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Bernhard Zünkeler, Robert Schelper and Arnold H. Menezes

of the craniocervical junction in all patients. Plain x-ray films and polytomograms were notable for the presence of nonspecific degenerative changes as might be expected in this elderly patient population. Occasionally small areas of calcification were seen in the location of the retroodontoid mass lesion. On axial CT images multiple small globular and occasionally linear areas of calcification were present within the mass lesions in all cases; however, erosion of the odontoid process was never seen. On T 1 -weighted MR images the lesions appeared mostly

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Douglas L. Brockmeyer, Andrew Jea, Alan R. Cohen and Arnold H. Menezes

Atlas the baleful: he knows the depths of all the seas, and he, no other, guards the tall pillars that keep the sky and earth apart. — Homer, “The Odyssey” This issue of Neurosurgical Focus is devoted to one of the most fascinating topics in neurosurgery: the craniocervical junction (CCJ). Like Atlas, the mythological Titan who held up the celestial spheres, the structures that make up the CCJ are responsible for support and protection of the critical cervicomedullary structures within. As shown by the wide variety of topics presented in this issue

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Albert J. Fenoy, Arnold H. Menezes and Kathleen A. Fenoy

: Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients . J Neurosurg 69 : 895 – 903 , 1988 20 Menezes AH , VanGilder JC , Graf CJ , McDonnell DE : Craniocervical abnormalities. A comprehensive surgical approach . J Neurosurg 53 : 444 – 455 , 1980 21 Mesiwala AH , Shaffrey CI , Gruss JS , Ellenbogen RG : Atypical hemifacial microsomia associated with Chiari I malformation and syrinx: further evidence indicating that Chiari I malformation is a disorder of the paraaxial mesoderm

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Arnold H. Menezes and John C. VanGilder

decompression. If instability is present following either the ventral or dorsal decompression, posterior fixation is required. All patients can be classified into one nonoperative and five operative categories for treatment purposes ( Table 1 ). This paper focuses on the 72 patients between the ages of 6 and 82 years who underwent ventral transoral-transpharyngeal decompression of the craniocervical junction ( Table 2 ). TABLE 1 Summary of surgical treatment at the craniovertebral junction (1977–1987) * TABLE 2 Pathology in 72 patients with transoral

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Arnold H. Menezes

of the posterior fossa and cervicothoracic spine demonstrating a proatlas segmentation abnormality with a significant bone indentation into the midportion of the medulla. Note the tonsillar descent to the level between the C-2 and C-3 posterior arches. A cervicothoracic syrinx is evident and the posterior fossa volume is reduced. B: Midsagittal 3D CT scan of the craniocervical junction from an interior view. Segmentation failure of C-2 and C-3 exists with atlas assimilation and proatlas segmentation abnormality with apparent extension of the clivus into the

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Arnold H. Menezes

holes rostrally and sublaminar cables around C-1 and C-2 posterior elements caudally provide stabilization. 9 , 24 , 29 A wide-diameter contoured rod is used to increase the semirigid fixation afforded by the sublaminar cables and to improve sagittal alignment ( Fig. 7 ). The main advantage of this technique is that it is simple and provides immediate semirigid fixation of the craniocervical junction and has been reported to provide excellent fusion results. However, this method is susceptible to axial compression loads because of sliding of the rods through the

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Evaluation and treatment of congenital and developmental anomalies of the cervical spine

Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2004

Arnold H. Menezes

and stabilization ensured by performing fusion. Fig. 12. Treatment algorithm for management of cervical congenital anomalies. Abbreviations used in this paper CCJ = craniocervical junction ; CMJ = cervicomedullary junction ; CT = computerized tomography ; MR = magnetic resonance ; VA = vertebral artery ; VB = vertebral body ; 2D = two-dimensional ; 3D = three-dimensional . References 1. Callahan BC , Georgopoulos G , Eilert RE

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Brian J. Dlouhy, Nader S. Dahdaleh, Arnold H. Menezes and MD

T he craniovertebral junction (CVJ), or the craniocervical junction as it is otherwise known, is composed of the occipital bone that surrounds the foramen magnum, the atlas vertebrae, the axis vertebrae, and their associated ligaments and musculature. 73 The CVJ contains the cervicomedullary junction (CMJ) and its associated blood supply—the ascending vertebral arteries that pass through the foramen magnum and form the basilar artery. The CMJ is the crossroads of the CNS as the brainstem transitions to the upper cervical spinal cord. The medulla contains

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Raheel Ahmed and Arnold H. Menezes

disorders affecting the CVJ. 15 Neurological manifestations of this congenital disorder arise as a consequence of bony compression of the craniocervical junction. We describe a rare clinical presentation in a patient with an underlying proatlas segment bony abnormality who presented with a palatal tremor. Palatal tremor is a rare movement disorder that consists of involuntary rhythmic muscular contractions of the palatal musculature. 21 In contrast to other treatments for the common causes of palatal tremor, surgical decompression of the underlying craniovertebral

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Nader S. Dahdaleh, Brian J. Dlouhy and Arnold H. Menezes

A mong the pathological processes involving the craniocervical junction, basilar invagination remains a therapeutic challenge. As illustrated in the widely used paradigm, 4 , 5 it is essential to determine whether the invagination is reducible. Reducible lesions obviate an anterior decompression, which often involves a transoral transpalatopharyngeal approach, or one of its variations. Reports suggest that in many patients, especially in the pediatric age group, the invagination can be preoperatively reduced, rendering a dorsal-only approach sufficient. 3