Syncope caused by congenital anomaly at the craniovertebral. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardiovascular manifestations poses a high anaesthetic risk to these patients. 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 craniovertebral junction is the most complex of the axial skeleton, residing between the skull and the upper cervical spine. Anomalies in the craniovertebral junction may be a rare cause of syncope. The assi monographs are a series of books commissioned by the association of spine surgeons of india with the goal of providing updated information, decisionmaking algorithms, and surgical techniques on selected spinal problems. Mechanisms of syncope relating to cvj anomalies remain unclear. Also, in 22 volunteers, examined using the multislice computed tomography msct, 14 parameters and 2 angles were measured in the neutral. However, in recent years, there has been an increase in cases of spine tuberculosis, and a few large series of tuberculosis of the cvj have been reported 1,2,9,10,14. Threedimensional evaluation and classification of the. The cvj anomalies are more common in india compared with the west. Cvj anomalies that decrease the volume of the posterior cranial fossa e.
The association between vertebrobasilar insufficiency and. Craniovertebral junction cvj anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. Craniovertebral junction cvj anomalies are rare and treatable risk factors for posterior circulation strokes pcss. Cv junction anomalies free download as powerpoint presentation. Cranio vertebral anomalies overview linkedin slideshare. Anomalies of the vertebral artery va at the craniovertebral junction cvj include. Usefulness of 3d printed models in the management of. Different types of cvj anomalies may accompany upper cervical kfs. Pdf pediatric craniovertebral junction surgery researchgate. I persistent first intersegmental artery pfia, ii fenestrated vertebral artery fva, iii lowlying posterior inferior cerebellar artery pica and iv highriding vertebral artery hrva 911,14. The journal is registered with the following abstracting partners. Cv junction anomalies human anatomy human head and neck. Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a.
Basic anatomy the cvj is composed of the occiput, the foramen magnumand the first two cervical vertebrae the atlas, and the axis. Patterns of skeletal abnormality at the craniovertebral junction in the normal population and in syndromes such as down. Craniovertebral junction anomalies radiology reference. Threedimensional computed tomography angiographic study. Images from case 7, a 25yearold man with complex cvj anomalies. The anatomy of the craniovertebral junction, although complex, may be well visualized by routine mr imaging. Aad, occipitalization of the atlas, and c2c3 vertebral fusion are the commonest anomalies in the order of commonality and mostly occur in combinations. Role of dynamic computed tomography scans in patients with. Imaging of the craniovertebral junction anomalies in. In india, atlantoaxial dislocation aad is the commonest skeletal craniovertebral junction cvj anomaly, followed by occipitalisation of atlas and basilar invagination.
The craniovertebral junction cvj is interposed between the unsegmented skull and the segmented spine. Intimate knowledge of the various surgical techniques in combination with an appreciation for anatomical variances, allows the surgeon develop a customized surgical plan tailored to each patients particular pathology and individual anatomy. The craniovertebral junction cvj is a complex anatomic region that is vulnerable to a wide spectrum of developmental and acquired anomalies owing to its unique developmental anatomy and high mobility. Our study used 3d printer model to understand the anatomy and variations in vertebral artery in live patients thereby providing an accurate idea about vertebral artery injury.
Careful exam reveals a small hypoplastic odontoid at the upper border of the body of the axis. Cvj anomalies are developmental disorders that affect the skeleton and. Anaesthesia management in craniovertebral junctional anomalies. These anomalies included various bony irregularities that suggest a congenital origin, which included one 1 more of the following. Craniovertebral junction anomalies linkedin slideshare. Craniovertebral junction cvj tuberculosis in a rare condition, accounting for 0. Cvj anomalies algorithms for management of various. Embryology and bony malformations of the craniovertebral junction. Pdf respiratory abnormalities due to craniovertebral. Tubercular spondylitis constitutes approximately 1% of all cases of tuberculosis tb. A preoperative ct sagittal reconstruction view showed aad, bi, and c1 assimilation. Evaluation of congenital anomalies of craniovertebral.
This essay discusses the anatomy of the complex articulations of the craniovertebral jun. Congenital odontoidaplasiaand posterior circulation stroke in childhood. Anomalies in the craniovertebral junction cvj may be a cause of syncope, but appear to be extremely rare. Embryology and bony malformations of the craniovertebral. B preoperative mri showed severe ventral compression of the cervicomedullary junction. Dynamic imaging of the craniovertebral junction is. True oblique axis fracture associated with congenital. Vertebral artery anatomy is highly variable in this region posing challenges during surgery. Pdf embryology and bony malformations of the craniovertebral. The craniovertebral junction cvj comprises the occiput, atlas, and axis and is visible in most magnetic resonance mr imaging studies of the brain. Review hypoplastic occipital condyle and third occipital condyle. Proatlas remnants were identified in 8 and atlas assimilation in 92 patients.
To discuss various congenital and acquired anomalies involving cvj. Different types of variations were expressed in terms of percentage. The vast majority of anomalies consist of various arch clefts, aplasias, and hypoplasias. Syncope can also be related to compression of the extracranial arteries in the neck. Agenesis of the posterior arch of the atlas and complex. Most of the literature comprises case reports and from varied specialities. Detailed study of the craniovertebral junction cvj is necessary to completely understand the mechanism of its flexion and extension. The usual presentation is progressive neurological deficit 7695% cases involving the high cervical cord, lower brainstem and cranial nerves. Craniovertebral junction tuberculosis neurosurgery. We also noted that some patients patients 3 and 10 may have mild facet joints subluxation without dynamic changes. We studied the course of 169 vas in 86 patients with congenital bony cvj anomalies basilar invagination 42, os odontoideum 33, and irreducible atlantoaxial dislocation 11. Cohengadol2 1pediatric neurosurgery, childrens hospital, birmingham, alabama 2goodman campbell brain and spine, department of neurological surgery, indiana university. Resegmentation of somites to form sclerotomes and changes of sclerotomal primordia to mature vertebral parts. These measurements can be taken on plain x rays,3d ct or on mri.
Morphometric multislice computed tomography examination of. Article information, pdf download for diagnosis and management of anomalies of. The normal adi obtained using ct scan in neutral position in a series of 100 patients without known cvj anomalies varied from 0. Download as ppt, pdf, txt or read online from scribd. Pdf the craniovertebral junction cvj has attracted more attention in pediatric medicine in recent years due to the progress in surgical. Functionally, the bony cvj can be divided into a central pillar consisting of the basiocciput and dental pivot and a twotiered ring revolving round the central. The mean age at presentation in those with cvj anomalies was 27. Type of anomaly, clinical presentation, other associated malformations were recorded. The management options for this disease entity remain. Craniovertebral junction anomalies in inherited disorders. Radiological workup of the craniovertebral anomalies.
Cervical canal stenosis in cv anomalies foramen magnum. The term craniovertebral junction refers to an area comprising the inferior. Cranio vertebral junction anomalies linkedin slideshare. Clinical manifestations of cv anomalies age sex familial occurrence precipitating factors onset 50. Imaging of the craniovertebral junction anomalies in children. Use of 3d printer model to study vertebral artery anatomy. Because of its unique anatomy, numerous craniometric indices have been devised. In this patient, the lateral radiograph showed a true oblique axis fracture, extending from the anteriosuperior border of both pars into the foramina transversarium, involving the lateral masses, pedicles, and inferior body of the axis figure 2a and b. Development of the cvj is complex and developmental anomalies may occur due to abnormal resegmentation of sclerotomes. Spinal instrumentation using rods and screws have become procedure of choice for posterior fixation. One cadaver head was sectioned in the sagittal plane.
Klippelfeil syndrome kfs is defined as congenital fusion of two or more cervical vertebrae resulting from a segmentation failure in the developing spine. Congenital and developmental osseous anomalies and abnormalities that affect the craniovertebral complex can result in neural compression, vascular compromise, and can manifest with abnormal cerebrospinal fluid dynamics. Basilar invagination bi and atlas occipitalization are usually associated with various bony anomalies, deformed joints, and abnormal course of vertebral arteries vas. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. Pdf dynamic imaging of the craniovertebral junction is. The present study was undertaken to know the embryological basis of the cvj and to identify commonly observed congenital cvj abnormalities, their frequency and mode of presentation. Diagnosed cases of cvj anomalies on computed tomography ct.
To describe vertebral artery va course at the c0c1c2 complex in patients with congenital bony craniovertebral junction cvj anomalies. Respiratory abnormalities due to craniovertebral junction compression in rheumatoid disease. Craniovertebral junctional cvj anomalies are developmental disorders that affect the skeleton and enclosed neuraxis at the junction of cranium and cervical spine. Diagnosis and management of anomalies of the craniovertebral. Existence of complex variable bony and vertebral artery va anomalies at craniovertebral junction cvj in subset of complex cvj anomalies demands individualized instrumentation policy and placing screws in each bone requires strategic preoperative planning and intraoperative skills. Hypoplastic occipital condyle and third occipital condyle. Craniovertebral anomalies pdf craniovertebral anomaliesdr. Congenital defects of the posterior atlas arch are rare, are generally asymptomatic, and are usually detected incidentally 1, 2, 3. Abstractobjective and importance four patients with craniovertebral junction anomalies cvj and klippelfeil cervical fusion defects associated with dermoid. Functionally, the bony cvj can be divided into a cent. In one series they affect children and young adults primarily.
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