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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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Liyong Sun, Jian Ren and Hongqi Zhang

Craniocervical junction dural arteriovenous fistula (CCJDAVF) is a rare and unique type of intracranial DAVF with complex neurovascular anatomy, making it difficult to identify the arterialized vein during operation. The authors report the case of a 50-year-old male who presented with symptoms of venous hypertensive myelopathy. Angiography demonstrated a left CCJDAVF. The fistula was successfully disconnected via a suboccipital midline approach. The selective indocyanine green videoangiography (SICG-VA) technique was applied to distinguish the fistula site and arterialized vein from adjacent normal vessels. Favorable clinical and angiographic outcomes were attained. The detailed operative technique, surgical nuances, and utility of SICG-VA are illustrated in this video atlas.

The video can be found here: https://youtu.be/GJYl_jOJQqU.

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H. Alan Crockard and Robert Bradford

T he transoral approach to anteriorly placed lesions at the craniocervical junction has been in use for over 20 years. 4, 15 It is now becoming established as a relatively safe and effective method for dealing with a variety of extradural lesions around the clivus, 6, 16 foramen magnum, 8, 9 atlantoaxial complex, 1, 5, 11, 14 and upper cervical spine. 2, 10 The use of the transoral route to treat intradural lesions, in particular basilar aneurysms, 3, 12 has been less successful. The most serious problem with this approach is the high incidence of

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Harry M. Rogers and Shelley N. Chou

posterior arch of C-1 narrowed the spinal canal at the cervicomedullary junction. Skeletal survey showed multiple lucent cortical defects in the proximal femora and tibias and in the left iliac wing. Pneumoencephalogram was normal, as was the CSF examination. The clinical impression was that he had a dysplastic process in the bone, in and around the craniocervical junction, which was responsible for his headaches. Fig. 1. Skull x-ray film showing multiple lytic lesions in the occiput, base of the skull, and upper cervical spine. Operation On October

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Grant W. Mallory, Grigoriy Arutyunyan, Meghan E. Murphy, Kathryn M. Van Abel, Elvis Francois, Nicholas M. Wetjen, Jeremy L. Fogelson, Erin K. O'Brien, Michelle J. Clarke, Laurence J. Eckel and Jamie J. Van Gompel

T he conventional approach to pathology of the ventral craniocervical junction remains the transoral route with or without various modifications to increase exposure as required by pathology. 20 However, combining the transoral route with splitting of the soft palate, maxillotomy, glossotomy, or mandibulotomy also increases morbidity. 6 , 22 , 28 To avoid additional tissue disruption, retraction of the soft palate and other approaches have been considered with or without the adjunct of an endoscope to improve exposure. 24 Other alternatives include a

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Benedicto O. Colli and Ossama Al-Mefty

prognosis of patients with chordomas. The objective of this study was to analyze the follow-up results of a group of patients with chordomas and chondrosarcomas of the craniocervical junction and to determine the prognostic factors for these patients. Clinical Material and Methods Patient Population In this study the authors analyzed data obtained in 63 consecutive patients with chordomas and chondrosarcomas of the craniocervical junction treated by the same surgeon (O.A.) at three different institutions (University of Mississippi Medical Center, Loyola

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Marcus D. Mazur, Vijay M. Ravindra and Douglas L. Brockmeyer

dysplasia (SED) or Down syndrome. In other cases, patients are symptomatic from medullary or spinal cord compression and present with myelopathy and/or bulbar findings. A standard biomechanical axiom states that to achieve the torsional rigidity necessary to facilitate fusion at the craniocervical junction (CCJ), it is necessary to have bilateral fixation. This concept has been passed down over many years, and has been examined in a small number of biomechanical studies that have evaluated atlantoaxial fixation. 13 , 14 In certain circumstances, such as when bone is

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Farhad M. Limonadi and Nathan R. Selden

the CCJ decompression and duraplasty. Patients undergoing the dura-splitting procedure return home faster and have significantly lower operative and total hospital costs. A prospective randomized study should be conducted to compare these techniques in a uniform patient population. Abbreviations used in this paper CCJ = craniocervical junction ; CSF = cerebrospinal fluid ; LOS = length of stay ; MR = magnetic resonance . References 1. Arnett B : Arnold-Chiari malformation. Arch Neurol

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Thomas J. Sorenson, Lucio De Maria, Leonardo Rangel-Castilla and Giuseppe Lanzino

Craniocervical junction dural arteriovenous fistulas (dAVFs) are rare vascular lesions with a potentially dangerous natural history due to the onset of neurological deficit secondary to intracranial hemorrhage or myelopathy due to venous congestion. Despite advances in endovascular techniques, many dAVFs located in this area continue to require surgical treatment as embolization is often not feasible or safe. In this video, the authors illustrate a patient with a symptomatic craniocervical junction dAVF who had undergone attempted Onyx embolization at another institution. Because of persistent filling of the fistula and worsening myelopathy after the previous attempt, the patient was referred to the authors’ clinic for definitive surgical treatment. The video illustrates the typical location of the early draining vein in most craniocervical junction dAVFs immediately below the emergence of the vertebral artery from the dura. The patient underwent successful definitive clip ligation of the fistula, which was exposed through a lateral suboccipital craniotomy.

The video can be found here: https://youtu.be/Bvg6VKLgwO0.