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Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation.
Eur Spine J. 2010 Mar; 19(3):361-6.ES

Abstract

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.

Authors+Show Affiliations

Orthopaedic Department, Peking University Third Hospital, No 49. North Garden Street, HaiDian District, 100191 Beijing, China.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19941013

Citation

Wang, Shenglin, et al. "Syringomyelia With Irreducible Atlantoaxial Dislocation, Basilar Invagination and Chiari I Malformation." European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society, vol. 19, no. 3, 2010, pp. 361-6.
Wang S, Wang C, Yan M, et al. Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation. Eur Spine J. 2010;19(3):361-6.
Wang, S., Wang, C., Yan, M., Zhou, H., & Jiang, L. (2010). Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation. European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society, 19(3), 361-6. https://doi.org/10.1007/s00586-009-1208-1
Wang S, et al. Syringomyelia With Irreducible Atlantoaxial Dislocation, Basilar Invagination and Chiari I Malformation. Eur Spine J. 2010;19(3):361-6. PubMed PMID: 19941013.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation. AU - Wang,Shenglin, AU - Wang,Chao, AU - Yan,Ming, AU - Zhou,Haitao, AU - Jiang,Liang, Y1 - 2009/11/26/ PY - 2009/10/27/received PY - 2009/11/27/entrez PY - 2009/11/27/pubmed PY - 2010/6/3/medline SP - 361 EP - 6 JF - European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society JO - Eur Spine J VL - 19 IS - 3 N2 - 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. SN - 1432-0932 UR - https://www.unboundmedicine.com/medline/citation/19941013/Syringomyelia_with_irreducible_atlantoaxial_dislocation_basilar_invagination_and_Chiari_I_malformation_ L2 - https://doi.org/10.1007/s00586-009-1208-1 DB - PRIME DP - Unbound Medicine ER -