Congenital arthrogryposis associated with atlantoaxial subluxation and dysraphic abnormalities

2000 ◽  
Vol 93 (1) ◽  
pp. 130-132
Author(s):  
Wolf O. Luedemann ◽  
Marcos S. Tatagiba ◽  
Sami Hussein ◽  
Madjid Samii
Keyword(s):  
Traffic Accident ◽  
Craniocervical Junction ◽  
Atlantoaxial Subluxation ◽  
Arthrogryposis Multiplex Congenita ◽  
Good Clinical Outcome ◽  
Content Type ◽  
Dorsal Stabilization ◽  
A Minor ◽  
Transoral Decompression ◽  
Fine Print

✓ The authors report the case of a 27-year-old woman with an arthrogryposis multiplex congenita (AMC) associated with atlantoaxial subluxation. To the authors' knowledge, this is the first report of its kind. The authors review the literature with reference to dysraphic abnormalities associated with atlantoaxial subluxation and with AMC. The patient presented with severe tetraparesis following a minor traffic accident. She underwent a procedure in which transoral decompression and dorsal stabilization were performed and, postoperatively, made a good clinical outcome. The authors stress the need for diagnostic neuroimaging of the craniocervical junction in patients with AMC.

Circumferential fracture of the skull base causing craniocervical dislocation

2002 ◽  
Vol 97 (1) ◽  
pp. 118-122 ◽  
Author(s):  
Ganesh Rao ◽  
Adam S. Arthur ◽  
Ronald I. Apfelbaum
Keyword(s):  
High Speed ◽  
Unusual Case ◽  
Motor Vehicle ◽  
Neurological Impairment ◽  
Craniocervical Junction ◽  
Motor Vehicle Accidents ◽  
Review Of The Literature ◽  
Content Type ◽  
Vehicle Accidents ◽  
Fine Print

✓ Fractures of the craniocervical junction are common in victims of high-speed motor vehicle accidents; indeed, injury to this area is often fatal. The authors present the unusual case of a young woman who sustained a circumferential fracture of the craniocervical junction. Despite significant trauma to this area, she suffered remarkably minor neurological impairment and made an excellent recovery. Her injuries, treatment, and outcome, as well as a review of the literature with regard to injuries at the craniocervical junction, are discussed.

Vertical translocation: the enigma of the disappearing atlantodens interval in patients with myelopathy and rheumatoid arthritis. Part I. Clinical, radiological, and neuropathological features

1997 ◽  
Vol 87 (6) ◽  
pp. 856-862 ◽  
Author(s):  
Adrian T. H. Casey ◽  
H. Alan Crockard ◽  
Jennian F. Geddes ◽  
John Stevens
Keyword(s):  
Cervical Myelopathy ◽  
Survival Rates ◽  
Cord Compression ◽  
Atlantoaxial Subluxation ◽  
Lateral Mass ◽  
Progressive Decrease ◽  
Content Type ◽  
Statistical Comparison ◽  
High Cervical ◽  
Fine Print

✓ This statistical comparison between patients with cervical myelopathy secondary to horizontal atlantoaxial subluxation and those with vertical translocation is designed to elucidate the mechanisms responsible for cranial settling and the effect of translocation on the development of spinal cord compression. In a 10-year study of a cohort of 256 patients, 186 suffered from myelopathy and 116 (62%) of these exhibited vertical translocation according to the Redlund-Johnell criteria. Vertical translocation occurred after a significantly longer period of disease than atlantoaxial subluxation (p < 0.001). Translocation was characterized clinically by a high cervical myelopathy with features of a cruciate paralysis present in 35% of individuals compared with 26% who exhibited horizontal atlantoaxial subluxation (p = 0.29), but there was a surprising paucity of cranial nerve problems. The patients with vertical translocation had a greater degree of neurological disability (p = 0.002) and poorer survival rates (p = 0.04). Radiologically, vertical translocation was secondary to lateral mass collapse and associated with a progressive decrease in the atlantodens interval ([ADI], r = 0.4; p < 0.001) and pannus (p = 0.003). Thirty percent of patients exhibited an ADI of less than 5 mm. This phenomenon has been termed pseudostabilization. The authors' studies emphasize that the ADI (frequently featured in the literature) is totally unreliable as an indicator of neuraxial compromise in the presence of vertical translocation.

Atlantoaxial stabilization in rheumatoid arthritis

1991 ◽  
Vol 74 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Stephen M. Papadopoulos ◽  
Curtis A. Dickman ◽  
Volker K. H. Sonntag
Keyword(s):  
Rheumatoid Arthritis ◽  
Spinal Cord ◽  
Spinal Cord Compression ◽  
Cord Compression ◽  
Atlantoaxial Subluxation ◽  
High Rate ◽  
Content Type ◽  
Operative Stabilization ◽  
Asymptomatic Patients ◽  
Fine Print

✓ Atlantoaxial subluxation in patients with rheumatoid arthritis is common. Operative stabilization is clearly indicated when signs and symptoms of spinal cord compression occur. However, many recommend early operative fusion before evidence of appreciable neural compression occurs because 1) the myelopathy in these patients may be irreversible; 2) the overall prognosis is poor once symptoms of cord compression are present; and 3) the risk of sudden death associated with atlantoaxial subluxation is increased even in asymptomatic patients. The authors believe that rheumatoid arthritis patients in relatively good health without advanced multisystem disease and less than 65 years of age should be considered for operative stabilization if mobile atlantoaxial subluxation is greater than 6 mm. Seventeen patients with severe rheumatoid arthritis and atlantoaxial subluxation treated with a posterior arthrodesis are presented. A new method of fusion, devised by the senior author (V.K.H.S.), was utilized in all cases. Indications for operative therapy in these patients included evidence of spinal cord compression in 11 patients (65%) and mobile atlantoaxial subluxation greater than 6 mm but no signs or symptoms of cord compression in six patients (35%). Thirteen patients developed a stable osseous fusion, two patients a well-aligned fibrous union, one patient a malaligned fibrous union, and one patient died prior to evaluation of fusion stability. The details of the operative technique and management strategies are presented. Several technical advantages of this method of fusion make this approach particularly useful in patients with rheumatoid arthritis. Because of multisystem involvement of this disease, a high rate of osseous fusion is often difficult to achieve.

Infection-related spontaneous atlantoaxial dislocation in an adult

1988 ◽  
Vol 69 (3) ◽  
pp. 455-458 ◽  
Author(s):  
W. Craig Clark ◽  
Michael Coscia ◽  
James D. Acker ◽  
Keith Wainscott ◽  
James T. Robertson
Keyword(s):  
Antibiotic Therapy ◽  
Conservative Therapy ◽  
Atlantoaxial Dislocation ◽  
Atlantoaxial Subluxation ◽  
Intravenous Antibiotic ◽  
Content Type ◽  
Fusion Procedure ◽  
The Third ◽  
Intravenous Antibiotic Therapy ◽  
Fine Print

✓ This paper reports the third described case of infection-related atlantoaxial subluxation in an adult. Like most of the similar cases seen in the pediatric literature, this case was associated with a parapharyngeal β-hemolytic streptococcal abscess. Based upon this experience, the authors advocate intravenous antibiotic therapy and 1) immediate reduction followed by application of a halo brace; 2) immobilization in a halo brace for at least 3 months; and 3) a C1–2 wiring and fusion procedure for patients who fail this trial of conservative therapy.

Anatomical data on the craniocervical junction and their correlation with degenerative changes in 30 cadaveric specimens

2005 ◽  
Vol 3 (5) ◽  
pp. 379-385 ◽  
Author(s):  
Stefan A. König ◽  
Axel Goldammer ◽  
Hans-Ekkehart Vitzthum
Keyword(s):  
Articular Cartilage ◽  
Articular Surface ◽  
Craniocervical Junction ◽  
Degenerative Changes ◽  
Content Type ◽  
Articular Surfaces ◽  
Anatomical Data ◽  
Grade Ii ◽  
Grade Iii ◽  
Fine Print

>Object. The goal of this project was to measure vertebral dimensions at the craniocervical junction and to investigate degenerative changes in this region and their correlations with the anatomical data. These studies will assist in an understanding of biomechanical conditions in this region, which are clinically relevant in cases of cervicogenic headaches and vertigo. Methods. The authors examined 30 cadaveric specimens obtained from patients ranging in age from 24 to 88 years at death. Measurements of angles of the vertebrae were conducted using an imprint method. Microsections of osseous endplates and articular cartilage were graded according to their degrees of degeneration by using the Petersson classification (0, no sign of degeneration; I, superficial degeneration with several fragmentations; II, deeper degeneration with cartilaginous disintegration and penetrating ulceration; or III, complete cartilaginous degeneration with the appearance of subchondral bone in > 50% of the articular surface). The authors found Grade I changes in 100% of the occiput specimens. In the superior articular cartilage of C-1 no changes (Grade 0) were found in two specimens, whereas 6% of the specimens exhibited Grade II changes and 89% exhibited Grade I changes. In the inferior articular cartilage of C-1, 57% of the specimens displayed Grade I changes, 14% Grade II, and 20% Grade III changes. In the superior articular cartilage of C-2, 62.5% of the specimens displayed Grade I changes and 25% Grade II changes. At the occiput—C1 level the authors found a higher frequency of degeneration at the upper left articular surface of the atlas (Quadrants 1 and 3), and at the C1–2 level they found a higher frequency of degeneration at the upper left and upper right articular surfaces of the axis (Quadrants 2 and 3, respectively). Using the McNemar test, the authors investigated the frequency of affection of single quadrants in a left—right side comparison (lateral reversal). Significant differences were identified for Quadrant 2 of the upper left articular surface of C-2 and Quadrant 3 of the upper right articular surface of C-2. These results correlate with the analysis of single articular surfaces of the axis, but contradict the results for the atlas, in which no significant difference in the left—right side comparison was found. Conclusions. Severe degeneration in the atlantooccipital joints appears to be a rare condition, with no Grade II or III degeneration found in the occipital condyles and 6% Grade I, 89% Grade II, but no Grade III changes in the superior articular cartilage of the atlas. Degeneration of the inferior articular cartilage of C-1 and the superior articular cartilage of C-2 indicates that the atlantoaxial joint faces more intense mechanical exposure, which is increased at the upper joint surfaces.

Dural arteriovenous shunts at the craniocervical junction

1998 ◽  
Vol 89 (5) ◽  
pp. 755-761 ◽  
Author(s):  
Hiroyuki Kinouchi ◽  
Kazuo Mizoi ◽  
Akira Takahashi ◽  
Yoshihide Nagamine ◽  
Keiji Koshu ◽  
...  
Keyword(s):  
Venous Drainage ◽  
Craniocervical Junction ◽  
Vertebral Angiography ◽  
Content Type ◽  
Arteriovenous Shunts ◽  
Dural Arteriovenous Shunts ◽  
Intradural Space ◽  
Tomography Scanning ◽  
Fine Print

Object. A retrospective analysis was conducted of 10 patients (three women and seven men) who were treated for spinal dural arteriovenous shunts (AVSs) located at the craniocervical junction. This analysis was performed to evaluate the characteristics of this unusual location in contrast with those of the more common thoracic and lumbar AVSs. Methods. Seven patients presented with subarachnoid hemorrhage (SAH) and one with slowly progressive quadriparesis and dyspnea due to myelopathy. The other two cases were detected incidentally and included a transverse—sigmoid dural AVS and a cerebellar arteriovenous malformation. Angiographic studies revealed that the spinal dural AVSs at the C-1 and/or C-2 levels were fed by the dural branches of the radicular arteries that coursed from the vertebral artery and drained into the medullary veins. Venous drainage was caudally directed in the patient with myelopathy. In contrast, the shunt flow drained mainly into the intracranial venous system in patients with SAH. Furthermore, in four of these patients a varix was found on the draining vein. In all patients, the draining vein was interrupted surgically at the point at which this vessel entered the intradural space, using intraoperative digital subtraction angiography to monitor flow. The postoperative course was uneventful in all patients and no recurrence was confirmed on follow-up angiographic studies obtained in seven patients at 6 months after discharge. Conclusions. If computerized tomography scanning shows SAH predominantly in the posterior fossa and no abnormalities are found on intracranial four-vessel angiographic study, proximal vertebral angiography should be performed to detect dural AVS at the craniocervical junction. The results of surgical intervention for this disease are quite satisfactory.

Inclination of the odontoid process in the pediatric Chiari I malformation

2003 ◽  
Vol 98 (1) ◽  
pp. 43-49 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
Paul A. Grabb ◽  
W. Jerry Oakes
Keyword(s):  
Odontoid Process ◽  
Postoperative Outcome ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Magnetic Resonance Images ◽  
Posterior Aspect ◽  
Content Type ◽  
Chiari I ◽  
Fine Print

Object. The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. Methods. Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB—C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. Conclusions. These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.

Selection of instrumentation and fusion levels for scoliosis: where to start and where to stop

2004 ◽  
Vol 1 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Keith H. Bridwell
Keyword(s):  
Adult Patient ◽  
Degenerative Changes ◽  
Final Decision ◽  
Adolescent Patient ◽  
Lumbar Curve ◽  
Content Type ◽  
Apical Rotation ◽  
A Minor ◽  
Selection Of ◽  
Fine Print

Object. Although there are several papers in the literature regarding selection of fusion levels in the adolescent patient, fewer articles pertain to this in the adult patient. The author reviewed his experience and the literature and reports on the choice of fusion levels in the adolescent and adult patient. Methods. After a review of available data, the author determined that the proximal and distal extent of the fusion should be based on defining curves as either major or minor in the adolescent patient. It is often possible to exclude minor curves from the fusion. Relative Cobb measurement, apical deviation from the plumb line, and apical rotation are the most useful means of distinguishing a major from a minor curve. Otherwise, the proximal and distal extent of a fusion should be performed in such a way that the proximal and distal vertebrae are both neutral and stable (bisected by the center sacral line) postoperatively. Additional segments may need to be included in the adult patient in whom extensive degenerative changes and subluxations are present. The decision of whether to terminate a long fusion at L-5 or the sacrum in an adult degenerative lumbar curve is complex and many factors have to be considered. Conclusions. Guidelines exist for fusion levels in both adolescent and adult patients. Not all curves require fusion. There are many coronal and sagittal considerations that have to be analyzed when making the final decision.

Transoral decompression for myelopathy caused by rheumatoid arthritis of the cervical spine

1972 ◽  
Vol 37 (4) ◽  
pp. 493-497 ◽  
Author(s):  
Michael H. Sukoff ◽  
Milton M. Kadin ◽  
Terrance Moran
Keyword(s):  
Rheumatoid Arthritis ◽  
Cervical Spine ◽  
Cervical Myelopathy ◽  
Transoral Approach ◽  
Anterior Decompression ◽  
Posterior Decompression ◽  
Content Type ◽  
Transoral Decompression ◽  
Fine Print

✓ A case of rheumatoid cervical myelopathy that responded to posterior decompression and fusion is presented. Progression of the disease ultimately required anterior decompression through a transoral approach.

Clivus epidural hematoma

1990 ◽  
Vol 72 (4) ◽  
pp. 660-662 ◽  
Author(s):  
Akihiro Kurosu ◽  
Keiichi Amano ◽  
Osami Kubo ◽  
Hiroshi Himuro ◽  
Takeki Nagao ◽  
...  
Keyword(s):  
Traffic Accident ◽  
Rare Case ◽  
Epidural Hematoma ◽  
Similar Case ◽  
Content Type ◽  
Hyperextension Injury ◽  
Fine Print

✓A rare case of epidural hematoma of the clivus is reported in an 11 -year-old girl involved in a traffic accident which caused a severe hyperextension injury. Only one similar case has been reported in the literature. The mechanism for the formation of the hematoma of this region is discussed.

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