Journal of the anatomical society of india 1998 dec. Original research article study of pleuroapophyseal. A study of the sagittal, coronal and axial images of the ct scan films provided by the radiology department is not always sufficient to make a mental picture of. We present here a pediatric case involving recurrent syncope and anomaly of the cvj.
The journal is registered with the following abstracting partners. The odontoid process is a part of the c2 or axis vertebra and forms pivot of the structures forming the craniovertebral junction cvj. Anterior aa ligament, posterior aa ligament transverse ligament of the atlas. Craniovertebral junction anomalies linkedin slideshare. The main problem that the surgeon faces in complicated cvj anomaly is the interpretation of the ct scan to understand the exact threedimensional anatomical relationship of c1 and c2. Cvj anomalies commonly present with progressive neurologic deficits due to compression of high cervical cord, lower cranial nerves, and brainstem. Jun 30, 2019 craniovertebral anomalies pdf craniovertebral anomaliesdr. Patient number 1 in this series had the following anomalies.
Pure cm patients without bony anomalies were excluded from the study. Among the infective processes, tuberculosis tb is an important condition to affect spine. The current literature on basiondens interval in children is sparse and based on bony measurements with variable values. Postoperative pulmonary complications in patients undergoing. Table of contents free full text articles from j craniovert. Craniovertebral junction especially with bony anomalies like basilar invagination bi and atlantoaxial dislocation aad have often been. This study was aimed at highlighting the underlying etiopathogenesis of resurgery following the primary surgery undertaken in cvj abnormalities. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle, and atlantooccipital joint axis angle. True oblique axis fracture associated with congenital.
An ebook reader can be a software application for use on a computer such as microsofts free reader application, or a booksized computer. Occiput anomalies include condylustertius, condylar hypoplasia, basiocciput hypoplasia, and atlantooccipital assimilation. The pathologies of odontoid can be congenital or acquired. Have craniovertebral junction anomalies been overlooked. Certain terms are conventionally used to express the anomalies, as follows 1. Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Atlantal assimilation, or occipitalization, ranges from complete incorporation of the atlas into the occiput to discrete osseous bridges between the two. Congenital fusion of the atlas with the occiput is one of the most common anomalies of the cvj, with a prevalence rate from 0. Sep 05, 2019 craniovertebral anomalies pdf craniovertebral anomaliesdr. Furthermore, individual chapters cover endovascular surgery, endoscopic skull base techniques, navigation and robotics, ensuring that surgeons stay uptodate. Threedimensional evaluation and classification of the.
The study aimed to shed light on the advantages of suture closure. The vast majority of anomalies consist of various arch clefts, aplasias, and hypoplasias. Furthermore, implantrelated factors, requiring implant revisionremoval, or the presence of surgical site infections may cause the patient to undergo resurgery. Use of 3d printer model to study vertebral artery anatomy and. T he craniovertebral junction cvj is an anatomically complex region bound together by an intricate network of ligaments and articulations, all of which contribute to its overall stability. With the widespread availability of radiation free magnetic resonance imaging mri. Embryology and bony malformations of the craniovertebral junction. Craniospinal abnormalities and neurologic complications of. Our goal was to establish the normal values of a recently described new imaging parameter, the.
Symptomatic in infancy breathing difficulties, including apneic episodes feeding difficulties always associated with myelomeningocele features of syringomyelia or hydrocephalusif present. Ct recognition of anomalies of the posterior arch of the atlas vertebra. Basilar invagination bi and atlas occipitalization are usually associated with various bony anomalies, deformed joints, and abnormal course of vertebral arteries vas. There are three main issues in dealing with congenital craniovertebral junction cvj anomalies, namely. Cvj is a notoriously susceptible region for syndromic cvj anomalies. The radiological profile of cvj tuberculosis included prevertebral collection, odontoid erosion and presence of granulation tissue. Problems in instrumentation of syndromic craniovertebral. The annual incidence of osteogenesis imperfecta in the united states and canada is one in 10,000 to 20,000 births 4, 5. I understand that my participation in the study is voluntary and that i am free. However, there is limited data on the incidence of postoperative pulmonary complications ppcs. 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.
One of the underrecognized cause of syndromic cvj anomalies is proatlas segmentation anomaly 3,4,10. This is called basilar impression or secondary basilar invagination. Since its symptoms are diverse with late manifestation, physicians should be aware of imaging features of congenital abnormalities in cvj. Scribd is the worlds largest social reading and publishing site. The patient was treated with surgical excision, carboplatin, and toceranib. 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. Access to the latest issue is reserved only for the paid subscribers. This paper reports 7 cases of craniovertebral junction cvj anomalies. Anomalies in the craniovertebral junction cvj may be a cause of syncope, but appear to be extremely rare. Aug 01, 2017 pediatric craniovertebral junction surgery 23 neurol med chir t okyo xx, month, 20xx fig. Widening of the basiondens interval is an important sign of cranioverterbral junction injury. The authors studied 189 consecutive cases of pediatric bony craniovertebral junction abnormalities operated between 2001 and march, 2010. Lower cervical spine disorders can usually be distinguished clinically based on level of spinal cord dysfunction and by neuroimaging.
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 occiputp. Some patients with osteogenesis imperfecta experience ligamentous laxity, joint hyperflexibility, and dental abnormalities. Pediatric posterior cerebral artery stroke as a presentation. Mechanisms of syncope relating to cvj anomalies remain unclear. A total of 7 procedures were performed in 55414 % patients.
The patient had an extended disease free interval and a survival time of 45. These anomalies included various bony irregularities that suggest a congenital origin, which included one 1 more of the following. Cvj tuberculosis was diagnosed based on the clinical profile, systemic investigations which include chest xray, erythrocyte sedimentation rate and the mantoux test and specific ct and mri features of the cvj. Have craniovertebral junction anomalies been overlooked as. In one series they affect children and young adults primarily. Similarly bharucha and dastur4 analysed 40 cases of cvj anomalies and found aad was more common both due to congenital or acquired cause. A craniocervical abnormality is suspected when patients have pain in the neck or occiput plus neurologic deficits referable to the lower brain stem, upper cervical spinal cord, or cerebellum.
Role for craniovertebral realignment atul goel neurol india. Craniovertebral junction the craniovertebral or craniocervical junctioncvj is a collective term that refers to the occiput posterior skull base, atlas, axis, and supporting ligaments. Congenital anomalies include various types of odontoid dysgenesis such as os odontoideum, condylus tertius, persistent osterminale, and odontoid aplasia. Craniocervical junction abnormalities neurologic disorders. These anomalies include aplasias, hypoplasias, and clefts ofthe atlas arches. Craniovertebral junction reducible and irreducible.
These anomalies often include the coexistence of basilar invagination and atlantoaxial dislocation. To study the clinical features and treatment outcome of pediatric patients with bony craniovertebral abnormalities. A rare presentation of an extraskeletal osteosarcoma, suspected to have evolved from chronic inflammation associated with a benign hair follicle tumor in a dog is described. Use of 3d printer model to study vertebral artery anatomy and variations in developmental craniovertebral junction anomalies and as a preoperative toolan institutional experience. Imaging of the craniovertebral junction anomalies in children. Association of craniovertebral junction anomalies, klippelfeil. The pathologies were developmental n 162, traumatic n 18 and. Cervical canal stenosis in cv anomalies foramen magnum. Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a. The subclinical manifestations may get aggravated in the postoperative period owing to anesthetic or surgical reasons. Most atlas anomalies produce noabnormal cvj relationships and are not associated with basilar invagination. Severity of basilar invagination and atlantoaxial dislocation.
Pediatric craniovertebral junction surgery 23 neurol med chir t okyo xx, month, 20xx fig. Following a brief introduction to the relevant anatomy and biomechanics of cvj, it explores the field of craniovertebral junction lesions and their management. Surgery of the craniovertebral junction enrico tessitore. The suspected mechanisms of syncope in the present case are discussed. Vertebral artery anatomy is highly variable in this region posing challenges during surgery. Magnetic resonance imaging of traumatic injury to the.
Complex cvj anomalies are often associated with anomalous va, the most common being the persistent first intersegmental artery. Cv junction anomalies free download as powerpoint presentation. Request pdf imaging of the craniovertebral junction anomalies in children the craniovertebral junction cvj is interposed between the unsegmented skull and the segmented spine. Craniovertebral junction anomalies radiology reference. Embryology and bony malformations of the craniovertebral. Yerramneni et al5 studied 100 cases of cvj anomalies and found developmental pathology is the commonest cause. Pediatric bony craniovertebral junction abnormalities. Basic anatomy the cvj is composed of the occiput, the foramen magnumand the first two cervical vertebrae the atlas, and the axis. Cv junction anomalies human anatomy human head and neck. 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. About 50 patients with cvj anomalies were taken for this prospective study. Pdf access policy full text access is free in html pages. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. Spinal instrumentation using rods and screws have become procedure of choice for posterior fixation.
In basilar invagination, there is crowding of structures in the small post. Different types of cvj anomalies may accompany upper cervical kfs. An unusual cause of vertebrobasilar insufficiency in a. Dec 31, 2010 congenital fusion of the atlas with the occiput is one of the most common anomalies of the cvj, with a prevalence rate from 0. Craniometric measurements used in radiologic assessment of cvj anomalies include the chamberlain line, wackenheim clivus baseline, welcher basal angle. Craniovertebral anomalies pdf craniovertebral anomaliesdr. Epub access policy full text in epub is free except for the current issue. Additionally, the combination of cvj anomalies, kfs and dc may represent a new syndrome that has previously gone unnoticed. Original article suture repair in endoscopic surgery for. Multidetector computed tomography and magnetic resonance. In patients with craniovertebral junction cvj anomalies, the respiratory system is adversely affected in many ways.
In conclusion, occipital condylar dysplasia is a rare congenital anomaly that can occur in cvj as an isolated anomaly or as part of a syndrome. Pdf occipitocervical junction is an important area of the spine. Ksmoker, md the craniovertebral junction cvj comprises the occiput, atlas, and. The craniovertebral junction cvj comprises the occiput, atlas, and axis and is visible in most magnetic resonance mr imaging studies of the brain. Use of 3d printer model to study vertebral artery anatomy. They vary in severity from gross failure of development incompatible with life, to anomalies that are discovered. Patients with vbi are rarely investigated for cvj anomalies, despite the fact that a significant number of patients with congenital cvj anomalies has vbi. Causes of resurgery could be divided into ventral redo or denovo transoral decompression tod or woundrelated complications, n 33, 40. Cvj anomalies that decrease the volume of the posterior cranial fossa e. In children with syndromic cvj anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Careful exam reveals a small hypoplastic odontoid at the upper border of the body of the axis. Problems in instrumentation of syndromic craniovertebral junction. Clinical manifestations of cv anomalies age sex familial occurrence precipitating factors onset 50.
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