The most significant and common of these anomalies are. 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. Aug 22, 2019 craniovertebral anomalies pdf cranio vertebral anomaliesdr. Craniovertebral junction cvj disorders in the occident are usually associated with systemic disorders.
This study shows the importance of the dynamic image in patients with congenital craniovertebral junction anomalies. Weare not aware of any previous record ofthis association. The authors view based on their own clinical and radiological experience is that the osodontoideum is the product of excessive. The craniovertebral junction pdf written by masters in the field, the craniovertebral junction. A, cloverleaf deformity offoramen magnum in31yearold malewith multiple occipitocervical abnormalities seefig. Jun 30, 2019 craniovertebral anomalies pdf cranio vertebral anomaliesdr. Each concise chapter focuses on a single pathology to guide readers through the nuances of these intricate and demanding surgical procedures. The cvj anomalies are more common in india compared with the west. 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 impetus was further strengthened by the desire to understand the anatomy and neurosurgical mystique of the craniovertebral junct i on.
Craniocervical junction abnormalities neurologic disorders. Anomalies in the craniovertebral junction may be a rare cause of syncope. Spillane and lambert rogers department of neurology and the surgical unit, welsh national school of medicine, the royal infirmary, cardiff. It has been frequently reported as fusion of an os odontoideum or a fractured odontoid process. We hypothesize that the measurement of the ephi accurately represents the cv angle when compared with the radiographical measurement. An extremely rare occurrence of the pituitary gland duplication inspired us to examine in detail the accompanying craniovertebral congenital anomalies in a patient involved. Unique axis fracture with craniovertebral junction anomalies different types of cvj anomalies may accompany upper cervical kfs. Syncope caused by congenital anomaly at the craniovertebral junction. 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. The journal is registered with the following abstracting partners. 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. Get a printable copy pdf file of the complete article 1.
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. 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. To download the pdf, click the download link below. Craniovertebral junction anomalies radiology reference. Joint hypermobility is a common clinical characteristic of patients with osteogenesis imperfecta oi, a disorder with serious comorbidities of scoliosis and cranial. Biomechanics of the craniovertebral junction intechopen. Cervical canal stenosis in cv anomalies foramen magnum. Neuroimaging in craniovertebral anomalies as seen in the. 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. Craniocervical instability and related pathologies of the craniocervical junction are an important topic for anyone diagnosed with chiari 1 malformation. Craniovertebral junction anomalies linkedin slideshare. Two patients with congenital anomaly of the craniovertebral junction causing disabling hemifacial spasm hfs are presented.
The craniovertebral junction is an intricate structure with unique anatomy and complex biomechanical characteristics. Congenital malformations of the craniovertebral junction. These are 2 atlantooccipital joints, 1 on either side between the atlas vertebra and occipital bone. These characteristics allow for significant flexion, extension, and axial rotation with remarkable stability under normal circumstances. Congenital anomalies of craniovertebral junction presenting.
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. Aad, occipitalization of the atlas, and c2c3 vertebral fusion are the commonest anomalies in the order of commonality and mostly occur in combinations. Fusion of a nonseparated dens is an even rarer anomaly. Dec 14, 2015 cranio vertebral anomalies overview 1. 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. Craniovertebral junction anomalies knowledge for medical. The right manifeto adam weishaupt to be identified as. One cadaver head was sectioned in the sagittal plane. Anomalies of the cvj may be congenital or acquired. Jul 28, 2015 craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago.
See payment details any international shipping and. Neurological syndromes associated with craniovertebral anomalies. Diagnosis, pathology, surgical techniques provides indepth coverage of uptodate management principles for this delicate anatomic region. Detailed study of the craniovertebral junction cvj is necessary to completely understand the mechanism of its flexion and extension.
Craniovertebral anomalies pdf craniovertebral anomaliesdr. 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. Although these are developmental anomalies, they manifest late in life, with trauma andor infection playing a promotive role. 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. Clinical manifestations of cv anomalies age sex familial occurrence precipitating factors onset 50. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle, and atlantooccipital joint axis angle. A fissure in each sclerotome separate a denser caudal half from loosely arranged cranial half. Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Aug 01, 2017 pediatric craniovertebral junction surgery 23 neurol med chir t okyo xx, month, 20xx fig. 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. Profile of patients with craniovertebral junction anomalies. The treatment of these malformations is a challenging problem. True oblique axis fracture associated with congenital. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown.
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. In majority of older patients, atlanto axial dislocation aad is secondary to rheumatoid arthritis, trauma and to a lesser extent, osteoarthritis of. Craniovertebral anomalies pdf search for happiness. A bizarre developmental anomaly of the occipital bone and upper cranioverttebral spine with striking and misleading neurologic manifestations. In contrast, in the orient, a greater incidence of isolated cvj anomalies is seen. Background the ccj is a complex region of the axial skeleton located between the skullbase and the. Respiratory abnormalities due to craniovertebral junction compression in rheumatoid disease. Pdf craniovertebral anomalies associated with pituitary. These lines are useful in determining the degree of basilar impression or invagination. Craniovertebral junction anomalies were only anatomical and radiological curiosities till not long ago. To assess the extent and severity of respiratory insufficiency associated with severe rheumatoid atlantoaxial dislocation and its relation to compression of the neuraxis. The upper portion of the clivus is formed by the basisphenoid, separated from the basi occiput by the sphenooccipital synchondrosis.
Embryology and bony malformations of the craniovertebral junction. 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. Assimilation can occur at the site of the anterior or posterior arches or lateral masses, or any combination of the above. Pdf on jan 1, 2005, atul goel and others published craniovertebral anomalies. In one series they affect children and young adults primarily. Embryology and bony malformations of the craniovertebral. Links to pubmed are also available for selected references. Case report open access syncope caused by congenital anomaly.
The anomalies were classified according to their embryonic origin. 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. 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. 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. Craniosynostosis paediatric craniovertebral anomalies. 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. 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.
Get a printable copy pdf file of the complete article 462k, or click on a page image below to browse page by page. All these are synovial joints of ellipsoidal variety. 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 cvj anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. To understand the anatomy of the craniovertebral junction. 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. Craniovertebral anomalies associated with pituitary gland duplication article pdf available in folia morphologica 744. Occult respiratory dysfunction in a craniovertebral anomaly. The first type of fusion in caves classification is that of a separated odontoid process with the anterior atlantal arch. Krohne dk800r dk800 variablearea flowmeter made in germany. S urgery for craniovertebral junctional anomalies can be a technical challenge.
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. Cranio vertebral anomalies overview linkedin slideshare. Occult respiratory dysfunction in a craniovertebral anomaly abbott j. Also, in 22 volunteers, examined using the multislice computed tomography msct, 14 parameters and 2 angles were measured in the neutral. The basi occiput forms the lower portion of the clivus. Measurement of craniovertebral angle with electronic head. Respiratory abnormalities due to craniovertebral junction compression in rheumatoid disease article pdf available in annals of the rheumatic diseases 532. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiputp. Craniovertebral anomalies associated with pituitary gland. The age at which this synchondrosis fuses ranges from after the 12 yr 14 yrs for girls and 16 yrs for boys. Investigations the investigations which are invaluable in the diagnosis of craniovertebral anomalies are enlisted below. 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.
Role of dynamic computed tomography scans in patients with. Morphometric multislice computed tomography examination of. While advances in molecular genetics have revolutionised our understanding of the various syndromes that may include craniosynostosis, and. David moro marked it as toread aug 31, there is more than one author in the goodreads database with this name. 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. Alternatively, you can also download the pdf file directly to your computer, from where it can be opened using a pdf reader. Embryology and bony malformations of the craniovertebral junction, childs. Association between joint hypermobility, scoliosis, and. Role for craniovertebral realignment find, read and cite all the research you need on researchgate. 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. 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. Hemifacial spasm and craniovertebral anomaly canadian. Scoliosis and craniovertebral anomalies are strongly associated in paediatric oi patients.
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. Craniovertebral junction anomalies in inherited disorders. We could demonstrate that 40% of the patients had facetary subluxation demonstrated in dynamic imaging and two of them. 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.