of the craniovertebral junction anomalies in. In children with syndromic cvj anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Craniovertebral junction surgery is complex, is anatomical, is biomechanical, is philosophical and needs highest degree of technical expertise and experience. 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 composed of the occiput, the foramen magnum, and the first two cervical vertebrae, encompassing the medulla oblongata and the upper cervical spinal cord. Craniovertebral junction abnormalities in down syndrome. If you continue browsing the site, you agree to the use of cookies on this website. 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. It encloses the soft tissue structures of the cervicomedullary junction medulla, spinal cord, and lower cranial nerves. Craniovertebral junction abnormalities thieme connect. Dc accompanied by craniovertebral junction cvj anomalies, kfs and mirror movement. Congenital, developmental, and acquired bony abnormalities, result in compression and distortion of the neural structures, the vertebrobasllar vascular. Association of craniovertebral junction anomalies, case.
Other readers will always be interested in your opinion of the books youve read. Whether youve loved the book or not, if you give your honest and detailed thoughts then people will find new books that are right for them. Enter your mobile number or email address below and well send you a link to download the free kindle app. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown. The craniovertebral junction cvj has attracted more attention in pediatric medicine in recent years due to the progress in surgical technologies allowing a direct approach to the cvj in children. A 10yearold japanese boy with a congenital anomaly in the. A cephalometric method to diagnosis the craniovertebral junction abnormalities in osteogenesis imperfecta patients. Vertebral artery anomalies at the craniovertebral junction in the us population. 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.
There are three main issues in dealing with congenital craniovertebral junction cvj anomalies, namely, arnold chiari malformation. Craniovertebral junction cvj anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. The craniovertebral junction cvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Craniovertebral junction the craniovertebral or craniocervical junction cvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Neuroimaging in craniovertebral anomalies as seen in the. Pdf access policy full text access is free in html pages. Congenital, developmental, and acquired bony abnormalities, result in compression and distortion of the neural structures, the vertebrobasllar vascular system and the cerebrospinal fluid channels. Diagnosis and management craniovertebral junction anomalies. Key words basilar invagination, craniovertebral junction, decruitment, transverse ligament. Then you can start reading kindle books on your smartphone, tablet, or computer no kindle.
Anomalies of the cvj may be congenital or acquired. Cvj anomalies that decrease the volume of the posterior cranial fossa e. The imaging characteristics and differential diagnoses of these lesions are provided. The craniovertebral junction cvj is the bony transition between the cranium and cervical spine. Neurosurgery surgery of the craniovertebral junction. Aai in the ds population may be secondary to bony anomalies such as os odontoideum.
Imaging of the craniovertebral junction anomalies in children article in advances and technical standards in neurosurgery 40. Congenital, developmental, and acquired bony abnormalities, result in compression and distortion of the. Craniovertebral junction anomalies knowledge for medical students and physicians. Tumors of the craniovertebral junction, whether extracranial with secondary involvement of the intracranial and intraspinal structures or primarily intracranial with secondary extension into the spinal canal, have characteristics that reflect compression of neighboring structures or traction.
The most significant and common of these anomalies are basilar. The craniovertebral junction cvj comprises the occiput, atlas, and axis and is visible in most magnetic resonance mr imaging studies of the brain. Congenital malformations of the craniovertebral junction. We further discuss current screening recommendations. Biomechanics of the craniovertebral junction intechopen. Fortytwo cases of the series were major abnormalities formed by.
Pdf a cephalometric method to diagnosis the craniovertebral. 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. The craniovertebral junction is the most complex of the axial skeleton, residing between the skull and the upper cervical spine. Abnormalities of the craniovertebral junction in the paediatric. Pdf congenital craniocervical junction anomalies incidentally.
Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. In contrast, in the orient, a greater incidence of isolated cvj anomalies is seen. Craniovertebral junction anomalies in inherited disorders. Embryology and bony malformations of the craniovertebral. Craniovertebral junction cvj disorders in the occident are usually associated with systemic disorders. Surgery of the craniovertebral junction, second edition is the surgical resource every spine and skull base surgeon should have on their bookshelf. There are three main issues in dealing with congenital craniovertebral junction cvj anomalies, namely, arnold chiari malformation acm, basilar invagination bi, and atlantoaxial dislocation aad. Problems in instrumentation of syndromic craniovertebral junction. We present an extremely rare case with anomaly in the craniovertebral junction and syncope, and discuss the mechanism of the syncope. Craniovertebral junction will be immensely beneficial to spine surgeons, neurosurgeons, orthopaedic surgeons, and spine fellows. These may occur in isolation or in any combination, i. Craniovertebral junction anomalies knowledge for medical. The journal is registered with the following abstracting partners. Diagnosis and management of anomalies of the craniovertebral.
Craniocervical junction anomalies in children mayo clinic. 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. Help us write another book on this subject and reach those readers. Gillman 1959 reported a case of congenital absence of the odontoid. Tumors of the craniovertebral junction clinical gate. The original and definitive reference on surgery of the craniovertebral junction, now in an updated second edition.
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. It is a biomechanically complex articulation comprising the. Craniovertebral junction anomalies radiology reference. Craniovertebral anomalies pdf craniovertebral anomaliesdr. Detailed description of the various fixation techniques currently used in the treatment of craniovertebral junction cvj anomalies. Vertebral artery anomalies at the craniovertebral junction. 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. Craniovertebral junction anomalies knowledge for medical students and physicians reduction in spasticity is appreciated in the immediate post operative period. Craniovertebral junction especially with bony anomalies like basilar invagination bi and atlantoaxial dislocation aad have often been. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle, and atlantooccipital joint axis angle. The anomalies were classified according to their embryonic origin. Embryology and bony malformations of the craniovertebral junction, childs nervous system, 2011, pp.