Syringomyelia as a complication of Paget's disease

1987 ◽  
Vol 66 (4) ◽  
pp. 611-613 ◽  
Author(s):  
Kost Elisevich ◽  
Suzanne Fontaine ◽  
Gilles Bertrand
Keyword(s):  
Paget’S Disease ◽  
Adult Life ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Paget's Disease ◽  
Content Type ◽  
Chiari Malformations ◽  
Fine Print

✓ Cranial settling and basilar invagination with medullospinal compression is believed to have resulted in the production of a syrinx in a case of Paget's disease of the cranium. This mechanism of compression at the craniovertebral junction resembles the development of syringomyelia in Chiari malformations presenting in adult life.

Scoliosis in pediatric Chiari malformations without myelodysplasia

1992 ◽  
Vol 77 (1) ◽  
pp. 69-77 ◽  
Author(s):  
Michael G. Muhonen ◽  
Arnold H. Menezes ◽  
Paul D. Sawin ◽  
Stuart L. Weinstein
Keyword(s):  
Mr Imaging ◽  
Surgical Intervention ◽  
Craniovertebral Junction ◽  
Spinal Curvature ◽  
Posterior Fossa Decompression ◽  
Content Type ◽  
The Mean ◽  
Spine Radiographs ◽  
Chiari Malformations ◽  
Fine Print

✓ A prospective study was undertaken in 1985 to better understand how the surgical manipulation of hind-brain herniation affected abnormal spinal curvature. Eleven patients under 16 years of age with Chiari malformation (not associated with myelodysplasia) and scoliosis of at least 15° were studied. The mean curve angle at the time of original treatment was 29°, with the convexity to the right in seven patients. The curvature was rapidly progressing in four patients. The most common presenting signs were myelopathy and weakness. Investigative procedures included spine radiographs with the patient standing and magnetic resonance (MR) imaging of the brain, spinal cord, and craniovertebral junction. Eight children had associated hydrosyringomyelia. Surgical intervention consisted of a dorsal posterior fossa decompression in all patients and a transoral ventral decompression of the cervicomedullary junction in five. All patients were followed at 3, 6, and 12 months, and at yearly intervals thereafter with clinical evaluations, spine radiographs in the standing position, and postoperative MR imaging. The mean follow-up period was 35 months. The scoliosis improved in eight patients, stabilized in one, and progressed in two. Only one child required postoperative spinal fusion and instrumentation for progression of scoliosis. Hematomyelia or hematobulbia was associated with persistent scoliosis in two patients. The presence of hydrosyringomyelia and bone erosion did not preclude curve improvement. All patients under 10 years of age had resolution of their scoliosis, despite preoperative curves of more than 40°. These findings emphasize the importance of early surgical intervention, with the restoration of normal cerebrospinal fluid dynamics at the craniovertebral junction in children with symptomatic Chiari malformations.

Treatment of basilar invagination by atlantoaxial joint distraction and direct lateral mass fixation

2004 ◽  
Vol 1 (3) ◽  
pp. 281-286 ◽  
Author(s):  
Atul Goel
Keyword(s):  
Odontoid Process ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Foramen Magnum ◽  
Atlantoaxial Joint ◽  
Lateral Mass ◽  
Content Type ◽  
Joint Distraction ◽  
History Of ◽  
Fine Print

Object. The author discusses the successful preliminary experience of treating selected cases of basilar invagination by performing atlantoaxial joint distraction, reduction of the basilar invagination, and direct lateral mass atlantoaxial plate/screw fixation. Methods. Twenty-two patients with basilar invagination—in which the odontoid process invaginated into the foramen magnum and the tip of the odontoid process was above the Chamberlain, McRae foramen magnum, and Wackenheim clival lines—were selected to undergo surgery. In all patients fixed atlantoaxial dislocations were documented. The 16 male and six female patients ranged in age from 8 to 50 years. A history of trauma prior to the onset of symptoms was documented in 17 patients. Following surgery, the author observed minimal-to-significant reduction of basilar invagination and alteration in other craniospinal parameters resulting in restoration of alignment of the tip of the odontoid process and the clivus and the entire craniovertebral junction in all patients. In addition to neurological and radiological improvement, preoperative symptoms of torticollis resolved significantly in all patients. The minimum follow-up period was 12 months and the mean was 28 months. Conclusions. Joint distraction and firm lateral mass fixation in selected cases of basilar invagination is a reasonable surgical treatment for reducing the basilar invagination, restoring craniospinal alignment, and establishing fixation of the atlantoaxial joint.

Neurological dysfunction in Paget's disease of the vertebral column

1972 ◽  
Vol 37 (6) ◽  
pp. 661-665 ◽  
Author(s):  
Edward S. Sadar ◽  
Robert J. Walton ◽  
Henry H. Gossman
Keyword(s):  
Neurological Deficit ◽  
Vertebral Column ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Neurological Dysfunction ◽  
Decompressive Laminectomy ◽  
Content Type ◽  
Sarcomatous Degeneration ◽  
Myelographic Block ◽  
Fine Print

✓ The authors report four cases of Paget's disease of the vertebral column causing neurological deficit, one with sarcomatous degeneration and one without compression of the spinal cord, and review 86 reported cases. Most cases responded well to decompressive laminectomy. The paradox of neurological deficit without myelographic block is discussed and the relatively common incidence of sarcomatous degeneration noted.

Extreme lateral—transatlas approach for resection of the dens of the axis

2002 ◽  
Vol 96 (1) ◽  
pp. 73-82 ◽  
Author(s):  
Uğur Türe ◽  
M. Necmettin Pamir
Keyword(s):  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Clinical Experiences ◽  
Occipitocervical Fusion ◽  
Content Type ◽  
Advantages And Disadvantages ◽  
Alternative Approach ◽  
Craniocervical Instability ◽  
Fine Print

Object. Various approaches have been described for resection of the dens of the axis, each of which has potential advantages and disadvantages. Anterior approaches such as the transoral route or its modifications are the most commonly used for resection of this structure. The transcondylar approach, however, which allows the surgeon to view the craniovertebral junction (CVJ) from a lateral perspective, has been introduced by Al-Mefty, et al., as an alternative approach. In this report, the authors describe the surgical technique of the extreme lateral—transatlas approach and their clinical experiences. Methods. The authors first examined the surgical approach to the dens from a lateral perspective in five cadaveric heads. They found that removal of the lateral mass of the atlas provided adequate exposure for resection of the dens. Following this cadaveric study, the extreme lateral—transatlas approach was successfully performed at the authors' institution over a 1-year period (September 1998–August 1999) in five patients with basilar invagination due to congenital anomaly of the CVJ and rheumatoid arthritis. Furthermore, during the same procedure, unilateral occipitocervical fusion was performed following resection of the dens. In all cases complete resection of the dens was achieved using the extreme—lateral transatlas approach. This procedure provides a sterile operative field and the ability to perform occipitocervical fusion immediately following the resection. No postoperative complications or craniocervical instability were observed. The mean follow-up period was 17.2 months (range 13–24 months). Conclusions. The extreme lateral—transatlas approach for resection of the dens was found to be safe and effective. Knowledge of the anatomy of this region, especially of the V3 segment of the vertebral artery, is essential for the success of this procedure.

A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation

2003 ◽  
Vol 99 (6) ◽  
pp. 1004-1009 ◽  
Author(s):  
Ricardo Vieira Botelho ◽  
Lia Rita Azeredo Bittencourt ◽  
José Marcos Rotta ◽  
Sergio Tufik
Keyword(s):  
Sleep Disturbances ◽  
Cranial Nerves ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Controlled Study ◽  
Control Group ◽  
Apnea Hypopnea Index ◽  
Respiratory Compromise ◽  
Content Type ◽  
Fine Print

Object. Craniovertebral junction malformation (CVJM) or Chiari malformation in adults, with or without syringomyelia and basilar invagination, produces neuronal dysfunction of the brainstem, cerebellum, cranial nerves, and upper spinal cord. The respiratory center and some of its afferent and efferent components can be altered in these diseases. The authors studied patients with and without CVJM to determine whether this physical feature contributed to sleep disturbances. Methods. Respiratory manifestations during sleep were studied prospectively, by using whole-night polysomnography, in 32 symptomatic patients (CVJM group) and 16 healthy volunteers (control group). Patients with CVJM presented with more sleep disturbances (reports of snoring and apnea) than those in the control group. The apnea/hypopnea index values were higher in patients with CVJMs than in the control group (13 ± 15 compared with 3 ± 6; p = 0.007) and the rate of central sleep apneas was higher in the CVJM than in the control group (22 ± 30 compared with 4 ± 8%; p = 0.009). The apnea/hypopnea index was highest in the subgroup with basilar invagination than in the other subgroups. The central apneic episodes were more frequent in the patients with basilar invagination (35 ± 40%; p = 0.001) and in those with syringomyelia (17.6 ± 24.6%; p = 0.003) than in the control group (4 ± 8%). Patients with symptomatic CVJM, especially those with basilar invagination, presented with more sleep respiratory compromise than did those in the control group. Conclusions. The incidence of sleep apnea/hypopnea syndrome is significantly higher in patients with CVJM.

Terminal ventriculostomy for syringomyelia

1977 ◽  
Vol 46 (5) ◽  
pp. 609-617 ◽  
Author(s):  
W. James Gardner ◽  
Herbert S. Bell ◽  
Pete N. Poolos ◽  
Donald F. Dohn ◽  
Marta Steinberg
Keyword(s):  
Clinical Course ◽  
Adult Life ◽  
Central Canal ◽  
Conus Medullaris ◽  
Fetal Life ◽  
Filum Terminale ◽  
Content Type ◽  
Fine Print

✓ The clinical course of 12 patients who underwent terminal ventriculostomy for syringomyelia is presented. Opening the central canal at the tip of the conus medullaris is a relatively benign procedure that improves the symptoms of syringomyelia and syringobulbia. This canal normally terminates at the tip of the conus, but in each of the 12 surgical specimens it continued into the filum terminale for distances up to 8 cm. In most cases the tip of the conus was located more caudally than normal, indicating some degree of tethering in fetal life. This belief is supported by the fact that the newborn, whose conus is tethered to a lipoma at the sacral level, may develop syringomyelia in adult life.

Stenosis of the central canal of the spinal cord following inoculation of suckling hamsters with reovirus type I

1994 ◽  
Vol 81 (1) ◽  
pp. 103-106 ◽  
Author(s):  
Thomas H. Milhorat ◽  
René M. Kotzen
Keyword(s):  
Spinal Cord ◽  
Adult Life ◽  
Central Canal ◽  
Early Years ◽  
Type I ◽  
Content Type ◽  
Human Spinal Cord ◽  
Reovirus Type ◽  
Infectivity Titer ◽  
Fine Print

✓ The central canal of the human spinal cord is partially or completely occluded in the vast majority of individuals by the early years of adult life. The authors describe an experimental lesion following virus-induced ependymitis that bears a striking resemblance to the condition in man. Suckling hamsters were inoculated with 0.06 ml of 10−3 infectivity titer of reovirus type I between the 2nd and 5th days of life. The pathological events consisted of necrotizing ependymitis, healing of the ependyma by gliovascular scarring, and obstruction of narrow bottlenecks such as the central canal. Histological findings were characterized by disorganization of the ependyma, formation of ependymal rosettes and microtubules, subependymal gliovascular scarring, and intracanalicular gliosis. These features are the same as those encountered clinically and provide strong evidence that stenosis of the central canal in man is a pathological lesion involving ependymal injury and scarring.

The influence of transoral odontoid resection on stability of the craniovertebral junction

1992 ◽  
Vol 77 (4) ◽  
pp. 525-530 ◽  
Author(s):  
Curtis A. Dickman ◽  
Jacqueline Locantro ◽  
Richard G. Fessler
Keyword(s):  
Rheumatoid Arthritis ◽  
Bone Destruction ◽  
Craniovertebral Junction ◽  
Transoral Surgery ◽  
Posterior Decompression ◽  
Radiographic Evidence ◽  
Content Type ◽  
Surgical Interventions ◽  
Fine Print

✓ Twenty-seven cases of craniovertebral junction compression treated with transoral surgery were reviewed to assess the influences of pathological processes and surgical interventions on spinal stability. All patients presented with signs and symptoms of spinal-cord or brain-stem dysfunction. Pathology included rheumatoid arthritis in 11 patients, congenital osseous malformations in 11, spinal fractures in two, plasmacytoma in one, osteomyelitis in one, and a gunshot injury in one. Instability was defined as clear radiographic evidence of mobile subluxation in conjunction with clinical assessment. Of 19 patients (70%) requiring internal fixation, nine underwent upper cervical fusion and 10 had occipitocervical fusion. When instability occurred, all subluxations were at the C1–2 level. There were no occipitoatlantal subluxations. Eight patients (30%) had preoperative instability of the craniovertebral junction due solely to their pathology, 11 patients (40%) suffered instability after transoral surgery, and eight (30%) were without clinical or radiographic evidence of instability (mean follow-up period 14 months). Craniovertebral junction instability predominated among patients with rheumatoid arthritis: 91% required fusion and 45% presented with pre-existing instability. Among individuals with congenital osseous malformations, 45% required fusion and only one patient (9%) had pre-existing instability. Patients who required subsequent posterior decompression of a Chiari malformation were at risk for developing instability; three of four became unstable after posterior decompression. Transoral resection of the dens, the anterior arch of C-1, and the lower clivus does not fully destabilize the spine; however, this operation may potentiate incipient pathological instability. The primary determinants of instability are the extent of pathological bone destruction, ligamentous weakening, and operative bone removal. Long-term follow-up monitoring is needed after transoral surgery to detect cases of late instability.

Primary spinal syringomyelia

2005 ◽  
Vol 3 (6) ◽  
pp. 429-435 ◽  
Author(s):  
Ulrich Batzdorf
Keyword(s):  
Spinal Cord ◽  
Cerebrospinal Fluid ◽  
Fluid Flow ◽  
Craniovertebral Junction ◽  
Arachnoid Cysts ◽  
Spinal Cord Tumors ◽  
Content Type ◽  
Cerebrospinal Fluid Flow ◽  
Different Types ◽  
Fine Print

✓ In the present review the author describes the different types of syringomyelia that originate from abnormalities at the level of the spinal cord rather than at the craniovertebral junction. These include posttraumatic and postinflammatory syringomyelia, as well as syringomyelia associated with arachnoid cysts and spinal cord tumors. The diagnosis and the principles of managing these lesions are discussed, notably resection of the entity restricting cerebrospinal fluid flow. Placement of a shunt into the syrinx cavity is reserved for patients in whom other procedures have failed or who are not candidates for other procedures.

Stability of the craniovertebral junction after unilateral occipital condyle resection: a biomechanical study

1999 ◽  
Vol 90 (1) ◽  
pp. 91-98 ◽  
Author(s):  
A. Giancarlo Vishteh ◽  
Neil R. Crawford ◽  
M. Stephen Melton ◽  
Robert F. Spetzler ◽  
Volker K. H. Sonntag ◽  
...  
Keyword(s):  
Axial Rotation ◽  
Craniovertebral Junction ◽  
Biomechanical Study ◽  
Occipital Condyle ◽  
Upper Cervical Spine ◽  
Lateral Bending ◽  
Content Type ◽  
Axial Translation ◽  
Flexion Extension ◽  
Fine Print

Object. The authors sought to determine the biomechanics of the occipitoatlantal (occiput [Oc]—C1) and atlantoaxial (C1–2) motion segments after unilateral gradient condylectomy. Methods. Six human cadaveric specimens (skull with attached upper cervical spine) underwent nondestructive biomechanical testing (physiological loads) during flexion—extension, lateral bending, and axial rotation. Axial translation from tension to compression was also studied across Oc—C2. Each specimen served as its own control and underwent baseline testing in the intact state. The specimens were then tested after progressive unilateral condylectomy (25% resection until completion), which was performed using frameless stereotactic guidance. At Oc—C1 for all motions that were tested, mobility increased significantly compared to baseline after a 50% condylectomy. Flexion—extension, lateral bending, and axial rotation increased 15.3%, 40.8%, and 28.1%, respectively. At C1–2, hypermobility during flexion—extension occurred after a 25% condylectomy, during axial rotation after 75% condylectomy, and during lateral bending after a 100% condylectomy. Conclusions. Resection of 50% or more of the occipital condyle produces statistically significant hypermobility at Oc—C1. After a 75% resection, the biomechanics of the Oc—C1 and C1–2 motion segments change considerably. Performing fusion of the craniovertebral junction should therefore be considered if half or more of one occipital condyle is resected.

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