To understand the anatomy of the craniovertebral junction. In one patient, complete cessation of the hfs occurred for a period of two years following simple bony decompression of the craniovertebral junction raising unanswered questions as to the exact pathogenesis of hfs. The age at which this synchondrosis fuses ranges from after the 12 yr 14 yrs for girls and 16 yrs for boys. Craniovertebral anomalies present an array of complex bony malformations leading to a range of symptoms secondary to neural compression, malalignment of bone and subtle or manifest instability. Also, in 22 volunteers, examined using the multislice computed tomography msct, 14 parameters and 2 angles were measured in the neutral. Each concise chapter focuses on a single pathology to guide readers through the nuances of these intricate and demanding surgical procedures. True oblique axis fracture associated with congenital. Craniovertebral junction especially with bony anomalies like basilar invagination bi and atlantoaxial dislocation aad have often been considered as the last bastion of the spine surgeons. The craniovertebral junction is an intricate structure with unique anatomy and complex biomechanical characteristics. Craniovertebral junction anomalies linkedin slideshare.
Assimilation can occur at the site of the anterior or posterior arches or lateral masses, or any combination of the above. 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. Hemifacial spasm and craniovertebral anomaly canadian. Pdf craniovertebral anomalies associated with pituitary. A craniovertebral junction and cervical spine magnetic resonance imaging study did not show neural alterations or any other associated abnormalities, such as protrusion of disk material, a syringomyelic cavity, narrowing of the sagittal diameter at the level of the fused segment, compression of the spinal cord, basilar invagination, chiari. Unique axis fracture with craniovertebral junction anomalies different types of cvj anomalies may accompany upper cervical kfs. In contrast, in the orient, a greater incidence of isolated cvj anomalies is seen. Exposure of the atlantoaxial joint can be relatively complex, particularly in patients with basilar invagination and neck deformities. Complex chiari, or the presence of craniovertebral abnormalities or instability in addition to the presence of cerebellar tonsillar herniation, is present in approximately one fourth of all cases of chiari 1 malformation. One of the most common anomalies of the craniocervical junction is assimilation of the atlas, which has been reported to have a prevalence rate of 0. Joint hypermobility that is much more common appears, however, to be a poor predictor.
The msct angiography presented a segmental dilatation of both vertebral arteries and the a2 segment of the anterior cerebral artery, as well as a duplication of the basilar artery. Anomalies of the cvj may be congenital or acquired. Occult respiratory dysfunction in a craniovertebral anomaly. One cadaver head was sectioned in the sagittal plane. 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.
Jul 28, 2015 craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago. Reading from smarttool angle finder represents craniovertebral angle when two indicator lines were aligned with pin markers seventh cervical c7 vertebra spinous process and tragus. S urgery for craniovertebral junctional anomalies can be a technical challenge. The cvj anomalies are more common in india compared with the west. David moro marked it as toread aug 31, there is more than one author in the goodreads database with this name. Measurement of craniovertebral angle with ehpi evaluated the validity of the ehpi in measuring cv angle by correlating the measurements of cv angle with aht in lateral cervical radiographs. Fusion of a nonseparated dens is an even rarer anomaly. Fusion of the atlas and the axis is probably the most rare cervical anomaly.
Although these are developmental anomalies, they manifest late in life, with trauma andor infection playing a promotive role. These lines are useful in determining the degree of basilar impression or invagination. The pdf file you selected should load here if your web browser has a pdf reader plugin installed for example, a recent version of adobe acrobat reader. Syncope caused by congenital anomaly at the craniovertebral junction.
Craniocervical instability and related pathologies of the craniocervical junction are an important topic for anyone diagnosed with chiari 1 malformation. Craniovertebral anomalies associated with pituitary gland. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Case report open access syncope caused by congenital anomaly. Pdf on jan 1, 2005, atul goel and others published craniovertebral anomalies. Aad, occipitalization of the atlas, and c2c3 vertebral fusion are the commonest anomalies in the order of commonality and mostly occur in combinations. These are 2 atlantooccipital joints, 1 on either side between the atlas vertebra and occipital bone. Alternatively, you can also download the pdf file directly to your computer, from where it can be opened using a pdf reader.
The journal is registered with the following abstracting partners. Craniovertebral junction surgery is complex, is anatomical, is biomechanical, is philosophical and needs highest degree of technical expertise and experience. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser. Written by masters in the field, the craniovertebral junction. Craniovertebral junction cvj anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. Neurological syndromes associated with craniovertebral anomalies. Congenital atlanto axial dislocation caad and basilar invagination bi due to congenital anomalies of the craniovertebral junction that commonly manifest in children and young adults. Measurement of craniovertebral angle with electronic head. T1wighted magnetic resonance imaging mri was performed, as well as the multislice computerised tomography msct and msct angiography in our patient, as well as in a control group of.
The treatment of these malformations is a challenging problem. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiputp. Craniosynostosis paediatric craniovertebral anomalies. This patient is unique due to complex craniovertebral congenital anomalies associated with a duplication of the pituitary gland. A bizarre developmental anomaly of the occipital bone and upper cranioverttebral spine with striking and misleading neurologic manifestations.
To download the pdf, click the download link below. Aug 01, 2017 pediatric craniovertebral junction surgery 23 neurol med chir t okyo xx, month, 20xx fig. In one series they affect children and young adults primarily. Fortytwo cases of the series were major abnormalities formed by the combination. Full text full text is available as a scanned copy of the original print version. In majority of older patients, atlanto axial dislocation aad is secondary to rheumatoid arthritis, trauma and to a lesser extent, osteoarthritis of. The authors view based on their own clinical and radiological experience is that the osodontoideum is the product of excessive. Craniosynostosis paediatric craniovertebral anomalies richard hayward frcs introduction craniosynostosis is defined here as the premature closure of one or more of the skull vault sutures. Get a printable copy pdf file of the complete article 462k, or click on a page image below to browse page by page. To assess the extent and severity of respiratory insufficiency associated with severe rheumatoid atlantoaxial dislocation and its relation to compression of the neuraxis. Occult respiratory dysfunction in a craniovertebral anomaly abbott j. Role of dynamic computed tomography scans in patients with. A, cloverleaf deformity offoramen magnum in31yearold malewith multiple occipitocervical abnormalities seefig.
Congenital anomalies of craniovertebral junction presenting. Association between joint hypermobility, scoliosis, and. Craniovertebral anomalies pdf search for happiness. We could demonstrate that 40% of the patients had facetary subluxation demonstrated in dynamic imaging and two of them. C1c2 distraction and compression technique for craniovertebral junction anomalies with basilar invagination and irreducible atlantoaxial dislocation vamsi krishna yerramneni 1amshi krishna kothav amanadha reddyr 1 1 department of neurosurgery, nizams institute of medical sciences, punjagutta, hyderabad, andhra pradesh, india.
Joint hypermobility is a common clinical characteristic of patients with osteogenesis imperfecta oi, a disorder with serious comorbidities of scoliosis and cranial. Neuroimaging in craniovertebral anomalies as seen in the. The upper portion of the clivus is formed by the basisphenoid, separated from the basi occiput by the sphenooccipital synchondrosis. 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. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. Craniocervical junction abnormalities neurologic disorders. Get a printable copy pdf file of the complete article 1. Embryology and bony malformations of the craniovertebral. Embryology and bony malformations of the craniovertebral junction. This study shows the importance of the dynamic image in patients with congenital craniovertebral junction anomalies. The craniovertebral junction cvj comprises the occiput, atlas, and axis and is visible in most magnetic resonance mr imaging studies of the brain. Craniovertebral junction anomalies in inherited disorders. Profile of patients with craniovertebral junction anomalies.
Aug 22, 2019 craniovertebral anomalies pdf cranio vertebral anomaliesdr. Craniovertebral anomalies associated with pituitary gland duplication article pdf available in folia morphologica 744. Clinical manifestations of cv anomalies age sex familial occurrence precipitating factors onset 50. All these joints are superbly adapted for eye and head coordination and serve as a universal joint and together allow horizontal and vertical scanning movements of the head. Detailed study of the craniovertebral junction cvj is necessary to completely understand the mechanism of its flexion and extension. 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. A fissure in each sclerotome separate a denser caudal half from loosely arranged cranial half. Background the ccj is a complex region of the axial skeleton located between the skullbase and the. Investigations the investigations which are invaluable in the diagnosis of craniovertebral anomalies are enlisted below. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown. Krohne dk800r dk800 variablearea flowmeter made in germany.
We hypothesize that the measurement of the ephi accurately represents the cv angle when compared with the radiographical measurement. Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Basilar invagination bi is a congenital craniovertebral junction cvj malformation characterized by the ascension of the upper cervical spine into the skull base and can be associated with other bone anomalies, such as atlas assimilation, klippelfeil syndrome, condyle hypoplasia and atlantoaxial instability14. While advances in molecular genetics have revolutionised our understanding of the various syndromes that may include craniosynostosis, and. To familiarize the viewer with the imaging findings of congenital malformations of the craniocervical junction ccj and discuss the place of mri and ct scan in making the diagnosis of these disorders. 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. Weare not aware of any previous record ofthis association. Biomechanics of the craniovertebral junction intechopen. Morphometric multislice computed tomography examination of. Complex craniovertebral junctional anomalies can be daunting to treat surgically, and preoperative information regarding the osseous abnormalities, course of the vertebral arteries, size of the pedicles, and location of the transverse foramina is invaluable to surgeons operating on these challenging cases. Respiratory abnormalities due to craniovertebral junction compression in rheumatoid disease article pdf available in annals of the rheumatic diseases 532. The craniovertebral junction pdf written by masters in the field, the craniovertebral junction.
Respiratory abnormalities due to craniovertebral junction compression in rheumatoid disease. Cvj anomalies that decrease the volume of the posterior cranial fossa e. Spillane and lambert rogers department of neurology and the surgical unit, welsh national school of medicine, the royal infirmary, cardiff. The basi occiput forms the lower portion of the clivus. Craniovertebral junction anomalies knowledge for medical. See payment details any international shipping and. Anomalies in the craniovertebral junction may be a rare cause of syncope. An extremely rare occurrence of the pituitary gland duplication inspired us to examine in detail the accompanying craniovertebral congenital anomalies in a patient involved. 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. Craniovertebral anomalies pdf craniovertebral anomaliesdr. Diagnosis, pathology, surgical techniques provides indepth coverage of uptodate management principles for this delicate anatomic region. It has been frequently reported as fusion of an os odontoideum or a fractured odontoid process. Chamberlains line and mcgregors line are drawn from the tip of the hard palate to the opisthion and the lowest point of the occipital squamosal surface respectively fig. Embryology and bony malformations of the craniovertebral junction, childs.
Cervical canal stenosis in cv anomalies foramen magnum. Role for craniovertebral realignment find, read and cite all the research you need on researchgate. Two patients with congenital anomaly of the craniovertebral junction causing disabling hemifacial spasm hfs are presented. Craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago. Dec 14, 2015 cranio vertebral anomalies overview 1. Cranio vertebral anomalies overview linkedin slideshare.
Congenital klippelfeil malformation eg, with turner syndrome or noonan syndrome, often associated with atlantooccipital anomalies atlas hypoplasia platybasia, often with basilar invagination, chiari malformations descent of the cerebellar tonsils or vermis into the cervical spinal canal, and other abnormalities. Scoliosis and craniovertebral anomalies are strongly associated in paediatric oi patients. The right manifeto adam weishaupt to be identified as. All these are synovial joints of ellipsoidal variety. Jun 30, 2019 craniovertebral anomalies pdf cranio vertebral anomaliesdr. Exact preoperative understanding of the location of the vertebral artery and its course in relationship with the facet of axis and in the region of the arch. The most significant and common of these anomalies are. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle, and atlantooccipital joint axis angle. Pdf respiratory abnormalities due to craniovertebral. The anomalies were classified according to their embryonic origin. The impetus was further strengthened by the desire to understand the anatomy and neurosurgical mystique of the craniovertebral junct i on.