Cases reported "Platybasia"

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1/9. Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.

    STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. methods: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.
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keywords = operative
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2/9. neuronavigation-assisted transoral-transpharyngeal approach for basilar invagination--two case reports.

    Two patients presented with congenital basilar invagination manifesting as progressive myelopathy. Both patients underwent surgery using a neuronavigation-assisted transoral-transpharyngeal approach. The Brain-LAB Vector Vision navigation system was used for image guidance. The registration accuracies were 0.9 and 1.3 mm. After decompression, posterior stabilization was performed. Both patients had an uneventful postoperative course. The transoral-transpharyngeal approach with the neuronavigation system provides safe exposure and decompression for basilar invagination.
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keywords = operative
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3/9. Myelopathy by lesions of the craniocervical junction in a patient with forestier disease.

    STUDY DESIGN: The authors report a case of a patient with diffuse idiopathic skeletal hyperostosis (DISH) associated with basilar impression resulting in tetraparesis. OBJECTIVE: To describe neurologic compromise associated with DISH. SUMMARY OF BACKGROUND DATA: Neurologic deficits due to DISH are very rare, and only 1 case of basilar impression associated with DISH has previously been reported in the literature. methods: diagnosis was confirmed by radiograph and MRI, which demonstrated basilar impression associated with a hyperintense signal in the spinal cord on T2-weighted sequences. Transoral resection of the dens associated with posterior occipitocervical fixation was performed during the same anesthesia. RESULTS: Postoperative outcome demonstrated regression of the pyramidal signs without recovery of unassisted walking. CONCLUSION: Early MRI should be performed in the evidence of spinal cord suffering in patients with DISH. Transoral approach allowed a good decompression of the spinal cord.
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ranking = 1
keywords = operative
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4/9. Endoscopic transoral surgery for craniovertebral junction anomalies. Technical note.

    Craniovertebral junction (CVJ) anomalies continue to be challenging for neurosurgeons because of the complex anatomy of this region. To date, microsurgical decompression via a transoral route is the standard treatment for anteriorly located compressive lesions of the cervicomedullary junction (CMJ). The results obtained by minimizing surgical trauma are fewer complications, shorter hospital stays, and reduced overall psychological burden. Endoscopic surgery is becoming a leading modality in minimally invasive neurosurgical treatment. The authors performed surgery in 11 patients with irreducible osseous dislocations resulting from CVJ abnormality during a 2-year period. Anterior CMJ decompression was achieved in all patients by performing neuroendoscopically controlled transoral excision of bone and soft tissues. The surgical technique and results will be discussed. The use of the endoscope offers several advantages in cases requiring a transoral approach to the lower clivus and atlantoaxial region. The use of minimally invasive endoscopic techniques has the potential to reduce the need for a wider cranial base opening and to decrease postoperative complications.
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keywords = operative
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5/9. Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation.

    Cine-mode magnetic resonance imaging provides simultaneous images of cerebrospinal fluid flow dynamics. A patient with a basilar impression accompanied by a Chiari malformation and von Recklinghausen's disease who underwent transoral decompression is reported. Preoperative cine-mode magnetic resonance imaging visualized an associated obstruction of cerebrospinal fluid pulsatile flow at the level of the foramen magnum. Tonsilar herniation (Chiari I malformation) and hydrocephalus were also present. Postoperatively, the obstruction of cerebrospinal fluid flow was resolved concomitant with the correction of the cervicomedullary angulation. On the basis of observations made by magnetic resonance imaging, the surgical treatment of basilar impression accompanied by Chiari malformation is briefly discussed.
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ranking = 2
keywords = operative
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6/9. The operative management of basilar impression in osteogenesis imperfecta.

    Four patients with osteogenesis imperfecta and neurologically significant basilar impression have been treated over the past 8 years. The experience has resulted in changes in our therapeutic strategy for this particularly difficult problem. These cases are discussed with respect to the disease process, neurological involvement, radiological findings, and modes of surgical therapy. The errors in management as well as the success resulting from our learning experience are described. Currently, we recommend the extensive removal of the anterior bony compression by a transoral approach. This should be followed by a posterior rigid fixation that transfers the weight of the head to the thoracic spine, in an effort to prevent further basilar invagination.
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ranking = 4
keywords = operative
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7/9. Successful treatment of adult arnold-chiari malformation associated with basilar impression and syringomyelia by the transoral anterior approach.

    A case of adult type I arnold-chiari malformation associated with basilar impression, syringomyelia, atlantoaxial dislocation, and occipitalization of the atlas is reported. Preoperative magnetic resonance imaging clearly revealed evidence of severe anterior compression of the cervicomedullary junction due to basilar impression and a sharp clivoaxial angle. Therefore, transoral anterior decompression and fusion were performed, resulting in an improvement of the patient's neurologic signs and symptoms. Postoperative magnetic resonance imaging showed an obvious reduction of the tonsillar herniation and syringomyelia, as well as an improvement of the cervicomedullary compression.
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ranking = 2
keywords = operative
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8/9. A cranio-cervical malformation presenting as acute respiratory failure.

    An 18-year-old, previously healthy male presented with bilateral pneumonia and acute respiratory failure with severe carbon dioxide retention. The presence of mild brainstem signs and hypoventilation led to the discovery of a platybasia, basilar invagination, and kinking of the medulla oblongata with early syrinx. He was operated upon but postoperatively was noted to have a mixed type of sleep apnea. This case illustrates the diagnostic challenge in acute respiratory failure in a previously healthy young person and the possible pathogenic mechanisms underlying it.
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ranking = 1
keywords = operative
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9/9. A case of Arnold-Chiari Type I malformation presenting with dysrhythmic breathing during sleep.

    A 43-year-old woman presented with dull headache, left tinnitus and dizziness. Neurological examination revealed down-beat gaze nystagmus, left tinnitus, positive Romberg sign, poor standing on the left foot, poor tandem gait, left spastic gait and positive pathological reflexes in the bilateral upper and lower extremities. Plain X-ray of the skull and cervical vertebrae demonstrated basilar impression and atlantoaxial fusion. magnetic resonance imaging of the brain and cervical spine showed cerebellar tonsil descent and syringomyelia located in the left side of the spine at the II-III vertebral level which communicated with the fourth ventricle. The patient was diagnosed as having cervical syringomyelia. Arnold-Chiari type I malformation and basilar impression. Preoperative polysomnography showed dysrhythmic breathing and bradypnea during sleep. Abnormal breathing improved after suboccipital decompression craniotomy and upper cervical laminectomy. It was suggested that dysrhythmic breathing was caused by a disorder of the medullary respiratory center. Herniation of the cerebellar tonsil and syringomyelia might have compressed the medulla.
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ranking = 1
keywords = operative
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