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Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation

By Shenglin Wang, Chao Wang, Ming Yan, Haitao Zhou and Liang Jiang


A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes. Radiographs showed occipitalization of the atlas, C2-C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ) compression from the odontoid, a Chiari type I malformation, and syringomyelia extending from the foramen magnum to C5. The patient underwent transoral atlantoaxial release followed by posterior internal fixation from the occiput to the axis, which resulted in a significant improvement in motor function in all extremities. Post-operative images showed anatomical reduction of the atlantoaxial joint. However, an MRI performed 8 days following surgery showed a new retro-odontoid pannus had developed that was compressing the spinal cord at CMJ. A follow-up CT scan performed at 6 months post-operatively demonstrated a solid bony fusion between the occiput and C2, while an MRI at that time showed complete resolution of the retro-odontoid soft tissue mass with correction of the Chiari I malformation, and resolution of the syringomyelia. Final follow-up at 2-years revealed an excellent clinical outcome.Clinical NeurologyOrthopedicsSCI(E)PubMed7ARTICLE3361-3661

Topics: Syringomyelia, Irreducible atlantoaxial dislocation, Basilar invagination, Surgery, Transoral release, DISTRACTION, FIXATION
Publisher: european spine journal
Year: 2010
DOI identifier: 10.1007/s00586-009-1208-1
OAI identifier: oai:localhost:20.500.11897/196485
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